Kimura Chapter 25

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Chapter 25
Polyneuropathies
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1 Introduction
Polyneuropathy consists of the triad of sensory changes in a glove and stocking distribution, distal
weakness, and hyporeflexia. Certain types of neuropathy may show widespread sensory symptoms, and
others may begin with more prominent proximal weakness. Positive sensory symptoms result from
ectopic impulse generation and autoexcitation of myelinated afferent fibers. 1 0 6 In general, but not
always, normal muscle stretch reflexes speak against peripheral neuropathy. Acute pandysautonomic
neuropathy characteristically shows severe postganglionic sympathetic and parasympathetic dysfunction,
with relative or complete sparing of motor and sensory function. 2 5 1 Milder autonomic dysfunction also
accompanies most peripheral neuropathies, but manifests clinically detectable symptoms only in a few
conditions, such as diabetes, amyloidosis, Guillain-Barr syndrome, porphyria, and familial
dysautonomia. Such autonomic disturbances usually result from acute demyelination or damage to small
myelinated and unmyelinated fibers. 5 6 0
A detailed history often reveals general medical conditions such as diabetes, alcoholism, renal disease,
malignancies, sarcoidosis, periarteritis nodosa, amyloidosis, and infectious processes such as diphtheria
and leprosy. Inflammatory neuropathies include Guillain-Barr syndrome and chronic inflammatory
demyelinative neuropathy. Metabolic neuropathies result from nutritional deficiencies or the toxic effects
of drugs or chemicals. The family history is essential in establishing the type of inherited conditions
associated with polyneuropathy. Sometimes a patient's own account may not provide sufficient
information, necessitating independent examination of family members. For some patients with an
unequivocal diagnosis of polyneuropathy, extensive studies may fail to uncover the exact etiology. 5 6 4 , 6 1 6
Hereditary and immunemediated polyneuropathy account for most cryptogenic cases. 3 1 3 In one study,
intensive evaluation permitted classification of 76 pecent of 205 patients with initially undiagnosed
neuropathy; the final diagnoses included inherited disorders in 42 percent, inflammatory-demyelinating
polyradiculoneuropathy in 21 percent, and neuropathies associated with systemic disorders in 13
percent. 2 2 9
Anatomic diagnosis depends on clinical and electrodiagnostic evaluation, but few specific patterns of
peripheral nerve involvement characterize a given disorder. Nerve conduction and electromyographic
studies delineate the extent and distribution of the lesions, and differentiate two major pathologic
changes in the nerve (see Chapter 4-6): axonal degeneration and demyelination. 9 1 , 2 0 4 An index based on
multiple electrophysiologic measures against standard norms may provide a better overall estimation, 7 8 9
as reported in the assessment of diabetic polyneuropathy. 7 0 6 Electrical studies alone rarely distinguish
clinical types of neuropathies or establish the exact etiology in a given case. Arriving at a specific
diagnosis and establishing a course of therapy depend heavily on clinical, electrophysiologic, and
histologic assessments. 1 1 , 5 1 4 , 6 1 8
This chapter reviews the essential characteristics of peripheral neuropathies as they relate to
electrophysiologic abnormalities. 2 0 4 Interested readers should consult
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other comprehensive reviews available elsewhere. 3 6 , 3 7 , 2 3 1
2 Neuropathies Associated With General Medical Conditions

Neuropathies associated with general medical conditions include some of the most commonly
encountered polyneuropathies. Despite a clear association with a general medical condition, the exact
cause of the neuropathies remains uncertain.
Diabetic Neuropathy
Diabetes often causes a symmetric polyneuropathy that likely has a metabolic basis. 2 3 2 One theory
postulates an increased amount of sorbitol in diabetic neural tissue. In hyperglycemia, glucose, shunted
through the sorbitol pathway, causes the accumulation of sorbitol in Schwann cells, which undergo
osmotic damage leading to segmental demyelination. In one study, the ulnar motor conduction velocity
and F-wave latency improved slightly but significantly after treatment with an aldose reductance
inhibitor. This finding would support the sorbitol pathway hypothesis. 2 4 5 Other factors considered
important in the pathogenesis include insulin deficiency and altered myoinositol metabolism.
An equally attractive alternative theory suggests that small vessel disease leads to infarcts within the
nerve, 6 8 8 resulting in asymmetric types of diabetic neuropathy and diabetic cranial mononeuropathies. In
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some of the affected patients, neuropathy is caused by an inflammatory vasculopathy. 4 6 3 , 7 3 0 , 9 2 9

Microvasculitis with infiltrative T cells may contribute to the pathogenesis. 9 2 9 The spatial distribution of
fiber loss also suggests ischemia and hypoxia similar to those found in experimental embolization of
nerve capillaries. 2 1 3 , 4 0 4 In fact, vascular insufficiency quantitatively aggravates diabetic neuropathy. 6 8 9
In animal studies, reduced endoneurial blood flow, insufficient to cause infarction, may result in
measurable functional and morphologic abnormalities in peripheral nerves. 7 8 2 Ischemic changes in the
nerve presumably result from proliferation of the endothelium in blood vessels and abnormalities of the
capillaries. 5 3 1
Overall, two thirds of diabetic patients have objective evidence for some variety of neuropathy, but only
about 20 percent have symptoms. 2 2 0 A wide spectrum of neuropathic processes develop. 9 1 2 The most
commonly used clinical classification 3 6 consists of (1) distal symmetric, primarily sensory, neuropathy;
(2) autonomic neuropathy; (3) proximal asymmetric painful motor neuropathy; and (4) cranial
mononeuropathies. Pathologic classification separates diabetic neuropathies into two groups:
predominantly large fiber or small fiber disease. In the larger fiber type, segmental demyelination and
remyelination predominate, perhaps as a secondary change to diffuse or multifocal axonal loss, which
seems to constitute the primary pathology. 2 2 2 This process would distort the normally linear relationship
between internodal length and fiber diameter. In the small fiber type, the primary impact of the disease
is on the axons with secondary demyelination. In some patients, abnormalities in the autonomic nervous
system closely parallel those in the peripheral nervous system. 2 In these cases, prominent histologic
changes include active axonal degeneration, affecting mainly unmyelinated and small myelinated fibers.
Distal axonopathy in experimental diabetes mellitus of the rat first affects the terminal portions of the
susceptible nerves. 9 5
The clinical presentation depends on varying combinations of the two basic types. On the whole, patients
with adult onset diabetes have the large fiber type, with symptoms consisting of distal paresthesias and
peripheral weakness. The patients have dissociated loss of vibratory, position, and twopoint
discrimination sense with relative sparing of pain and temperature sense. The vulnerability at the
common sites of compression may cause multiple pressure palsies. The small fiber type of neuropathy
characteristically affects those with insulindependent juvenile diabetes. Dysautonomia and pain
predominate, often awakening the patients at night with painful dysesthesias,
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thus the designation autonomic or painful diabetic neuropathy. Charcot's joints, perforating ulcers, and
other trophic changes of the feet may develop after severe loss of pain. Impotence and postural
hypotension result from involvement of the autonomic nerves. Parasympathetic pupillary dysfunction
precedes sympathetic pupillary denervation. 4 8 9 Quantitative measures of impaired sudomotor function
correlate well with the severity of polyneuropathy. 4 3 1 , 4 3 3 Acute painful neuropathy may follow precipitous
weight loss, but severe symptoms subside within 10 months. Histologic studies show degeneration of
both myelinated and unmyelinated axons. 1 1 7 Spontaneous axonal regeneration abounds even in advanced
cases. 7 3 1
Mononeuropathies most often involve the femoral nerve and lumbosacral plexus and, to a lesser extent,
the sciatic, common peroneal, median, ulnar, and cranial nerves. 7 , 8 3 0 Unilateral femoral neuropathy
commonly develops as a complication in elderly men with poorly controlled diabetes. Thigh pain precedes
wasting of the quadriceps and other proximal muscles of the anterior thigh. Unlike the distal symptoms
of diffuse polyneuropathy, the proximal weakness tends to improve with adequate control of the
diabetes. This condition, although usually distinguished as diabetic amyotrophy, probably represents a
form of diabetic mononeuropathy rather than a separate entity. 3 1 , 1 4 5 The sudden onset of pain may
herald involvement of a major proximal nerve trunk, including the lateral cutaneous nerve of the calf. 2 5 8
Patients may have a rapidly evolving course or continued progression for many months. The rapidly
evolving form, considered ischemic in nature, and the more slowly progressive condition, regarded as
metabolic in origin, may overlap, causing confusion. 4 5
Diabetic thoracic radiculopathy produces a distinct syndrome characterized by radicular involvement,

abdominal or chest pain, and weight loss; it has a relatively good prognosis 4 3 4 and sometimes mimicks a
myelopathy. 8 9 5 Polyradiculoneuropathy and truncal mononeuropathy may accompany advanced distal
polyneuropathy. 8 1 3 The episodes of diabetic truncal neuropathy may selectively involve the distribution of
the ventral or dorsal rami of the spinal nerves, or branches of these rami, or varying combinations of
these distributions. 8 0 5 Focal, unilateral protrusion of the abdominal wall on this basis may mimic
abdominal hernia. 6 5 8
Electrophysiologic studies have revealed a number of abnormalities in diabetic neuropathies. 1 5 0 , 2 1 8 , 5 2 4

Patients with signs of neuropathy have slower nerve conduction velocities and smaller amplitudes than
those without symptoms, 4 9 5 showing a close correlation between clinical findings and the degree of
conduction changes. 3 3 0 , 3 8 7 In juvenile patients, those with the longest duration of disease have the
highest incidence of abnormalities. 2 3 9 Patients with diabetes have abnormal persistence of sensory
evoked potentials during induced ischemia, which may herald other electrophysiologic abnormalities. 3 7 0
The degree of resistance shows a correlation with hemoglobin Ale and therefore metabolic control, but
not with the state of neuropathy. 6 8 3 Studies of spinal somatosensory evoked potentials suggest
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impairment of peripheral as well as central afferent transmission. 1 6 8 , 3 2 5 Increased interpeak latencies of

the brainstem auditory evoked responses also suggest the presence of a central neuropathy in some
cases, 2 0 3 but not in others. 8 8 0
Conduction abnormalities develop diffusely along the entire length of the nerve, but more so in distal
segments than in proximal segments (see Fig. 1811). 1 5 8 , 4 4 4 Axon loss alone cannot explain the degree
of slowing conduction velocity. 9 0 9 Abnormalities predominate at the common sites of compression, for
example, across the carpal tunnel for the median nerve. 1 2 , 4 0 2 showing a delay with no major conduction
block. 3 Studies reveal lengthdependent changes involving the tibial and peroneal nerves more than the
median and ulnar nerves, 4 4 4 with preferential involvement of the fastest conducting large myelinated
fibers. 2 0 9 Some advocate the amplitude ratio between sural and radial sensory potential as a sensitive
measure of neuropathy. 6 5 9 , 7 2 3 The disease can affect any part of the body, including the phrenic nerve. 9 1 7
Minimal F-wave latency is the most sensitive. 2 1 , 1 5 8 , 4 4 4 and reproducible 4 5 3
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measure in the assessment of conduction abnormalities of diabetic neuropathy. Motor unit number
estimates reveal an axonal loss that parallels the severity of the demyelinative process. 3 3 9
Electromyography detects fibrillation potentials and positive sharp waves in patients with prominent
axonal degeneration. Singlefiber studies provide a measure of reinnervation, 8 8 and reveals the degree
of axonal loss as the eventual cause of weakness. 2 2
Most patients with sensory motor peripheral neuropathy also show absence of sympathetic skin response
and other abnormalities of sudomotor function. 6 0 5 , 8 0 6 Useful measures for detecting a subclinical
neuropathy include nerve conduction abnormalities in two or more nerves and quantitative autonomic
examination of heart beat during deep breathing or the Valsalva maneuver. 1 5 1 , 1 5 7 , 2 1 9 , 2 6 5 , 3 5 8 In one
study, 5 9 8 combined cardiorespiratory and nerve conduction scores predicted survival better than separate
scores.
Some studies emphasize other measures to characterize and quantitate the severity of a
neuropathy. 2 2 1 , 8 3 3 Thermal threshold testing confirms lengthdependent abnormalities of the small
myelinated and unmyelinated nerve fibers, showing a good correlation with the severity of
polyneuropathy. 3 4 0 , 5 9 7 Quantitative study of vibration perception threshold, in contrast, provides a useful
measure in the assessment of the large diameter fibers. 4 4 9 These quantitative sensory tests complement
nerve conduction studies, although sural sensory potentials serve as a better predictor of diabetic
neuropathy. 6 9 2
Many studies have dealt with improved clinical management of diabetic neuropathy. 9 1 2 A controlled
doubleblind study suggested the efficacy of uridine for modifying neurophysiologic measures of
neuropathy. 2 8 1 Desipramine relieved pain caused by diabetic neuropathy with an efficacy similar to that
of amitriptyline. 5 5 0 Some investigators have suggested the therapeautic effect of ganglioside in
promoting the recovery of sensory and compound muscle action potentials presumably by fascilitating
the process of reinnervation, 4 8 but without subsequent confirmation. 3 3 4 In one study, correction of
hyperglycemia resulted in a slight increase in conduction velocity after 6 hours. 8 4 3 Another carefully
controlled study, however, revealed little improvement in conduction at the end of 3 days. 7 5 5 In a further
series, nerve conduction velocity improved by 2. 5 m/s after 1 year of improved glucoregulation with
continuous subcutaneous insulin infusion. 2 3 3 Attempts for better glycemic control, in general, show
encouraging results. 2 0 0 , 8 9 4 Good glycemic control also plays an important role in the prevention of
neuropathy in children and adolescents with diabetes mellitus. 2 8 0
Alcoholic Neuropathy
In the United States, alcohol is a major cause of peripheral neuropathy. It primarily affects those who
drink large quantities for a number of years and improves once a person abstains. 3 6 0 In addition to the
possible toxic effect of the alcohol itself, dietary insufficiency and impaired absorption may play
important roles. Indeed, many alcoholic patients have a vitamin B1 or thiamine deficiency, 1 8 2 which
alone can cause similar clinical findings. The pathologic changes include reduced density of large and
small myelinated fibers, acute axonal degeneration and regeneration, 5 2 and secondary paranodal
demyelination involving the most distal segment.
Clinical symptoms usually appear insidiously over weeks or months, but sometimes more acutely over a
period of a few days. The initial sensory complaints consist of distal pain, paresthesias, and
dysesthesias, first in the legs and later in the arms. Burning sensations in the extremities resemble
those in diabetic neuropathy. Trophic changes such as plantar ulcers develop when patients subject
insensitive tissues to unusual amounts of trauma. 7 2 7 More advanced cases involve bilateral footdrop,
associated with distal muscular atrophy involving the extensors more than the flexors. Neuropathic
changes predominate in chronically weak and atrophic muscles. Sensory symptoms may respond to daily
administration of vitamin BI, but
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muscular atrophy tends to persist despite therapy.
Electrophysiologic evaluations demonstrate impaired function of small caliber motor fibers and large
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cutaneous sensory fibers. Despite the traditional emphasis on the role of conduction velocity, early
abnormalities consist of decreased amplitude of sensory nerve and compound muscle action potentials.
Thus, nerve conduction studies initially reveal either normal or only slightly reduced velocities in most
patients. 5 2 , 1 1 6 As in other axonal neuropathies, conduction velocity decreases in proportion to the loss of
evoked sensory and motor responses. 4 0
Conduction abnormalities may involve not only the distal but also the proximal segments of the nerve. 3 0 0
Assessments of sural nerve and late responses improve the diagnostic yield. 1 8 4 Electromyography reveals
fibrillation potentials and other neuropathic changes. Usually abnormalities involve the lower limbs
earlier and more prominently than the upper limbs, reflecting the lengthdependent degeneration of
axons. Other reported abnormalities include those seen in sympathetic sudomotor responses,
sympathetic skin responses, and cardiorespiratory reflexes, 5 9 9 as well as visual and brainstem auditory
evoked potentials. 1 3 1
Uremic Neuropathy
A variety of neuropathies result from the complex effect of renal failure on peripheral neurons, myelin,
and Schwann cells. 7 2 9 Uremic neuropathy often develops in patients with severe chronic renal failure or
in patients undergoing chronic hemodialysis. The use of neurotoxic drugs such as nitrofurantoin can
contribute to the nerve damage. Histologic findings comprise axonal degeneration, secondary segmental
demyelination, and, less frequently, segmental remyelination. 3 3 , 7 0 9 , 7 2 9 , 8 3 1
Clinical symptoms of neuropathy usually develop abruptly, with a sudden rise in vibratory threshold as
one of the early signs. The lower limbs tend to show earlier and more prominent disturbances than the
upper limbs. Some patients have restless legs as a presenting symptom. 8 3 1 After successful treatment
with hemodialysis, vibratory perception returns to normal, followed by improvement in other clinical
findings. A distal ischemic neuropathy has developed following the placement of bovine arteriovenous
shunts for chronic hemodialysis. 7 3 Proximal muscle weakness may also appear in uremic patients
receiving hemodialysis. 4 9 3 Patients may have paradoxical heat sensation in response to low temperature
stimulation. 9 2 5 Thermal threshold testing reveals only infrequent abnormalities in endstage renal
failure, showing little correlation with clinical and electrophysiologic evidence of polyneuropathy. These
findings indicate relative sparing of small diameter axons. 2 5
Patients with severe renal insufficiency often have motor and sensory conduction abnormalities in all
limbs, with greater deficits in the peroneal than the median nerve. 6 0 8 As a sensitive indicator of
neuropathy, facial nerve latency may rival the conduction studies of the peroneal, median, and ulnar
nerves. 5 8 0 Studies of late responses and sural nerve conduction also reveal a high degree of
abnormality. 4 In acute renal failure, the muscle action potential may show a marked reversible reduction
in amplitude, presumably as the result of conduction block. 9 9 The partly reversible acute uremic
neuropathy may show some demyelinating features simulating Guillain-Barr syndrome. 7 0 9 In chronic
renal failure, such diminution in size of the compound potentials signals axonal degeneration usually but
not always associated with fibrillation potentials. 7 5 Most uremic patients have an abnormal pattern shift
in visual evoked potentials and somatosensory potentials. 7 1 5 Electrophysiologic findings generally, but
not exactly, correlate with the clinical signs, levels of serum creatinine, and pathologic changes of the
peripheral nerve. 8 7 1 , 9 0 5 Mild electrical abnormalities sometimes herald clinical manifestations. 9 3 0
Conduction velocities decrease with the deterioration of signs and symptoms and increase with
improvement after dialysis or kidney transplantation, 1 8 3 , 6 0 9 , 6 2 7 , 8 8 4 but the question Still remains
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whether nerve conduction studies can monitor the adequacy of renal dialysis. 6 7 5
Neuropathies in Malignant Conditions

Malignant processes affect the peripheral nerve directly or indirectly. 1 6 9 , 6 8 0 , 8 1 1 Lymphomas and leukemias
may invade or infiltrate through hematogenous spread, 4 6 4 , 8 9 2 whereas nonlymphomatous solid tumors
may cause external compression. Occasionally a metastasis may involve the dorsal root ganglia. 4 0 3
Neuralgic amyotrophy may develop in association with radiation therapy for Hodgkin's disease. 5 3 3
Paraneoplastic neuropathies, as an autoimmune disorder, 2 1 1 result from the distant effects of

lymphoma, 1 6 6 , 4 0 7 bronchogenic carcinoma, 3 5 4 pancreatic carcinoma, 1 1 0 or, less commonly, tumors of the
ovary, testes, penis, stomach, or oral cavity. 6 1 1 Approximately one third of patients with malignancies
develop clinically latent neuropathies. 6 6 1 Patients with lung cancer have a slightly higher incidence.
Remote malignancies usually affect the dorsal root ganglia, but also occasionally the anterior horn cells.
Pathologic features include (1) neuronal degenerations with secondary peripheral or central axonal
changes; (2) demyelination reminicent of acute or chronic idiopathic polyneuritis 5 1 1 ; (3) microvasculitis
with active wallerian degeneration, causing mononeuritis multiplex 6 3 0 ; (4) perineuritis defined as
perineurial thickening and inflammation 7 9 4 ; and, possibly, (5) opportunistic neuropathic infection. Both
cytotoxic T cellmediated attack against neurons and humoral mechanisms play a role in paraneoplastic
subacute sensory neuronopathy. 8 9 3
Patients have clinical findings of sensory or motor deficits or, more commonly, mixed involvement.
Sensory motor neuropathy represents a group of heterogenous conditions with overlapping clinical and
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histologic features. 2 7 , 1 2 5 Occasional patients develop a pure motor neuropathy mimicking myasthenic
syndrome or polyradiculopathy seen in meningeal carcinomatosis. Systemic cancer may initially cause
the symptom of mental neuropathy causing numb chin 5 4 5 or intestinal pseudoobstruction. 5 0 4 Although
results are generally disappointing, some patients with anti-Hu-associated paraneoplastic sensory
neuropathy will respond to early aggressive immunotherapy. 6 2 8
Distinguishing between paraneoplastic and nonparaneoplastic sensory neuronopathies can tax the
clinician. Prominent neuropathic pain, neurologic dysfunction involving more than the peripheral sensory
system, or an increased cerebrospinal fluid protein should prompt a careful search for a cancer. 1 2 5
Subacute sensory neuropathy of oat cell carcinoma may result in severe sensory loss secondary to dorsal
root ganglionitis. 2 0 6 In one case, morphometric studies at autopsy showed preferential loss of large
diameter sensory nerve cell bodies, marked loss of large myelinated fibers in the dorsal root and sural
nerve, and almost total loss of myelinated fibers in the fasciculus gracilis. 6 3 2 Chronic idiopathic ataxic
neuropathy 1 8 5 denotes the same type of progressive sensory neuropathy seen without evidence of
cancer. 4 2 2
Quantitative sensory testing may uncover subclinical abnormalities involving both large and small
fibers. 5 1 0 The conduction studies reveal only mild slowing of sensory or motor fibers or both with
substantial reduction in amplitude of sensory nerve, 6 8 1 or muscle action potentials, or both.
Electromyography typically shows fibrillation potentials and highamplitude, long-duration motor unit
potentials in atrophic muscles. 6 6 1 Small, short-duration polyphasic motor unit potentials occasionally
seen in wasted proximal muscles probably result from neuropathic abnormalities of the intramuscular
axonal twigs. 4 7
Neuropathies Associated with Paraproteinemia

A number of studies have demonstrated a clear association between IgM and IgG 8 1 2 and, to a lesser
degree, IgA 7 7 2 monoclonal proteins and peripheral neuropathy. 3 1 1 , 5 6 5 Most affected patients have benign
monoclonal gammopathy, sometimes with a genetic predisposition. 3 9 9 Other syndromes occasionally
encountered include
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primary systemic amyloidosis, osteosclerotic myeloma, and, less frequently, osteolytic multiple myeloma,
Waldenstrom's macroglobulinemia, cryoglobulinemia, gamma heavy chain disease often associated with
hepatitis C infection, 2 9 , 1 9 1 Castleman's disease, or angiofollicular lymph node hyperplasia with
vasculopathy, papilledema, organomegaly, endocrinopathy, and paraproteinemia, 2 0 2 and the syndrome of
polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
(POEMS). 5 7 5 , 7 3 2 , 7 7 6 , 8 4 7 Table 25-1 summarizes the main clinical and laboratory features of these entities.
In multiple myeloma and macroglobulinemia, neuropathy may develop as a feature of the underlying
disorder or as the result of paraproteins. 8 1 7 , 8 5 7
Benign monoclonal gammopathy occurs in 10 percent of all patients with idiopathic peripheral
neuropathy. 2 0 7 , 4 2 6 Conversely, 30-70 percent of those with benign monoclonal gammopathy develop
chronic sensory motor neuropathy. The clinical features closely mimic those of chronic inflammatory
demyelinating polyradiculoneuropathy (CIDP) with progressive sensorimotor loss 7 1 , 9 3 , 1 6 2 , 6 1 2 , 7 7 1 and
occasional tremor. 6 6 5 The association with a central lesion, 4 9 9 although uncommon, includes cerebral
lymphoma. 2 3 7 Plasma exchange rapidly lowers the level of monoclonal antibody, with some recovery of
motor function. 6 3 4 , 7 6 0 Some patients also respond to highdose intravenous immunoglobulin
therapy 1 5 9 , 1 8 8 , 5 3 6 and immunosuppressive treatment. 2 4 1 , 6 1 5 The characteristic laboratory findings include
IgM and IgG, and less commonly, IgA gammopathy and a high level of cerebrospinal fluid protein.
Electrophysiologic and morphologic studies show evidence of demyelination, although axonal loss is a
major finding in a few. 7 8 4
This category also includes various polyneuropathy syndromes associated with antibodies either to
peripheral nerve myelin or myelinassociated glycoprotein (MAG) 3 9 , 1 3 4 , 5 0 1 , 6 1 1 , 8 6 1 , 8 8 9 or to sulfated
glucuronyl paragloboside (SGPG). 6 7 2 , 8 6 2 Slowly progressive sensory motor neuropathies of this type often
show disproportionate prolongation of distal motor latency as evidenced by increased residual latency
and decreased terminal latency indices (see Chapter 5-4), 4 1 4 showing a higher correlation with anti-MAG
than anti-SGPG titers. 8 4 2 A combined syndrome of gait ataxia and polyneuropathy seen in some of these
patients often improves after intravenous immunoglobulin or other immunosuppresive therapy. 6 7 0
Primary systemic amyloidosis or the lightchain type of amyloidosis affects multiple
Table 251 Main Features of Monoclonal Protein-Peripheral Neuropathy Syndromes
Sensory Autonomic CSF

Type of PN Topography Weakness Loss Loss Course Protein MNCV Pathology
Benign Distal, rarely ++ ++ + Chronic ++ Mild SD + AD
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monoclonal (IgG, proximal progressive slowing

IgA)
Benign Distal, ++ ++ 0 Chronic ++ Very

monoclonal symmetric progressive slow
gammopathy
(IgM)
Amyloidosis, light Distal, +/++ +++ ++ Chronic + Mild

chain type symmetric progressive slowing
Osteosclerotic Distal, +++ + 0 Chronic +++ Very SD(+AD)

myeloma symmetric progressive slow
Waldenstrom's Distal, ++ ++ 0 Chronic ++ Very SD(occ'l

macroglobulinemia symmetric progressive slow
MNCV = motor nerve conduction velocity; AD = axonal degeneration; SD = segmental demyelination; PN = peripheral neuropathy;
CSF = cerebrospinal fluid.
From Kelly,425 with permission.
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organ systems with symptoms similar to those of malignancy or collagen vascular disease. Patients with
amyloidosis, however, have plasma cell dyscrasias and amyloidogenic immunoglobulins. 2 6 0 , 3 0 2 , 8 4 4 Amyloid
accumulates in the flexor retinaculum, causing carpal tunnel syndrome. Diffuse peripheral neuropathy
develops as the result of metabolic or ischemic changes or direct infiltration by amyloid. 1 5 2 , 4 2 5 The
clinical features consist of a painful sensory and motor neuropathy, with prominent autonomic
dysfunction affecting multiple systems. Axonal degeneration predominates in small myelinated and
unmyelinated fibers, 8 3 5 usually sparing the large myelinated fibers. This accounts for the typical
dissociated sensory loss with predilection for pain and temperature sense with relative sparing of
vibratory and position sense, that is, the reverse of the findings in large fiber type diabetic neuropathy.
Electrophysiologic abnormalities include slight slowing of the motor nerve conduction velocity with mild
reduction in amplitude of the compound muscle action potential; absence of the sensory nerve action
potentials with distal stimulation of the ulnar, median, or sural nerve; and evidence of superimposed
compression of the median nerve at the wrist. Electromyography reveals evidence of denervation
diffusely but more conspicuously in the distal muscles of the leg. 4 2 9 An in vitro study of sural nerve
compound action potentials has shown a selective reduction in C and A delta potentials in familial
amyloid neuropathy. 2 2 4 This supports the view that amyloid neuropathy predominantly causes distal
axonal damage first in the sensory and then in the motor fibers.
Skeletal osteosclerotic lesions, although seen only in less than 3 percent of myeloma patients as a
whole, develop in at least 50 percent of those with myeloma neuropathy. 4 2 8 This type of myeloma
commonly affects younger patients and takes a benign clinical course although the patient often develops
demyelinating neuropathy that resembles CIDP. 2 0 5 Neuropathy may improve following surgery, radiation,
and chemotherapy. 7 1 6 Electrophysiologic and histologic evidence of prominent demyelination suggests an
immunologic effect of the monoclonal protein on a myelin antigen as a precipitating cause. 4 2 7 , 5 4 6
Intraneural injection of patient serum into rat sciatic nerve, however, produces no demyelinative
lesion. 4 2 8 Instead, the morphologic features suggest axonal attenuation or distal axonal degeneration
with secondary demyelination. 6 3 1
Patients with osteolytic multiple myeloma may have amyloid neuropathy much like the type seen in
systemic amyloidosis without multiple myeloma. These cases show, in addition to prominent distal axonal
loss and carpal tunnel syndrome, atypical features such as radiculopathy and mononeuritis multiplex. In
this condition, a peripheral neuropathy also develops without amyloidosis in 30-40 percent of cases,
based on electrophysiologic and histologic findings. 1 2 3 , 4 2 5 Diverse clinical and electrophysiologic features
resemble various subgroups of carcinomatous peripheral neuropathy. Sensorimotor types show distal
axonal degeneration and mild decrease in nerve conduction velocity. The patients with primary sensory
involvement characteristically have a loss of proprioception but few deficits in the motor system. 4 2 4
Those with primary motor abnormalities have features similar to CIDP, with prominent slowing of nerve
conduction velocities.
Patients with Waldenstrom's macroglobulinemia may develop a primarily demyelinating sensory motor
neuropathy of the type commonly associated with benign monoclonal gammopathy. 6 1 3 Occasional
patients, however, have axonal degeneration and amyloid infiltration, as in osteolytic multiple myeloma.
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Electrophysiologic studies typically reveal predominant segmental demyelination and, less frequently,
evidence of axonal changes as a major finding. 8 8 7
In cryoglobulinemia, 1 2 3 two types of neuropathy develop: (1) a mild distal neuropathy probably the
result of vasa nervorum microcirculation occlusion caused by intravascular deposits of cryoglobulins and
(2) a severe distal symmetric sensory motor neuropathy with necrotizing vasculitis. 2 8 6 Hepatitis C virus
may play a role in nonsystemic vasculitic mononeuropathy multiplex associated with
cryoglobulinemia. 2 8 , 4 3 2 , 7 4 7 Electromyography
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and sural nerve biopsy specimens show axonal degeneration with a preferential loss of large diameter
fibers, confirming a major role of ischemia in the pathogenesis 1 2 1 , 2 8 9 , 6 0 2 Treatment consists mainly of
plasmapheresis. 8 2 6 A reversible sensory autonomic neuropathy seen in cold agglutinin disease may have
a similar pathogenesis to those proposed for cryoglobulinemia. 8 3 9
Necrotizing Angiopathy
In necrotizing angiopathy, which is probably related to autoimmune hypersensitivity, patients have
systemic or nonsystemic vasculitic neuropathy. 2 1 5 , 6 3 6 The inflammatory process, possibly through
endothelial cell activation, 6 4 7 involves the small- and mediumsized arteries in multiple organ systems,
including the thoracic and abdominal viscera, the joints and muscles, and the nervous system. Necrosis
of the media gives rise to small aneurysms and thrombosis of the vessels, with palpable nodules along
the affected arteries. This type of neuropathy also occurs in association with known or suspected
connective tissue disease such as rheumatoid arthritis, systemic sclerosis, nonvasculitic, steroid
responsive mononeuritis muliplex, 5 1 5 and Sjgren's syndrome 3 5 2 , 6 3 6 or other multisystem diseases such
as Wegener's granulomatosis 4 0 0 , 6 1 0 and cryoglobulinemia with an IgM kappa M protein. 8 2 9
The clinical symptoms and signs, which may appear either abruptly or insidiously, consist of malaise,
fever, sweating, tachycardia, and abdominal and joint pain. Approximately one half of the patients
develop neuronal disturbances such as diffuse polyneuropathy and mononeuritis multiplex. Neuropathy
presumably results from ischemia caused by thrombosis of the nutrient arteries heavily infiltrated with
inflammatory cells. The disease may remit spontaneously despite a generally poor prognosis, with
survival of only a few months to a few years after the onset of clinical symptoms. In one series, 10 of 16
patients had features of mononeuritis multiplex, and the remaining 6 had a distal symmetric sensory
motor polyneuropathy. 4 4 8 In another study of 23 patients with giant cell arteritis, 11 had generalized
neuropathy, 9 had mononeuritis multiplex, and 3 had a mononeuropathy. 1 1 4 Nerve conduction studies
show slow velocity in proportion to reduced amplitude of the compound muscle and sensory potentials in
the affected limbs. A conduction block may result from subinfarctive nerve ischemia affecting the
segment outside the usual sites of compression mimicking a demyelinative neuropathy. 3 0 1 , 5 8 4 , 7 0 7 Serial
studies, however, usually demonstrate conversion of the electrophysiologic findings to those most
consistent with severe axonal loss. 5 5 1 Electromyography reveals spontaneous activities in atrophic
muscles as expected in acute or subacute axonal neuropathy. 8 1 , 1 7 5
Sarcoid Neuropathy
Patients with sarcoidosis develop distal sensory motor polyneuropathy as a rare complication. 6 0 3 Typical
neuropathies associated with this disorder include Gullain-Barre syndrome, mononeuritis multiplex,
lumbosacral plexopathy, and purely sensory neuropathy. 9 3 8 Histologic studies reveal granulomata or
inflammatory changes in the epineural and perineural spaces that lead to periangitis, panangitis, and
axonal degeneration. 6 2 5 Electrophysiologic abnormalities include reduced amplitude of the compound
sensory and muscle action potentials and mild slowing in conduction studies, 1 2 6 , 6 2 5 and prominent
fibrillation potentials and positive sharp waves in electromyography. In one case, morphologic studies
confirmed the electrodiagnostic impression of an acute axonal and demyelinating neuropathy. 6 0 3
Differential diagnosis should include rare nerve root involvement causing polyradiculopathy. 4 5 2
Sjgren's Syndrome
Patients with Sjgren's syndrome develop dryness of the eyes, mouth, and other mucous membranes.
The disease involves various anatomic structures such as
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joints, blood, internal organs, skin, muscle, and central and peripheral nervous systems. Subacute
sensory neuropathy may develop as a presenting symptom, 3 1 5 sometimes primarily affecting the
distribution of the trigeminal nerve. 5 3 2 Other forms include mononeuropathy multiplex, distal sensory
neuropathy, distal sensory motor neuropathy, and pure sensory neuropathy. 4 1 9 , 4 2 0 , 8 9 0 Electrophysiologic
and sural nerve biopsy studies reveal an axonal neuropathy in these cases. 6 7 3 In one series of 33
cases, 5 6 7 symmetric sensory motor polyneuropathy occurred most frequently, followed by symmetric
sensory neuropathy. Approximately one fourth of patients had superimosed autonomic neuropathy,
mononeuropathy, or cranial neuropathy. The symptoms, generally mild at the onset, slowly progress. 8 9 0
Nerve biopsy specimens may reveal evidence of necrotizing vasculitis, with axonal degeneration more
than demyelination. Some of the clinical and neurophysiologic findings suggest the involvement of the
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spinal ganglion and postganglionic sympathetic ganglion cells. 4 6 9
Other Neuropathies
Sensory-motor neuropathy may accompany some multisystem atrophy such as Shy-Drager syndrome, 2 7 9
and the syndrome of skin pigmentation, edema, and hepatosplenomegaly. 8 2 3 , 8 4 5 Patients with
hypothyroidism or hyperthyroidism may have sensory and motor conduction abnormalities
diffusely 5 0 , 4 5 5 , 6 7 6 or localized at the common sites of compression. 7 5 2 In systemic lupus erythematosus,
patients may have a predominantly motor or sensory demyelinating polyneuropathy as the presenting
feature. 5 5 3 , 6 3 9 , 6 4 0
The neuropathy associated with the hypereosinophilic syndrome develops at the onset of marked
eosinophilia. 2 9 5 , 4 3 0 , 8 9 9 It affects both the sensory and motor fibers with multifocal conduction
abnormalities and evidence of severe axonal degeneration. 2 1 0 The eosinophilia myalgia syndrome 4 6 6
develops in some patients taking preparations containing L-tryptophan, causing sensory motor
neuropathy characterized by segmental demyelination and distal axonal degeneration. 1 0 2 , 2 0 8 , 2 6 6 , 3 5 1
Migrant sensory neuritis of Wartenberg has a benign relapsing and remitting course. Movement of the
limbs induces a stretch, leading to pain and subsequent loss of sensation in the distribution of individual
cutaneous nerves. Stimulation of the affected nerves may elicit small or no sensory action potentials. 5 4 9
Patients with polymyalgia rheumatica with muscle aching, tenderness, and weakness may have not only
steroid-responsive myositis 9 2 but also peripheral neuropathies. 7 2 8 , 7 5 1
Meningococcal septicemia may cause a mixed sensory motor neuropathy with electrophysiologic findings
consistent with axonal degeneration. 7 0 4 Critically ill patients may develop a severe motor and sensory
polyneuropathy of unknown cause 7 4 , 9 3 7 and other neuromuscular diseases with prolonged ventilator
dependency. 7 9 9 Some investigators advocate the term critical illness neuropathy as a useful clinical
concept, 7 2 whereas others argue that the enormous complexity encountered in critical illness weakness
makes the implication of a neuropathy as the cause of syndrome untenable. 8 7 Histologic investigations of
muscle atrophy in two critically ill patients with generalized weakness revealed marked type I and type II
muscle fiber atrophy and only minor axonal degeneration of sural nerves and intramuscular nerve
fibers. 9 1 6 Limb compression during unattended coma may also cause multiple peripheral nerve injuries.
The unique combination of swollen limbs, pressure blisters, and myoglobinuria constitutes the
compartment syndromes. 7 6 4
The neuropathy associated with polycythemia vera involves large and small myelinated fibers with mild
slowing of motor and sensory conduction. 9 2 2 Distal axonal degeneration follows ischemia produced by
thromboembolic occlusion of a major proximal limb artery, 9 0 2 especially in patients at risk with uremia,
diabetes, 6 9 9 or peripheral arterial disease. 2 4 0 Multiple sclerosis occasionally accompanies hypertrophic
demyelinating neuropathy with typical nerve conduction changes. 6 7 9 , 7 5 8 Denervation of the rectal
sphincter characterizes multisystem atrophy, which resembles primary autonomic failure with an
autonomic neuropathy as a common feature. 6 9 1 Burn patients may
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have undiagnosed neuropathy. 5 4 0 Polyneuropathy may also result from lightning injury, 3 5 3 severe
hypothermia, 6 and graftversushost disease. 1 8
Other systemic disorders sometimes associated with mild polyneuropathy include Whipple's disease, 1 7 7
celiac sprue, 4 2 3 multiple symmetric lipomatosis, 5 9 6 acromegaly, 3 9 6 Crohn's disease, 3 7 5 Leigh's
disease, 1 5 4 , 3 9 2 xeroderma pigmentosum, 3 5 9 '417 cerebrotendinous xanthomatosis, 8 8 8 Bthalassemia, 6 4 8
hemophagocytosis syndrome, 3 6 7 sickle cell anemia, 7 6 3 juvenile Parkinson's disease, 8 2 0 tyrosinemia, 2 9 8
Machado-Joseph disease, 1 5 5 and multiple symmetric lipomatosis. 5 9 5
3 Inflammatory, Infective, And Autoimmune Neuropathies

Inflammatory, infective, and autoimmune neuropathies include a wide range of disorders, from Guillain-
Barr syndrome and related disorders 3 6 5 , 3 6 9 , 7 0 8 , 8 0 2 to diphtheria and leprosy as well as acquired
immunodeficiency syndrome (AIDS).
Guillain-Barr Syndrome
Although of unknown etiology, Guillain-Barr syndrome and related demyelinative neuropathies closely
resemble experimental allergic neuritis, 7 4 9 either by active immunization with extracts of peripheral
nerve 9 4 , 3 5 0 or by repeated transfer of P2 proteinreactive T cell lines. 4 9 0 Some patients with this
syndrome have human immunodeficiency virus (HIV), 1 6 4 herpes zoster virus, 6 4 1 or hepatitis B virus
infection. Other possibilities include Campylobacterje unienteritis, 2 3 8 , 3 2 0 , 3 4 9 , 4 7 4 , 7 5 9 , 9 3 4 Mycoplasma
infection with anti-Gal-C antibody, 4 7 6 and Cyctospora infection. 6 9 7 In most, however, repeated attempts
have failed to isolate infective agents. These findings support an autoimmune pathogenesis rather than
direct invasion of the nerve by infectious agents. 3 4 9
Serum and cerebrospinal fluid (CSF) anti-GM 1 antibodies may play a key role in the pathogenesis of
demyelination 6 8 6 , 7 7 7 as well as axonal degeneration. 3 9 3 The serotypic determinant of PEN 19 of
Campylobacter jejuni may aide in the production of anti-GM 1 antibody by a GM 1 like
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lipopolysaccharide. 9 3 2 In vitro demyelination by serum antibody from patients with Guillain-Barr

syndrome requires terminal complement complexes. 7 4 4 In one series, 4 9 the relative change in anti-GM 1
titers showed an inverse relationship with muscle performance. In another study, 7 5 7 circulating tumor
necrosis factor correlated with electrophysiologic abnormalities of demyelination.
An inflammatory demyelinative neuritis affects all levels of the peripheral nervous system, 4 1 6
occasionally with retrograde degeneration in the motor cells of the spinal cord or brainstem. In mild
cases, pathologic changes may consist of only slight edema of the nerves or roots with only minimal
inflammatory infiltrates. 7 1 0 , 7 5 0 In contrast, the fulminant syndrome may show universal inexcitability of
the peripheral nerves with axonal degeneration secondary to inflammation. 5 9 , 2 4 9 The segment of maximal
involvement varies from one patient to the next. This helps explain the diversity of clinical findings and
of conduction abnormalities in different cases. Guillain-Barr syndrome consists of a number of subtypes
showing different clinical and pathologic features. These include, in addition to the common acute
inflammatory demyelinating polyneuropathy (AIDP), Fisher syndrome, acute motor sensory axonal
neuropathy (AMSAN), and acute motor axonal neuropathy (AMAN), or acute flaccid paralysis in China. A
substantial proportion of the patients initially diagnosed with Guillain-Barr syndrome may turn out to
have a neuropathy with another etiology, especially heavy metal intoxication. 2 5 0
Although the clinical and pathologic findings vary even among patients with the classical syndrome,
certain diagnostic criteria have emerged. 3 4 , 3 6 5 In about two thirds of the cases, neurologic symptoms
follow a mild, transient infectious process of either the respiratory system or, less commonly, the
gastrointestinal system. Some patients seem to have other precipitating events such as polio, 4 4 7 rabies
vaccine treatment, 1 0 5 and allogeneic bone
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marrow transplantation. 8 9 7 The first symptoms of neuropathy usually appear in about 1-2 weeks, when
the infection has resolved. Occasionally, the disease takes the form of
encephalomyeloradiculoneuropathy with progressive central nervous system disease, 5 9 2 bilateral
deafness, 6 0 1 or severe sensory motor neuropathy. 8 9 8 Rarely, seizures and other signs of cerebral
involvement may signal the onset of illness in children. 9 0 6 Weakness initially involves the lower limb,
sometimes rapidly progressing to the upper limb and the face within a few days. Paralysis of proximal
muscles and facial diplegia contrast with the distal weakness characteristic of other forms of neuropathy.
Respiratory problems develop in approximately one half of the patients. 7 1 1 Occasional patients have an
acute, severe, and progressive illness with quadriplegia in 2-5 days, requiring mechanical
ventilation. 2 7 2 , 5 7 4 In addition to these features, unfavorable predictive factors include old age, preceding
infection, bulbar paralysis, and onset of paralysis in proximal muscles. 2 9 7
Other early signs include diminished or lost muscle stretch reflexes, miminum sensory loss despite
painful distal paresthesias, and, occasionally, myokymia and even involuntary contraction resulting from
continuous motor unit discharges. 6 8 2 Careful testing usually reveals deficiencies in vibratory sense, two-
point discrimination, and pain perception. The autonomic dysfunction mainly results from axonal
degeneration of the vagus and splanchnic nerves as seen in experimental allergic neuritis 5 6 3 involving
both sympathetic and parasympathetic fibers of the cardiovascular, sudomotor, gastrointestinal, and
other systems. 9 3 6 Some patients have transient elevation or fluctuation of blood pressure and heart rate
as the result of sympathetic hyperactivity. 2 6 1 , 7 1 2 The CSF typically contains high protein levels and no
cells with the exception of some lymphocytes in occasional cases.
The disease follows an acute or subacute course with usual progression up to 6 weeks after onset. 3 7 4 The
symptoms and signs then plateau for a variable period before gradually improving. Occasionally acute
relapses occur after long asymptomatic intervals. 8 , 9 0 1 These patients have a high incidence of an
antecedent illness, lack an apparent response to immunosuppressive therapy, and have a normal CSF
protein level. 3 1 4 Although some patients improve dramatically following corticosteroid therapy, 6 2 4
prednisone may adversely affect the eventual outcome of the disease. 3 7 3 Plasma exchange 3 5 , 3 9 7 can be
beneficial but not universally. 5 6 8 Some patients show antibody rebound after therapy, with deterioration
of nerve conduction studies. 7 0 5 , 7 2 0 Treatment with intravenous immunoglobulin may 3 0 6 , 3 4 9 , 8 6 8 , 8 6 9 or may
not be beneficial. 1 1 8
The time course of recovery depends on the extent of demyelination and, more importantly, axonal
degeneration. In one series, severe residual deficits developed in the patients with highly elevated anti-
GM 1 activity 8 6 3 and in another in those with high IgG antibody titers against GD 1 a ganglioside. 9 3 3 '935
Some patients have severe axonal loss without inflammation or demyelination 2 4 6 , 2 4 7 , 6 9 4 , 8 6 6 , 9 3 1 or
secondary to demyelination. 5 7 , 1 7 3 , 2 4 9 Such patients may not regain motor function for 1-2 years. Although
specific treatment has shortened the duration of mechanical ventilation, elderly patients with pre
existing pulmonary disease tend to require tracheostomy. 4 9 1 In some patients, impaired joint mobility
becomes a major disability despite an improving neurologic status. 7 9 5
Electrodiagnosis plays a key role in the evaluation. 1 0 , 1 4 , 1 6 1 , 7 1 3 In advanced stages of disease, nerve
conduction studies usually show velocities reduced by more than 30-40 percent from the normal mean
value and abnormal temporal dispersion of the compound muscle action potential (see Figs. 5-8A,B). In
milder forms, studies may reveal less dramatic changes because initial weakness commonly results from
proximal conduction block without distal abnormalities. 9 8 Indeed, 15-20 percent of cases have entirely
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normal nerve conduction studies distally during the first 1-2 weeks. 2 3 6 , 4 4 3 Thus, normal conventional
conduction studies by no means precludes the diagnosis. 4 8 3 In fact, the initial absence and later delay of
the F-wave with normal distal conduction (see Figs. 18-6 and 18-10 and Table 18-4) characterizes
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the typical pattern of abnormalities, indicating vulnerability of the most proximal, possibly radicular
portions of the motor fibers, with little changes along the main nerve trunk at the onset of
illness. 2 9 9 , 4 3 9 , 4 4 3 , 4 4 6 , 5 5 9 As in any neuropathy, the early changes may also selectively involve the common
sites of nerve compression 9 8 , 4 8 0 , 4 8 3 and the most terminal segment, presumably reflecting the longest
distance from the cell body. Immunemediated attacks on the axolemma of motor fibers may also give
rise to rapidly resolving conduction slowing and conduction block in the absence of demyelination. 4 8 1
Early and severe demyelination with secondary axonal damage may mimic acute motor axonal variant
clinically and electrophysiologically because of inexcitability of motor nerves. 4 1 1 , 5 4 4
Despite the clinical pictures of predominantly motor involvement, sensory or mixed nerve conduction
studies 5 2 0 show distinct, albeit milder, abnormalities of the median and ulnar nerves. Interestingly, the
disease tends to spare the sural nerve sensory action potential, often regarded as one of the first
affected in other neuropathies. 5 9 0 Quantitative thermal threshold measurements may uncover early
abnormalities at small nerve fibers. 8 2 7 Phrenic nerve conduction time may provide a sensitive measure in
predicting impending ventilatory failure. 3 1 2 Studies of the blink reflex frequently reveal conduction
abnormalities as expected from clinical facial palsy (see Figs. 17-4 and 17-12 and Tables 17-1 and 17-2).
Although less sensitive than F-wave studies, 6 3 7 somatosensory evoked potentials to median nerve
stimulation may demonstrate a proximal conduction delay between Erb's point and the cervical cord in
patients with normal sensory conduction distal to Erb's point during the first few weeks of onset. 9 8 , 2 9 9
Spontaneous activities include facial or limb myokymic discharges (see Fig. 1412B), which may appear
early, sometimes persisting during the course of illness, 5 4 7 and, rarely, continuous motor unit
discharges, or neuromyotonia. 6 8 2 Otherwise, electromyography usually shows only a reduced interference
pattern indicating neurapraxia without axonal degeneration. Occasional patients with typical clinical
features, however, may have a primarily axonal neuropathy and prominent denervation first detectable
2-3 weeks after onset 2 4 7 , 5 5 9
Sequential conduction studies show great variability among different patients and even from one nerve
to another in the same patient. 4 4 2 Relatively common patterns of conduction failure include a length
dependent and uniform reduction of compound muscle action potentials presumably based on a random
distribution of lesions. 8 6 7 Reversible proximal conduction block often underlies rapid recovery. 6 2 In
contrast, reduction in amplitude of compound muscle action potentials with distal stimulation generally
suggests axonal degeneration, especially when accompanied by normal conduction velocities. 1 6 5 , 1 8 1 , 3 1 9 , 5 7 3
Here, functional recovery depends on axonal regeneration, which takes considerably longer than
remyelination. Very small distally evoked potentials, however, may also result from primary
demyelination of terminal branches. 2 7 2 , 3 3 2 Thus, this finding does not necessarily imply a poor prognosis,
especially in children. 9 2 7 After treatment, conduction studies may or may not revert toward normal
values. 8 4 0 The nerve conduction velocity often becomes slower while the patient begins to improve,
demonstrating again the lack of a strong correlation between clinical and electrophysiologic assessments.
Miller Fisher Syndrome

The Miller Fisher syndrome 2 5 9 consists of ataxic gait, absence of muscle stretch reflexes and
ophthalmoplegia. Despite immunologic peculiarities of this subgroup, 5 3 4 as evidenced by its association
with serum antibodies to GQ 1 b ganglioside, 1 4 0 , 1 4 1 , 3 9 1 , 4 7 7 , 4 7 8 , 9 0 7 it probably constitutes a cluster within the
overlapping spectrums of Guillain-Barr syndrome. 8 2 5 One atypical patient with this syndrome had
abnormal pupils and normal eye movements; 9 0 4 another patient had a late central demyelination. 2 5 6
Patients with acute ataxic neuropathy, which resembles this syndrome, had severe sensory loss, no
motor deficits, and a poor prognosis. 8 2 1
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Antibody against QD 1 b may play a role in the pathogenesis of sensory ataxic neuropathy. 4 7 9 , 5 7 8 , 6 2 0
Electrophysiologic studies usually show characteristics of an axonal neuropathy or neuronopathy with

prominent sensory nerve changes in the limbs and motor damage in the cranial nerves. 2 6 9 The findings in
one series included normal distal motor nerve conduction velocities, F-wave latencies, and blink reflex
and abnormal sensory action potentials. 7 4 3 Serial studies in such a case, however, may show a time
course of conduction changes similar to those in Guillain-Barr syndrome. 3 9 5 , 8 5 2 Electromyography
usually reveals only slight abnormalities in the limbs and evidence of facial denervation.
Immunoabsorption plasmapheresis, while improving ophthalmoplegia, may not prevent facial palsy,
possibly because it fails to remove responsible antibodies. 1 4 2
Chronic Inflammatory Demyelinating Polyneuropathy

Apart from its chronicity following axonal changes, the disease may continue to worsen with persistent
evidence of ongoing demyelination. 4 4 , 5 5 5 This variety, referred to as chronic inflammatory demyelinative
polyradiculopathy (CIDP), has progressive or relapsing, usually motor and sensory, but rarely only motor
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or sensory, dysfunction of a peripheral nature involving more than one limb, developing over at least 2
months. 2 0 Other clinical features include hyporeflexia or areflexia, usually involving all four limbs. The
disease may follow a progressive course over several years with severe generalized disability, 2 1 4 , 6 8 4 or
affect only upper limb. 3 1 0 , 8 3 4 Although rare, focal neuropathy may precede the onset by several years or
asymmetrical polyneuropathy may show a stepwise progressive course. 8 8 1 , 8 8 6 A chronic demyelinating
neuropathy may accompany a relapsing multifocal central nervous system disorder whose clinical
features resemble multiple sclerosis. 5 6 9 , 5 9 3 , 8 3 8 In these cases, electrophysiologic studies reveal a slowing
of peripheral conduction velocity as well as an increased central conduction time. The occurrence of both
peripheral and central demyelination resembles chronic relapsing experimental allergic
encephalomyelitis and neuritis. Chronic motor axonal polyneuropathy (CMAN) may constitutes a variant
of CIDP. 1 4 8 , 3 0 8 , 4 1 1 , 8 5 3
Other possible features include subclinical central nervous system involvement, 6 3 3 , 6 4 2 dropped head
syndrome, 3 6 4 dysautonomia, 3 8 0 and pure sensory presentation 6 2 9 , 7 7 3 labeled chronic sensory
demyelinating neuropathy. 6 1 The risk of relapse increases during pregnancy. 5 5 4 Familial occurrence may
indicate a genetic predisposition. 2 7 4 , 3 7 8 Steroidresponsive hereditary sensory neuropathies may imply
superimposed acquired demyelination. 6 7 , 2 3 0 The clinical features in children may mimic a genetically
determined disorder. 7 8 1 Compared with adults, children tend to have a more precipitous onset, a higher
incidence of gait abnormalities, and greater neurologic deficits. 7 7 4 , 7 7 5
Nerve conduction studies reveal evidence of diffuse demyelination with characteristics quite similar to
those of Gullain-Barr syndrome, except for chronicity. 4 4 1 Other electrophysiologic abnormalities include
an increase in fiber density 2 8 3 and macromotor unit potential, and myokymic and continuous motor unit
discharge. 4 7 5 , 5 7 0 The CSF cell count is less than 10/mm3 unless the patient is HIV seropositive. Magnetic
resonance imaging may show abnormal enhancement reflecting inflammation. 1 7 2 Nerve root
hypertrophy 1 9 4 , 5 4 8 , 5 8 1 may cause lumbar stenosis. 3 0 5 Nerve biopsy reveals unequivocal pathologic
evidence of demyelination and remyelination by either electron microscopy or teased fiber studies.
Prednisone causes a small but statistically significant improvement over no treatment. 2 2 8 , 2 4 6 Plasma
exchange is a useful therapy, especially in cases with features of demyelination rather than axonal
degeneration. 2 6 7 , 3 0 7 , 6 7 7 , 8 9 1 Additional modes of therapy include cyclosporin, 4 2 , 3 6 3
immunoglobulin, 2 2 7 , 3 6 1 , 8 2 2 , 8 7 0 , 8 7 2 , 8 8 2 and interferon 2 a . 3 0 9 , 7 2 4
Successful treatment with plasma exchange suggests a role for pathogenic humoral factors. 3 2 8 Systemic
passive transfer of immunoglobulin is known to cause demyelinative disease in monkeys with substantial
reduction of conduction velocity. 3 5 7
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Antimyelinassociated glycoprotein antibodies may develop later during the course of the disease. 8 5 9 The
GM 1 and GM 3 autoantibodies may play a role in the pathogenesis of CIDP in systemic lupus
erythematosus. 7 7 8 Patients may have immunoglobulin and complement deposits in the nerve. 1 8 7
Multifocal Motor Neuropathy with Conduction Block

As a unique variant of CIDP, multifocal motor neuropathy (MMN), 6 5 4 , 6 5 6 , 6 7 1 is a potentially treatable
condition that needs to be distinguished from amyotrophic lateral sclerosis (ALS) and other motor neuron
syndromes. Affected patients develop chronic asymmetric predominantly motor neuropthy with multifocal
conduction delay and persistent conduction block. 4 6 1 , 8 0 9 , 8 1 4 , 8 6 4 Although MMN typically causes distal upper
limb weakness and atrophy, proximal muscles, biceps brachii in particular, may show hypertrophy
possibly associated with continuous motor unit activity. 6 3 5 Similar to earlier reported cases with sensory
and motor involvements, 2 9 6 , 5 0 3 , 7 3 9 the longlasting conduction block suggests chronic demyelination as
the pathologic basis. Patients often have normal or occasionally even increased stretch reflexes 4 0 9 with a
normal or only slightly elevated CSF protein (Table 25-2). Some patients develop cranial nerve
involvement 4 1 3 , 6 8 5 others, central demyelination. 5 0 6 , 6 6 7 These features make it difficult to diagnose the
condition solely on the basis of clinical examination. 4 4 0
Conduction blocks typically involve unusual sites such as the median nerve in the forearm or brachial
plexus 4 1 2 rather than the common sites of compression seen in multiple entrapment neuropathies. 6 5 Most
patients have selective involvement of motor fibers with normal sensory conduction through the sites of
motor conduction block. Both motor conduction block and abnormally increased threshold probably
reflect a chronic focal demyelinating lesion that for yet undetermined reasons becomes persistent
without repair. 4 0 8 , 4 4 1 , 9 2 3 Some patients with features indistinguishable from ALS have multifocal motor
nerve conduction abnormalities. 5 , 9 1 4 In one series, 17 of 169 patients clinically diagnosed as having
motor neuron disease had some abnormalities in motor nerve studies, including 10 with conduction
block. 4 8 8 Demonstration of motor conduction block at multiple sites differentiates this potentially
treatable clinical entity from the small subgroup of ALS patients with only lower motor neuron
involvement. 4 8 5
Electrophysiologic studies must confirm the diagnosis before therapeutic trials are initiated with, for
example, immunosuppressants such as cyclophosphamide. 1 3 6 Several authors have documented
successful treatment with intravenous immunoglobulin. 4 1 3 , 4 6 1 , 6 5 3 , 6 5 6 , 6 5 7 , 6 7 1 , 8 6 4 Outcomes of therapy with
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either immunosuppressants or, immunoglobulin, however, vary considerably among different reported
cases. 1 8 6 Some patients improve
Table 252 Characteristics of MMN and CIDP
MMN CIDP
Pure motor manifestation Frequen Rare
Multiple mononeuropathy Yes No
Remission and exacerbation No Yes
Generalized areflexia No Yes
CSF protein level Often normal Elevated
Sites involved in conduction block Forearm brachial plexus Entrapment sites, root
Elevated Anti-GMl antibody Frequent Rare
Choice of therapy Immunosuppressants, immunoglobulin Steroids, plasma exchange
CIDP = chronic inflammatory demyelinating polyneuropathy; CSF = cerebrospinal fluid; MMN = multifocal motor
neuropathy.
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but do not return to normal, others stabilize, some require long-term therapy, and still others become
refractory to any from of treatment. Most studies suggest better results with cyclophosphamide or
human immunoglobulin therapy 1 3 5 , 6 1 4 than with prednisone or plasmapheresis.
In our series, 4 1 2 , 4 1 3 two patients with MMN had focal conduction block involving motor but not sensory
fibers at the site of nerve swelling (see Fig. 716A,B). A nerve biopsy taken adjacent to the enlargement
in one patient revealed subperineurial edema and slight thickening of the perineurium under lowpower
light micrographs. 4 1 2 The perivascular area at the center contained scattered largediameter axons
almost devoid of myelin or with very thin myelin. These thinly myelinated axons usually had small onion
bulbs. The presence of cytoplasmic processes covered with basement membrane suggested their
Schwann cell origin. A nerve biopsy specimen from another patient also revealed a perivascular area
containing scattered demyelinated axons surrounded by small onion bulbs. Morphometric studies with
highpower light micrographs showed a fiber density of 6458 fibers/mm 2 compared with 7906 fibers/mm 2
in the control. Axonal diameter and myelin thickness had a linear relationship in the normal subjects. In
contrast, the patient had numerous largediameter axons with thinner myelin, although some normally
myelinated large axons remained.
The underlying pathogenic mechanism centers on elevated titers of anti-GM 1 antibodies found in a wide
variety of neuromuscular conditions, 4 8 2 but more commonly in some lower motor neuron disorders and in
MMN. 4 5 6 , 6 6 9 Antibodies may have a predilection for the GM 1 component of motor fibers, which have a
longer carbon chain than sensory fibers. 6 2 2 Autoantibodies may exert their effect, in part, by binding to
GM 1 on the surface of motor neurons. 1 6 0 Anti-GM 1 antibodies may 7 3 8 or may not 3 5 1 , 3 6 2 , 4 1 1 , 6 4 9 cause motor
dysfunction by binding to the nodal and paranodal regions. Sera of patients with MMN but not with
progressive spinal muscular atrophy induced conduction block in rat tibial nerves despite a similar
elevation of anti-GM 1 titers in both categories. 8 5 4 These antibodies however, may not have a causal
relationship with MMN, as evidenced by many patients without raised levels. 4 8 7 , 6 5 2 Surfacebound
antibodies directed against a major axoplasmic antigen may be interfering with remyelination rather than
causing demyelination. 4 0 8 , 4 1 1 In some cases, nerve ischemia may play a role in the pathogenesis. 6 1 9
In an extraordinary case, 7 3 8 a patient had received a duck embryo rabies vaccine 3 months before the
onset of her motor neuron disorder. She had multifocal conduction block, elevated levels of anti-GM 1 IgM
antibodies, and deposits of IgM at nodes of Ranvier. Aside from attacking motor neurons guided by the
abundant GM 1 on the cell surface, anti-GM 1 antibodies may cause conduction block in peripheral nerves
by binding to the nodes of Ranvier. An autopsy study in another patient showed findings consistent with
both ALS and MMN. 8 8 3 It is necessary to clarify the exact pathogenesis underlying these findings to
properly classify the motor neuron disease and MMN.
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Acute Motor Axonal Neuropathy in China

Annual summer epidemics of acute onset flaccid paralysis occur in northern China. Based on a historical
analysis of more than 3200 patients, distinctive features include a high incidence in children and young
adults residing in rural areas. Patients develop rapidly progressive ascending tetraparesis often with
respiratory failure without fever, systemic illness, or sensory involvement followed usually by a
satisfactory recovery. 2 8 4 , 3 1 7 , 3 1 8 , 5 5 7 , 9 1 8 The CSF shows no cells with an elevated protein content in the
second or third week of illness. Electrodiagnostic studies show reduced compound muscle action potential
amplitudes, normal motor distal latencies and limb conduction velocities, and normal sensory nerve
action potentials. When elicitable, F waves also fall within the normal range in latency. Autopsy studies
have shown wallerianlike degeneration of motor fibers. Thus, despite its inclusion as a variant of
Gullain-Barr syndrome, this acute motor axonal neuropathy (AMAN), mostly seen in China but
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possibly elsewhere, probably constitutes a distinctive entity. 3 8 9 A similar relationship exists between
CIDP and its presumed variant steroidsensitive chronic motor axonal neuropathy (CMAN). 8 5 3
Diphtheritic Neuropathy
Prophylactic immunization and early use of immune sera and antibiotics in infected patients have
drastically lowered the incidence of diphtheritic polyneuropathy, which occurs in about 20 percent of
patients. The exotoxin of Corynebacterium diphtheriae becomes fixed to the nerve and produces
segmental demyelination after several weeks. Local paralysis of the palatal muscles may immediately
follow an infection of the throat. Neuropathy may also develop in adults after contracting cutaneous
diphtheria, which still prevails in the tropics.
The clinical signs resemble those of Guillian-Barr syndrome. 1 7 0 The symptoms typically develop 2-4
weeks after the initial infection. Patients have a high incidence of lower cranial nerve dysfunction, most
notably palatal and pharyngolaryngoesophageal weakness. Blurring of vision results from paralysis of
accommodation. Other autonomic abnormalities include cardiac vagal dysfunction. 3 7 6 The involvement of
sensory and motor nerves causes paresthesias and weakness of the affected limbs. A rapidly descending
paralysis may lead to respiratory problems. The primary pathologic change consists of segmental
demyelination involving the sensory and motor fibers. 7 9 1 Conduction abnormalities usually begin a few
weeks after the onset of neurologic symptoms and peak after clinical recovery has already begun. 4 7 2 F-
wave studies also help establish serial changes in motor conduction. 2 9 1
Leprosy
An acidfast bacillus, Mycobacterium leprae, transmits leprosy, a chronic infectious disease, by close and
prolonged contact. Although rare in the United States, the disease still prevails in Africa, India, and
South and Central America. The organism seems to have a predilection for great auricular, ulnar, radial,
peroneal, facial, and trigeminal nerves. Of the two clinical forms, the lepromatous, or neural, type
causes extensive and widespread granulomatous infiltration of the skin, leading to characteristic
disfiguration. The diffuse sensory neuropathy seen in this variety results from direct invasion of the
nerve trunks by the bacillus. The thickened perineurium by an overgrowth of connective tissue
compresses the myelin sheath and the axons. In the other type, the tuberculoid form, more focal
involvement of the skin causes patches of the depigmented, maculoanesthetic areas. Here, swelling of
the nerves does not necessarily imply direct invasion by the organisms. The two types of clinical
presentation commonly overlap without clear separation, giving rise to an intermediate or mixed form.
Nerve biopsy material taken from sites remote from skin lesions reveals subperineurial edema and
various amounts of loss of myelinated and unmyelinated fibers. Similarities in some of the pathologic
changes observed in the two types of leprosy suggest a common mechanism of nerve damage in the
early stages. 7 6 2 Teased fiber studies in each leprosy type also reveal paranodal demyelination affecting
successive internodes. 3 9 4
Clinical features suggest mononeuritis multiplex or slowly progressive diffuse polyneuropathy. Common
manifestations include facial palsy involving the upper half of the face, wristdrop, footdrop, and claw
hands. Neural leprosy may begin with a small erythematous macule that soon enlarges, forming
anesthetic depigmented areas. The loss of pain and temperature sensation causes ulcerated necrosis of
the skin. Palpation of the affected nerve reveals characteristic fusiform swelling caused by an infective
granulomatous process. Electrophysiologic abnormalities consist of moderately to markedly slowed motor
and sensory conduction, not only across enlarged segments 5 2 7 , 8 1 8 but also along the unpalpable
portions. 5 6 2 In one series, radial nerve sensory abnormalities correlated best with the clinical findings, 7 5 3
whereas in another ulnar sensory studies revealed more prominent changes. 9 7 Electromyography
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reveals denervation in the atrophic muscles. The denervated muscle shows histopathologic changes of
fascicular atrophy and inflammatory nodules.
Acquired Immunodeficiency Syndrome

Patients with acquired immunodeficiency syndrome (AIDS) develop various types of
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neuropathy 4 3 , 2 9 3 , 3 0 4 , 3 2 4 , 3 3 1 , 3 7 2 , 9 1 3 as evidenced by nerve conduction studies. 1 8 0 , 2 7 1 , 4 8 4 , 7 8 5 In this entity,

cellmediated tissue destruction results from human immunodeficiency virus (HIV) infection and serves
as the pathogenetic mechanism of AIDS neuropathy. 1 9 3 Peripheral neuropathy may complicate all stages
of HIV infection. 1 3 7 , 1 6 3 , 4 8 6 , 5 0 0 , 5 7 2 Acute inflammatory demyelinating polyneuropathy, 6 8 7 sensory and
sympathetic ganglia neuritis, 2 4 2 and acute cranial nerve palsy all may occur 2-3 weeks after acute HIV
infection, sometimes in otherwise asymptomatic patients. Cytomegalovirus, a common pathogen in AIDS,
also causes a wide spectrum of peripheral nervous system disorders, including multifocal demyelinative
polyneuropathy. 8 5 , 5 8 8 Neuropathy is also one of the most common neurologic manifestations in AIDS-
related complex, affecting as many as 20 percent of patients.
In contrast to the autoimmune basis of demyelinative neuropathy, 8 1 0 less clearly established

pathogenetic mechanisms of distal symmetric polyneuropathy include infections, toxins, and nutritional
causes. Polyradiculopathy most likely results from infections with such agents as cytomegalovirus and
herpes simplex virus. These cause a severe selective destruction of the motor neurons of ventral spinal
roots and motor cranial nerves. 5 1 , 2 3 4 Zidovudine may induce mitochondrical myopathy but causes no
clear neurotoxicity. 4 9 7 Electromyography reveals severe diffuse denervation distally, despite only mildly
slowed nerve conduction velocities. Multifocal or distal symmetric inflammatory neuropathy may herald
the onset of AIDS in some homosexual men with lymphadenopathy. 5 0 8 , 5 4 3 These patients may have
reduced sural nerve action potentials as the sole electrophysiologic abnormality. 7 8 7
Other Neuropathies
Subacute sensory neuropathy is a rare complication of Epstein-Bar virus infection. 7 1 9 Herpes zoster may
cause a painful neuropathy in addition to the more common postherpetic neuralgia. 5 8 6 , 5 8 7 Hepatitis B
viral infection, albeit rarely, may cause mononeuritis multiplex during acute stages. 1 5 3 , 3 8 1 In paralytic
rabies, vascular and inflammatory changes predominate in the central nervous system but peripheral
nerves may show segmental demyelination, remyelination, and wallerian degeneration with variable
axonal loss. 1 4 6 Electrophysiologic abnormalities include slowing of motor and, to a lesser extent, sensory
conduction velocities, and reduced numbers of motor unit potentials and fibrillation potentials. 8 1 6
Demyelinating neuropathy may occur as a rare manifestation of Creutzfeldt-Jakob disease. 6 0 4
A tick (Ixodes) bite may result in meningoradiculoneuritis with electromyographic evidence of

denervation, prolongation of distal motor latency, and low sensory amplitude, suggesting axonal
degeneration. 8 5 8 Lyme borreliosis causes a severe, predominantly axonal polyradiculoneuropathy
typically with cranial neuropathy and lymphocytic meningitis. 3 3 5 , 5 1 6 , 5 2 8 , 7 4 6 The syndrome of acute sensory
and autonomic neuropathy often show a focal onset, suggesting an immunemediated or vascular process
at the level of the posterior root or the dorsal root ganglion. 6 6 2 , 9 1 0 Other infective diseases occasionally
associated with neuropathy include rickettsial disease, 3 3 6 Chagas' disease, trypanosomiasis, 7 6 7 and other
types of insect and spider stings. 1 7 1
4 Metabolic And Toxic Neuropathies

Metabolic neuropathies consist of two groups, those representing nutritional disturbances and those
resulting from toxic causes. Neuropathies attributable to
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a specific nutritional deficiency include beriberi, pellagra, and pernicious anemia. Toxic neuropathies
develop after the administration of various drugs or the exposure to chemical substances such as lead or
arsenic. Many neuropathies associated with general medical conditions also belong to this broad
category.
Nutritional Neuropathies
Children with insufficient protein or calorie intake suffer from retarded myelination or segmental
demyelination. 1 4 7 They have abnormalities of motor and sensory nerve conduction related to the severity
of the malnutrition. Severe malabsorption from blind loop syndrome also causes vitamin E
deficiency. 8 9 , 8 9 6 Alcoholic and paraneoplastic neuropathies result, at least in part, from inadequate food
and vitamin intake, although some toxins may also interfere with the metabolism of the nerves. 1 8 2 In
primary biliary cirrhosis, a sensory neuropathy develops from poor nutrition, xanthomatous infiltrates, or
immunologic abnormalities. 1 3 3
Diets deficient in vitamins and other nutritional factors play a major role in the polyneuropathy
associated with beriberi, pellagra, pernicious anemia, dysentery, and cachexia. 1 9 Beriberi, or thiamine
deficiency, causes signs and symptoms similar to those of alcoholic polyneuropathy. 3 6 6 They consist of
pain, paresthesias, distal sensory loss and weakness, and absent stretch reflexes. A similar neuropathy
may develop during intended weight reduction 7 9 6 or anorexia nervosa. 5 2 5 Histologic studies reveal
conspicuous axonal degeneration and less prominent demyelination. Pellagra, another deficiency disease
involving the vitamin B 1 complex, often affects malnourished patients with chronic alcoholism. The
clinical features consist of gastrointestinal symptoms, skin eruptions, and disorders of the peripheral and
central nervous systems. Neuropathic characteristics include paresthesias, loss of distal sensation,
tenderness of the nerve trunks, hyporeflexia, and mild paralysis. Isolated vitamin E deficiency, in the
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absence of lipid malabsorption, may cause ataxia and peripheral neuropathy. 3 8 8 Peripheral neuropathy
may also develop from a serum proteinase inhibitor deficiency 2 6 4 and hypophosphatemia as a rare
postoperative complication. 7 6 8
Pernicious anemia results from a deficiency of intrinsic factors in gastrointestinal secretions that mediate
absorption of vitamin B 1 2 Pathologic changes primarily involve the dorsal and lateral funiculi of the spinal
cord, thus the name combined system disease. The peripheral nerves also show fragmentation of myelin
sheaths and degeneration of axons. 4 5 7 The presenting clinical symptoms consist of paresthesias,
dysesthesias, and loss of vibration and position sense. The patients commonly have spastic paraparesis
during the early stages, followed by areflexia as the disease progresses. Somatosensory evoked
potentials show marked abnormalities in the peroneal nerve and milder changes in the median nerve, in
addition to peripheral conduction changes consistent with sensory motor axonopathy, 2 5 7 , 6 9 5 or rarely
demyelinating neuropathy. 9 Most untreated patients have reduced conduction velocity in part because of
a thiamine deficiency. 1 6 7 Patients with prominent axonal degeneration have diffuse spontaneous
discharges detected electromyographically but nearly normal motor nerve conduction velocities. 4 5 7
Appropriate treatment arrests the progression of neuropathy, but residual neurologic abnormalities
persist. 5 5 2
Toxic Neuropathies
Toxic neuropathies may have three presumed sites of cellular involvement: (1) neuronopathy affecting
cell bodies, especially those of the dorsal root ganglion; (2) myelinopathy or Schwannopathy with
primary segmental demyelination; and (3) distal axonopathy causing dyingback axonal degeneration. Of
these, the first two types include rare acute sensory neuronopathy following antibiotic treatment 8 0 4 and
segmental demyelination by perhexiline maleate used for therapy of angina pectoris. 7 9 Administration of
diphtheria toxin 5 5 6 or tetanus toxoid 6 9 3 or chronic exposure to lead may also cause
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myelinopathy. Distal axonopathies, the most common form of toxic neuropathy, often involve not only
peripheral but also central axons, causing centralperipheral distal axonopathy. In experimental
acrylamide neuropathy, recovery begins in the largest peripheral axons perhaps at the expense of central
axons. 4 1 0
A variety of drugs and industrial chemicals cause distal axonopathy. Drugs with known neurotoxicity
include allopurinol, 3 8 amiodarone, 1 3 2 , 2 6 3 , 3 9 0 , 6 6 6 chloramphenicol, cisplatin, 6 9 8 , 7 0 3 colchicine, 4 7 0 , 7 2 2 , 9 2 8
dapsone, 4 5 4 diphenylhydantoin, 6 9 0 2', 3 '-dideoxycytidines 6 0 disulfiram, 2 6 , 6 3 8 FK 5 0 6 , 9 0 8 gold, 4 2 1 isoniazid,
lithium, 1 3 0 , 8 7 5 L-tryptophan, 2 9 5 melarsoprol, 2 9 4 metronidazole, 8 4 misonidazole, 5 6 nitrofurantoin, nitrous
oxide, 4 9 2 , 7 2 5 penicillamine, 6 6 4 perhexiline maleate, 7 9 , 7 2 6 phenytoin, 7 6 6 pyridoxine, 6 3 , 6 5 5 , 9 1 1 taxol, 2 3 5 , 5 0 9 , 8 6 0
suramin, 7 9 2 thalidomide, 2 7 3 and vincristine. 8 6 , 1 1 5
Some drugs show a characteristic pattern of neuropathic involvement. For example, vincristme causes
primarily motor neuropathy, whereas pyridoxine abuse leads to a pure sensory centralperipheral distal
axonopathy. 6 5 5 Studies in chick embryos show that exogenous administration of gangliosides may
attenuate the neurotoxicity of vincristine in vitro. 3 7 1 Cisplatin used to treat maligant tumors induces an
axonopathy that bears a great resemblance to sensory neuropathy sometimes associated with such a
neoplasm. 4 6 0 This dosedependent sensory neuropathy primarily causes a distal lesion, affecting large
sensory neurons as well as the spinal cord and brainstem. 4 6 2 The adrenocorticotropic hormone analogue
Org 2766 can prevent or attenuate cisplatin neuropathy. 8 6 5 Psychiatric patients treated with the
phenothiazine derivative perazine may develop subacute axonal neuropathy after intense sun
exposure. 7 0 2
Industrial chemicals causing toxic axonal neuropathy include acrylamide, 4 1 0 , 6 0 0 carbon disulfide, 6 4 6
isofenphos, 1 2 0 inorganic mercury, 1 3 , 3 0 , 7 8 0 methyl n-butyl ketone, 1 6 , 7 9 8 n-hexane, 1 2 9 , 6 2 3 , 6 6 0 , 7 8 3 nitrous
oxide, 8 8 5 organophosphate ester mecarbam, 8 0 0 organophosphate parathion, 5 1 7 , 8 7 7 polychlorinated
biphenyl, 1 3 9 tellurium, 8 5 0 thallium, 1 9 2 , 9 2 4 triorthocresyl phosphate, 8 7 6 and vinyl chloride. 6 6 8 These toxic
axonal neuropathies generally affect the largediameter fibers, first in the distal segments with
subsequent progression proximally toward the cell body. The pathologic process then spreads to small
diameter axons.
The sudden development of clinical symptoms in distal axonopathy reflects the acuteness of intoxication.
In contrast, an insidious onset suggests chronic lowlevel exposure. Toxins often affect the longer and
more vulnerable nerves of the lower limb initially. Early signs include distal weakness, hypesthesia or
paresthesia in a glove and stocking distribution, as well as reduced ankle stretch reflexes. Symptoms
may worsen after termination of exposure. Despite this phenomenon, referred to as coasting, the
removal of the neurotoxin eventually leads to a gradual recovery. The axons, once degenerated,
regenerate slowly over months to years, with incomplete return of function. The selection of proper
electrophysiologic tests depends largely on the nature of the condition under study. 4 9 4 A few specific
toxins such as perhexiline maleate result in demyelination as evidenced by motor nerve conduction
studies. 7 9 Toxic exposure to nhexane causes a primarily axonal polyneuropathy with secondary
demyelination 6 2 3 , 7 8 3 and pathologic features consistent with giant axonal neuropathy. 1 2 8 Most other
toxins lead to axonal loss, showing reduced amplitude of the compound nerve and muscle action
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potentials. In these cases, substantial degeneration of large, fastconducting fibers accounts for a slight
increase in distal latency and a decrease in conduction velocity. Electromyography shows fibrillation
potentials and positive sharp waves. Lead and arsenic, two specific agents responsible for distal axonal
neuropathies, merit further attention.
The general features of lead poisoning include abdominal cramps, encephalopathy, and the occasional
appearance of a blue lead line along the gingival border. Laboratory tests reveal the presence of
basophilic stippling of erythrocytes and elevated lead levels. Neuropathy occurs primarily in adults
occupationally exposed to lead or following accidental ingestion of contaminated food but may also affect
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children with known plumbism or pica. 2 5 2 Predominent involvement of motor fibers innervating the
extensor muscles of the upper limbs produces bilateral radial nerve palsies without sensory loss. The
removal of the toxin leads to a gradual recovery over a period of several months. Lead produces
segmental demyelination in some animal species, possibly because extravasated lead in the interstitial
fluid injures the Schwann cells directly. 5 9 1 This type of pathologic change does not necessarily
characterize the neuropathy seen in human cases, 1 0 1 which show severe axonal loss. 9 1 9 A group of
workers exposed to lead had temporally dispersed compound muscle action potentials but normal
maximal conduction velocity. 1 1 9
Arsenic poisoning usually results from accidental ingestion of rat poison or exposure to industrial
sprays. 2 5 3 The administration of melarsoprol, an organoarsenic compound, may also cause toxic arsenic
accummulation in the presence of renal and hepatic dysfunction. 2 9 4 Polyneuropathy develops several
weeks after acute poisoning or more slowly with chronic lowlevel exposure. Pale transverse bands
bearing the eponym Mee's lines appear parallel to the lunula in all fingernails and toenails about 4-6
weeks after arsenic ingestion. In one study, serial examination revealed maximal sensory and motor loss
within 4 weeks of the estimated time of exposure and only partial improvement 2 years after the onset of
illness. 5 8 9 Arsenic is found in the urine during acute exposure and in the hair and nails later. These
clinical features resemble those of alcoholic neuropathy with early loss of stretch reflexes and painful
paresthesias and sensory loss in a glove and stocking distribution. Flaccid paralysis may develop later,
beginning in the lower limbs and eventually affecting the upper limbs. Electrophysiologic studies show
marked sensory abnormalities indicative of axonal degeneration, 6 2 6 progressive slowing of motor
conduction velocity, 5 8 9 and evidence of denervation in electromyography. Timely removal of the toxin
leads to nearly complete recovery of conduction abnormalities. An acute demyelinating polyneuropathy
may develop following acute exposure in contrast to the distal axonal predominantly sensory involvement
associated with chronic lowlevel toxicity. 3 1 6
5 Inherited Neuropathies
Hereditary motor and sensory neuropathy (HMSN) comprises several types: hypertrophic and neuronal
varieties of Charcot-Marie-Tooth disease (CMT), Dejerine-Sottas disease, Refsum disease, and those
associated with spinocerebellar degeneration, optic atrophy, and retinitis pigmentosa. Patients with these
familial demyelinative neuropathies characteristically have uniform conduction slowing of all nerves
without signs of major conduction block. This stands in sharp contrast to the typical findings in an
acquired demyelinative neuropathy with multifocal slowing and conduction block and differential
involvement of various nerves and nerve segments. 5 0 2 Other inherited polyneuropathies include
hereditary neuropathy with liability to pressure palsies, Friedreich's ataxia, acute intermittent porphyria,
cerebral lipidosis, hereditary sensory neuropathy, lipoprotein neuropathy, giant axonal neuropathies,
Fabry's disease, and familial amyloid neuropathy.
Genetic Classification of Hereditary Motor and Sensory

Neuropathies
Charcot-Marie-Tooth disease (CMT), although long regarded as a single entity, consists of two major
varieties, hypertrophic and neuronal. 1 0 0 , 1 2 4 , 3 4 6 , 3 8 2 Genetic linkage studies provide evidence for further
heterogeneity. 6 8 , 3 2 3 , 6 5 0 The most common hypertrophic or demyelinative form (Table 25-3) usually has an
autosomal dominant inheritance genetically localized on chromosome 17 (CMT1A) or chromosome 1
(CMT1B). 1 9 6 , 3 3 3 , 3 4 5 The most prevalent form, CMT1A, has a tandem duplication of chromosome 17pll.212
with trisomic expression of the peripheral myelin protein 22 (PMP-22) gene 5 2 1 or, less frequently, a
missense mutation of PMP-22. 1 2 7 , 8 5 6 Men tend to have a more severe form of the disease
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Table 253 Genetic Classification of Hereditary Motor and Sensory Neuropathy
Locus Gene Mechanism
CMT1 (HMSN type I)
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CMT1A 17pll.212 PMP22 Duplication/point mutation
CMT1B lq2123 Po Point mutation
CMT1C Unknown Unknown Unknown
CMT2 (HMSN type II)
CMT2A lp3536 Unknown Unknown
CMT2B 3ql322 Unknown Unknown
CMT2C Unknown Unknown Unknown
CMTX (X-linked HMSN)
CMTX1 Xq13.1 CX32 Point mutation
CMTX2 Xp22.2 Unknown Unknown
CMTX3 Xq26 Unknown Unknown
Dejerine-Sottas disease (HMSN type III)
DSD type A 17pll.212 PMP22 Point mutation
DSD type B lq2223 Po Point mutation
Hereditary neuropathy with pressure

palsies
HNPP type A 17pll.212 PMP22 Deletion/point mutation
HNPP type B Unknown Unknown Unknown
CMT = Charcot-Marie-Tooth disease; CMTX = CMT, X-linked dominant or recessive; DSD = Dejerine-Sottas
disease; HMSN = hereditary motor and sensory neuropathy; HNPP = hereditary neuropathy with pressure
palsies.
than women, who may have formes frustes. 3 4 6 In contrast to CMT1A, the less common CMT1B has a
linkage to chromosome lq21-23, showing point mutations in myelin protein zero (P0). 5 2 2 , 7 7 9 Another type,
CMT1C, has no linkage to either chromosome 1 or chromosome 17.
Genetic linkage analysis has identified at least three different forms of the neuronal type (CMT2)
mapping to chromosomes lp, 3 q and 7p: CMT2A (lp35-36), CMT2B (3q 13-22), CMT2C (unknown loci), and
CMT2D (7pl4). 5 2 2 , 7 3 3 Other reported sites of mutation include chromosome lq21-23 (P0). 5 4 1 A neuronal
type with onset in early childhood shows none of the regenerative features considered characteristic of
autosomal dominant CMT2. 2 7 6 Occasional patients have an autosomal recessive, 3 4 6 X-linked dominant
pattern (CMTX1), 6 7 4 or a recessive (CMTX 2 and CMTX 3) pattern. 3 8 6 Clinical electrophysiologic and
histologic findings also support primary axonal or demyelinating neuropathy in the X-linked disorder
(CMTX), which includes X-linked dominant CMTXl(Xql3. 1 ) with connexin 32(CX32) point
mutations, 6 9 , 7 3 7 , 8 1 9 and X-linked recessive CMTX2(Xp22. 2 ) and CMTX3(Xq26) without CX32 point
mutations. 3 8 2
Some families with autosomal dominant HMSN have calf enlargement caused by muscle fiber hypertrophy
predominantly of type I fibers, 1 9 5 , 7 3 4 and others have neuropathy with optic atrophy. 7 9 3 In one family
with HMSN, some members had features of myotonic dystrophy, and others had only its genetic markers
on chromosome 19. 7 9 7 A large group of clinically unequivocal cases show a bimodal distribution of nerve
conduction velocities. 3 4 6 Some kinships have both the neuronal and hypertrophic types and some
investigators emphasize the existence of an intermediate variety. 8 0 , 2 9 2 , 6 7 4
Linkage analyses in autosomal dominant cerebellar ataxia have demonstrated genetic heterogeneity and
subclassification:467 spinocerebellar ataxia type 1 to type 7 (SCA1 to SCA7) with five identified genes all
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showing expanded and unstable CAG repeat, SCA1 on 6p22-23, SCA2 on 12q23-24. 1 , SCA 3/Machado-
Joseph disease (MJD) on 14q24. 3 , SCA6 on 19pl3, and SCA7 on 3pll-13, and two unidentified genes,
SCA4 and SCA5 on chromosomes 16 and 11.
In another disorder called hereditary neuropathy with liability to pressure palsy (HNPP) with autosomal
dominant inheritance, 4 9 6 slight traction or compression leads to motor and sensory deficits in an
otherwise asymptomatic patient. In most families thus far studied, patients have a
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1.5 megabase (Mb) deletion in a segment of chromosome 17p11.2-12 that contains the PMP-22
gene. 4 1 8 , 5 3 7 , 6 0 7 , 9 2 6 The duplication in CMTIA and deletion in HNPP in the same region are probably
consequences of unequal crossingover during germ cell meiosis. 1 2 7 Both neuropathies result from an
imbalance in PMP-22 expression. 2 7 8 In one series of 51 patients with multifocal neuropathies, DNA
analysis detected the deletion of 17p11. 2 in 24, establishing the diagnosis of HNPP. 8 4 8 In another study,
underexpresssion of PMP-22 mRNA correlated with disease severity and with mean axon diameter. 7 4 8
Reports of kinships without the typical 1. 5 Mb deletion suggest genetic heterogeneity. 1 9
Charcot-Marie-Tooth Disease Type 1 (HMSN Type I)

The hypertrophic variety of CMT1 affects both sexes, but men more commonly than women. Histologic
studies reveal enlargement of the peripheral nerves, segmental demyelination and remyelination with
onion bulb formation, and axonal atrophy. 8 7 3 Despite some studies suggesting a primary neuronal
disturbance based on axonal atrophy, morphologic and morphometric investigations reveal a lack of
small- and largediameter myelinated axons at an early stage, and a demyelinating process followed by
axonal loss. 2 7 7 In a kindred displaying a dominant inheritance, marriage between two heterozygotes
resulted in two homozygous offspring. The homozygotes had clinical features of the classic Dejerine-
Sottas disease. Unusual and sometimes devastating clinical features may result from a rare chance
association of CMTIA with such disorders as facioscapulohumeral muscular dystrophy, 1 0 3 myasthenia
gravis, 1 3 8 Noonan syndrome, 7 6 9 and posterior interosseous nerve syndrome. 1 1 2
The symptoms begin insidiously during the first two decades, sometimes with subtle clinical signs
appearing even in children before 1 year of age. These include pes planus, distal foot wasting, weakness
of ankle eversion, and dorsiflexion and areflexia. 2 4 8 Atrophy initially involves the peroneal musculature
and then the thigh and the upper limbs, sparing the trunk and girdle musculature. Some patients develop
diaphragmatic paralysis with respiratory or cardiac failure. 3 4 3 The classic stork leg configuration develops
only rarely in the hypertrophic type. Bilateral footdrop causes a characteristic gait difficulty. The patient
has paresthesias, dysethesias, and muscle pain associated with foot deformity. Typical findings include
palpable nerves, loss of vibratory and position senses, reduced cutaneous sensations, and diminished
stretch reflexes, first at the ankle and later diffusely. The disease progresses very slowly over many
decades, at times showing spontaneous arrest. Muscle atrophy and weakness may incapacitate the
patient, but not always. Many investigators consider Roussey-Levy syndrome with a static tremor of the
hands as a variant of this type. 1 0 7 Patients may suffer from temporary worsening of otherwise stable
symptoms during pregnancy. 7 2 1 Neurologic deficits may result from compression of the spinal cord,
vertebral arteries, or neural foramina by the hypertrophic nerve roots. 7 1 4 Occasionally, a patient with
CMT1 will develop superimposed chronic inflammatory demyelinating polyradiculoneuropathy, which may
respond to immunosuppressive therapy 5 7 7 or corticosteroids. 6 7 Possible surgical therapies for upper limb
neuropathy include standard tendon transfers, nerve compression release, soft tissue releases, and joint
fusions. 9 6
Nerve conduction studies show a marked, diffuse, and uniform slowing as a hallmark of CMTl. 1 5 6 , 3 4 7 , 8 5 1
The uncommon recessive forms have slower conduction than the dominant form. 3 4 7 The motor conduction
velocities in affected family members average less than one half those of normal individuals, varying
from 9 to 41 m/s with a mean of 25 m/s. 2 2 5 The range of conduction velocities found in affected
individuals show no overlap with those of their clinically normal relatives, indicating complete penetrance
of the gene from early childhood. 6 0 6 Slowing of conduction is completely concordant with the presence of
the segmental duplication in CMTIA. 4 1 5 The great variation in conduction velocity emphasizes the
influence
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of factors apart from the shared genetic mutation on phenotypic expression. Prolonged terminal
latencies in the early stages Indicate distally prominent slowing. 3 2 6 The disease affects both peripheral
and central sensory fibers, as evidenced by delay and reduction of sensory potentials as well as
somatosensory evoked potentials. 4 0 5
Despite slowing, a limited degree of temporal dispersion indicates a homogeneity of the pathologic
process. The extent of the conduction abnormality varies little, not only among members in the same
family but also from one nerve to another in the same patient. 4 0 5 Such uniformity helps differentiate this
entity from acquired inflammatory polyneuropathy. Conduction abnormalities may herald clinical onset of
neuropathy. 8 7 4 Motor nerve conduction velocities attain maximal slowing over the first 3-5 years of life 3 2 6
and remain relatively stable thereafter, 4 3 5 whereas compound muscle action potentials decline in
amplitude, reflecting a progressive axonal loss. 7 1 7 Both measures, despite an inverse relationship to
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clinical severity, show no correlation with age, 3 6 8 probably because the primary pathologic process
remains inactive after childhood. 2 4 4 Serial electrophysiologic studies can detect the CMT1A gene
abnormalities in infancy and early childhood. 2 8 5 For purposes of genetic counseling, a clinically and
electrophysiologically normal subject at 6 months of age has a very small risk of having inherited the
CMT1 gene, 5 5 although the florid clinical picture may not occur until the second decade of life. 2 8 5
Other electrophysiologic abnormalities include absent or delayed F waves, a finding 4 4 2 that matches the
slowing of motor nerve conduction in the distal segment (see Figs. 18-7 and 18-10 and Table 18-3). 4 3 8
Studies of facial nerve; 3 0 3 , 4 3 7 and phrenic nerve 1 1 1 also show increased latencies despite relatively
normal strength of the facial muscles and diaphragm (see Figs. 17-12A and 17-14 and Tables 17-2 and
17-4). Recording isometric force during fastest voluntary contraction shows a prolongation in contraction
time and a reduction in maximal rate of rise of tension. 5 1 3 In many patients, studies of evoked potentials
detect a minor degree of involvement of visual 1 0 8 and auditory 4 5 9 , 7 4 5 pathways. Some patients also have
impaired central conduction 7 9 0 and autonomic dysfunction, 7 9 0 but not universally. 3 7 9
Charcot-Marie-Tooth Disease Type 2 (HMSN Type II)

In the neuronal variety of CMT, patients have neither hypertrophic nerves nor prominent segmental
demyelination. Inherited as an autosomal dominant disorder, symptoms and signs appear in early
adulthood or later. Rarely the disease appears in early childhood sporadically or with autosomal
recessive or dominant inheritance. 6 4 5 Most consider a third type of CMT disease, designated as the spinal
form, as a variant of the neuronal type or of distal spinal muscular atrophy.
The clinical features, although much less generalized, resemble those of CMT1 with less conspicuous
sensory disturbances. As the name peroneal muscular atrophy indicates, affected patients develop
selective muscular wasting of the legs with limited involvement of the upper limbs in early states. An
almost total loss of muscle bulk below the knee gives rise to a stork leg appearance. Despite footdrop
with severe weakness of the plantar flexors and clubfeet, patients often walk fairly well, rarely showing
total incapacitation. Some affected individuals have tremors of the hands, but much less commonly than
those with CMT1. Plexiform neurofibroma of the cauda equina may mimic peroneal muscular atrophy. 5 8
Electrophysiologic studies reveal mild slowing of nerve conduction velocities, consistent with a reduction
in amplitude of the compound sensory nerve and muscle action potentials. 5 6 , 3 4 7 Electromyographic
studies typically show large motor unit potentials, fasciculation potentials, fibrillation potentials, and
positive sharp waves. 2 2 3
Charcot-Marie-Tooth Disease X-linked Dominant Type 1

The genetically heterogeneous group of hereditary motor and sensory neuropathies includes a rare
variant with X-linked dominant inheritance. 7 1 8 In a large Canadian kindred traced through six
generations, 3 2 9
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affected fathers had no male-to-male transmission, whereas all their daughters expressed the disease.
The typical clinical features included onset in early childhood, pes cavus, distal muscular atrophy, and
sensory abnormalities. Electrophysiologic observations indicated a substantial loss of distal motor and
sensory nerve fibers with primary axonal degeneration, a nonuniform slowing of motor conduction
velocities and dispersion of compound action potential reminiscent of acquired chronic demyelination. 8 1 9
Hypertrophic Polyneuropathy of Dejerine-Sotas (HMSN Type III)

Dejerine (1890) 1 9 7 and Dejerine and Sottas (1893) 1 9 8 described a very severe, generalized form of
demyelinating sensory motor neuropathy inherited as an autosomal recessive trait. 7 4 2 The disorder shows
a considerable genetic heterogeneity 5 2 2 with a mutation in either PMP-22 3 8 3 , 3 8 5 , 5 3 9 , 7 0 1 or P o 3 5 5 or linkage
to chromosome 8. 3 8 4 The affected nerves have marked thickening, onion bulb formation, segmental
demyelination, and thinning of the myelin surrounding the nerve. Symptoms appear in infancy with
delayed development of motor skills, especially in walking. Clinical features consist of pes cavus, muscle
cramps, incoordination, kyphoscoliosis, weakness, sensory loss, and abducens and facial nerve palsies.
Adult patients often have paraparesis and severe truncal ataxia, requiring the use of a wheelchair.
Patients with this disorder have a higher incidence of ataxia, areflexia, and hypertrophic nerves than
those with CMT1. Pathologic analysis reveals greater loss of myelinated fibers, a larger number of onion
bulbs with more lamellae per each, and a higher ratio of the mean axon diameter to the fiber
diameter. 6 4 4 Nerve conduction studies reveal marked slowing of the motor and sensory fibers. In one
series of 11 patients, all but one had median and ulnar motor conduction velocities less than 6 m/s. 5 4
The differential diagnosis should include congenital demyelinating motor and sensory neuropathy with
focally folded myelin sheaths. 2 7 5 In this condition, nearly all teased fibers have an abundance of focal
myelin thickenings, or tomacula, which serve as a striking discriminating feature. The clinical, genetic,
and electrophysiologic characteristics otherwise resemble those of Dejerine-Sottas disease. In contrast
to the generalized form, rare localized hypertrophic neuropathy consists of isolated mononeuropathy with
focal nerve enlargement. 7 8 6 This entity represents a localized form of Dejerine-Sottas disease, an
entrapment neuropathy, or an intraneural neurofibroma. In some patients, morphologic findings in the
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localized areas of enlarged nerves consist of primary perineurial cell hyperplasia or perineurinoma. 5 7 9
Nerve conduction studies suggest severe motor and sensory axonal loss with no evidence of slowed
conduction velocity. Electromyography also indicates focal axonal loss with evidence of severe
denervation limited to the territory of the affected nerve.
Hereditary Ataxic Neuropathy of Refsum (HMSN Type IV)

Hereditary ataxic neuropathy of Refsum is a rare disorder transmitted by an autosomal or a recessive
gene that has characteristic pathologic changes in the olivocerebellar tracts, anterior horn cells, and
peripheral nerves. 7 3 6 The typical clinical features comprise deafness, anosmia, night blindness with
retinitis pigmentosa, ichthyosislike skin, cerebellar signs, and nystagmus. Involvement of the peripheral
nerves causes lightning pain in the legs, wasting of muscles, hyporeflexia, hypotonia, and diminished
vibration and position sense. A metabolic defect in the oxidation of branched chain fatty acids elevates
serum phytanic acid, which for unknown reasons leads to a hypertrophic neuropathy. Patients develop
recurrent segmental demyelination and motor and sudomotor axonal losses in parallel with exacerbations
of weakness, showing an apparent long-term clinical stabilization. 4 7 1 , 8 3 2 Electrophysiologic studies reveal
decreased sensorimotor conduction velocities in all limbs. 2 0 4 Severe axonal involvement in the lower limb
may characterize other cases. 2 8 8 Dietary restriction of phytol results in considerable improvement
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of symptoms. Some patients with retinitis pigmentosa and ataxia have a syndrome that clinically
resembles Refsum's disease without detectable biochemical abnormalities. In these cases,
electrophysiologic studies reveal mildly delayed, lowamplitude sensory action potentials but no evidence
of hypertrophic neuropathy. 8 4 6
Autosomal Dominant Cerebellar Ataxia

Autosomal dominant cerebellar ataxia with neuropathy (ADCA) superficially resembles CMT with distal
wasting and weakness involving the legs more than the arms. 7 4 0 , 8 0 7 Some patients show muscle wasting
presumably reflecting the loss of motor neurons. 1 Most patients have an extensor plantar response with
normal or increased stretch reflexes in the upper limbs and at the knee, but often absent ankle jerks. In
one series, 4 6 7 sensory or sensory motor polyneuropathy was found in 42 percent of patients with SCA1,
80 percent of SCA2 and 54 percent of SCA3. Further, SCA1 patients with polyneuropathy had a
significantly higher CAG repeats than those without polyneuropathy.
Electrophysiologic abnormalities include lower than normal mean motor and sensory nerve conduction
velocities and reduced amplitude of sensory nerve action potentials. 3 4 8 , 5 6 1 Median nerve somatosensory
evoked potentials reveal decreased amplitude of N 1 3 and N 2 0 with increased interpeak latencies,
implicating central and peripheral sensory pathways. 5 8 5 Sural nerve biopsies show fewer myelinated
fibers and normal unmyelinated fibers. 5 6 1 Peripheral neuropathy also develops in some patients with
infantile onset 4 5 8 and late onset 2 4 3 , 6 1 2 spinocerebellar degeneration, sometimes associated with ceroid
lipofuscinosis. 9 1 5
A predominantly sensory axonal neuropathy, seen in olivopontocerebellar atrophy, affects those patients
with glutamate dehydrogenase deficiency, but not those with normal enzymatic activities. 1 4 3 Such a
distinction may serve as an electrophysiologic marker for differentiating the subtypes. The postmortem
examination of one patient revealed olivopontocerebellar atrophy, demyelination of the posterior
columns, degeneration of anterior horn and dorsal root ganglion cells, and reduced myelinated fibers in
the sural nerve. 1 4 4
Hereditary Neuropathy with Liability to Pressure Palsies

Hereditary neuropathy with liability to pressure palsies (HNPP) is a familial disorder of autosomal
dominant inheritance. 4 9 6 Histopathologic changes include focal, sausagelike, or tomaculous thickening
of the myelin sheaths and noncompacted loose myelin lamellae together with segmental demyelination
and remyelination. 5 3 , 5 2 6 , 8 4 1 The most prominent feature of the disease is pressureinduced, reversible
motor weakness, although sensory symptoms may also appear. 2 1 2 Compression palsy commonly affects
the ulnar, radial, and peroneal nerves, with recovery occurring slowly over weeks or months. Occasional
patients may develop acute anterior interosseous neuropathy 2 5 4 or recurrent familial brachial plexus
palsies or other acute painless mononeuropathies 6 5 1 as the only or predominant clinical
manifestation. 5 4 2 , 8 0 8 Others may have acute recurrent polyneuropathy 4 0 6 , 4 9 8 or chronic sensory motor
neuropathy as the presenting symptom. 2 5 5 , 5 3 0 Rare associated features include central nervous system
demyelination, 1 7 and the syndrome of moving toes and myoclonus. 7 5 6
Motor and sensory studies show focal conduction abnormalities at usual compression sites 8 5 1 in paretic
limbs but also in some clinically unaffected nerves. 8 4 1 Evaluations of clinically normal nerves reveal
electrophysiologic abnormalities in approximately one half of the patients and some asymptomatic
relatives. 1 7 9 A pathologically thick myelin sheath probably causes longlasting conduction block and the
slowing of conduction velocities seen in some cases, 7 5 4 although segmental demyelination also plays a
role. 7 6
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Friedreich's Ataxia
Friedreich's ataxia is an autosomal recessive disorder associated with a GAA trinucleotide
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repeat expansion in the first intron of the X25 gene on chromosome 9q 13-21. 1 . Patients who develop
mild symptoms without cardiomyopathy later than the usual onset may have limited GAA
expansions. 2 8 7 , 2 9 0 , 5 2 3 The disease primarily affects the spinocerebellar tracts, corticospinal tracts, and
posterior columns of the spinal cord. In advanced cases, the degeneration also involves the dorsal roots
and peripheral nerves. Despite the severe loss of large myelinated fibers, wellpreserved unmyelinated C
fibers conduct normally. 2 2 6 , 6 4 3 The only consistent clinical findings within 5 years of presentation consist
of limb and truncal ataxia and absent stretch reflexes in the legs. 3 4 4 All patients eventually develop
dysarthria, signs of pyramidal tract dysfunction in the legs, and loss of joint, position, and vibration
sense. Other less frequent clinical features include cardiomyopathy, kyphosis, scoliosis, pes cavus, distal
amyotrophy, optic atrophy, nystagmus, and deafness. On average, patients lose the ability to walk by
the age of 25 years and become chairbound by the age of 44 years. 3 4 4 Common variabilities include late
onset, preservation of the lower limb tendon reflex, and slow progression. 1 7 4
Electrophysiologic studies show absent or considerably reduced sensory nerve potentials 5 5 8 , 7 3 5 and
essentially normal motor conduction studies except for a modest slowing in some patients. 6 4 3 Nerve
biopsy reveals a severe loss of large myelinated fibers, but no demyelination. 1 1 3 Somatosensory evoked
potentials may reveal abnormal peripheral as well as central conduction. 1 9 9 , 6 6 3 Transcortical magnetic
stimulation indicates an abnormal central motor conduction time, which progressively worsens as the
disease advances. 1 7 8 Patients rarely complain of visual impairment, but most have an increased latency
or reduced amplitude of the visual evoked potential. 1 0 9 , 5 1 2 , 6 6 3
Porphyria
An acute, primarily motor neuropathy characterizes several forms of porphyria, a rare hereditary
disorder that belongs to the category of inborn errors of metabolism. 2 3 These include acute intermittent
porphyria, variegate porphyria, and hereditary coproporphyria. 4 6 A partial defect in hepatic heme
synthesis results in overproduction of delta aminolevulinic acid and porphobilinogen. The disease has a
higher incidence in women, autosomal dominant inheritance, and variable degrees of expression. Clinical
features include abdominal pain, vomiting, peripheral neuropathy, neurogenic bladder, seizures, and
mental status changes, but no skin photosensitivity. Excessive quantities of porphyrin intermediates
excreted in the urine impart a deep red color with formation of polypyrroles from porphobilinogen on
exposure to light. Patients experience acute attacks either spontaneously or after inadvertent ingestion
of barbituates, sulfonamides, or certain other drugs.
Acute axonal neuropathy affects motor fibers regularly and sensory fibers in about 50 percent of
patients. Weakness progresses rapidly, involving the axial muscles more than the distal muscles. The
sensory loss, although relatively mild, may also predominate proximally. Nerve conduction studies show
lowamplitude compound action potentials with normal conduction velocities. Electromyography reveals
prominent fibrillation potentials and positive sharp waves in the proximal muscles 1-2 weeks after
onset. 1 5 , 7 8
Cerebral Lipidosis
Polyneuropathy accompanies at least two types of cerebral lipidosis: Krabbe's disease and metachromatic
leukodystrophy. In both entities, a marked slowing of nerve conduction helps establish the clinical
diagnosis, although confirmation comes from a nerve or cerebral biopsy. 2 8 2 , 5 3 8
Krabbe's disease, an autosomal recessive disorder, affects the white matter of the central and peripheral
nervous systems. A galactocerebrosidase (GALS) deficiency causes accumulation of undegraded
psychosine, leading to the pathologic hallmarks of globoid cell leukodystrophy. Identification of a
homozygous point mutation in the GALS gene confirms the diagnosis. 7 4 1 Histologic studies in Krabbe's
globoid cell leukodystrophy reveal diffuse
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loss of myelin throughout the cerebral white matter and peripheral nerves. Prominent perivascular cuffs
appear, consisting of greatly enlarged cells with the accumulation of cerebroside. Affected infants,
normal at birth, develop severe neurologic disturbances within the first few months of life. The disease
often follows a fulminant course, with rigidity, head retraction, optic atrophy, bulbar paralysis, a
decorticate posture, and, finally, death before the end of the first year. Neuropathy, usually a late
manifestation, is occasionally one of the presenting features. 1 7 6 , 5 0 5
In metachromatic leukodystrophy, 2 6 8 , 4 6 5 , 9 2 0 a deficiency of arylsulfatase leads to an abnormal breakdown

of myelin. Metachromatic staining properties result from cerebroside sulfate, which accumulates in the
nervous tissue. Neurologic signs include spasticity, ataxia, dementia, and neuropathy. The disease
usually affects infants, but rarely children 1 4 9 , 3 3 7 or adults. 7 7 Electrophysiologic studies reveal
substantially slowed nerve conduction as would be expected in a demyelinative neuropathy.
Morphometric studies reveal a marked reduction in sheath thickness, particularly in the large myelinated
Page 22 of 72
fibers. 4 1
Hereditary Sensory and Autonomic Neuropathy

Hereditary sensory neuropathy consists of four distinct entities. Type I has autosomal dominant
inheritance with degeneration of the dorsal root ganglias, early loss of sensory nerve action potential,
and preservation of the sympathetic skin responses. 7 6 5 In one family, sural nerve biopsies showed a
marked loss of all myelinated fibers and a comparable loss of unmyelinated fibers. 1 8 9 Clinical findings
include loss of pain and temperature sensation, areflexia, and development of ulcers in the lower limbs
with almost complete sparing of the upper limbs. The disease tends to progress slowly after its onset in
the second decade of life. Deafness, diarrhea, and ataxia occasionally develop in affected individuals.
Type II has autosomal recessive inheritance with onset in infancy or early childhood. It affects both
upper and lower limbs equally, with a higher incidence of chronic ulceration than in type 1. 1 2 2
Characteristic features include progressive sensory neuropathy, spastic paraplegia, and a mutilating
lower limb acropathy. 8 2 4 , 8 3 7 Nerve conduction studies show absent sensory action potentials and
borderline slow motor nerve conduction velocities.
Type III is the same as familial dysautonomia or Riley-Day syndrome, 7 0 0 , 8 3 0 and type IV is a rare
congenital loss of C fibers with complete insensitivity to pain. 5 1 8 Other entities in this category include
familial sensory autonomic neuropathy with arthropathy in Navajo children. 4 0 1
Lipoprotein Neuropathies
Two types of lipoprotein disorders accompany neuropathies. Patients with BassenKornzweig syndrome,
mostly Jewish children, have malabsorption, cerebellar signs, retinitis pigmentosa, acanthocytosis, and
virtual absense of betalipoprotein in the serum, or abetalipoproteinemia. Diminished stretch reflexes and
the absence of position and vibratory senses suggest a peripheral neuropahy. Neurologic signs resemble
those of Friedreich's ataxia and Refsum syndrome. In one histologic study, the sural nerve showed a
decreased number of large fibers with diameters greater than 7 um, regeneration, and paranodal
demyelination. 9 0 0
Electromyographic findings include signs of chronic denervation in distal limb muscles; myotonic
discharges; largeamplitude, longduration motor unit potentials; and poor recruitment. Sensory nerve
conduction studies reveal reduced amplitude with a slight slowing in distal conduction velocity. 9 0 0 Motor
conduction studies show normal or slightly reduced amplitude with normal conduction velocities. 5 1 9 , 5 7 1
Other electrophysiologic abnormalities may include a prolonged latency of visual and somatosensory
evoked potentials. 9 0 The fiber diameter spectrum of the sural nerve indicates a loss in the 8-12 uam
diameter range.
Patients with Tangier disease have a low level of highdensity lipoprotein and cholesterol
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in the serum. Their enlarged tonsils have a characteristic bright orange color from the deposition of
cholesterol esters. Their skin and rectal mucosa display similar changes. Both myelinated and
unmyelinated fibers show degeneration. 4 5 0 Dissociated losses of pain and temperature sensation, not
unlike those seen in syringomyelia, suggest selective involvement of the small fibers. 2 1 7 Patients may
have a relapsing and remitting mononeuropathy with prominent demyelination and remyelination or
slowly progressive neuropathy with advanced axonal degeneration. 6 7 8 Conduction studies may reveal
abnormal velocities in some patients but not in others. 2 8 2
Giant Axonal Neuropathy

Children with progressive peripheral giant axonal neuropathy 3 2 , 2 0 1 , 8 2 8 usually have minor central nervous
system involvement and intellectual dysfunction. 5 8 2 The disease shows an autosomal recessive
inheritance trait with the responsible gene localized to chromosome 16q24. 2 6 2 The accumulation of
neurofilamentous material leads to ballooning and degeneration of the axons, 3 2 2 , 4 6 8 affecting the motor
fibers more than the sensory fibers. The clinical features in a large kindred included infantile onset,
progressive distal amyotrophy of four limbs, brisk reflexes, diffuse fasciculations, bulbar signs, and deep
sensory loss in both lower limbs. 3 3 8 Patients characteristically have tightly curled, reddish hair, in
contrast to the sparse hair seen in Menke's kinky hair disease.
Electrophysiologic studies suggest the presence of secondary demyelination triggered by axonal

enlargement, although available data are insufficient to characterize the condition. Abnormalities
demonstrated by evoked potential studies confirm clinical and pathologic findings of central nervous
system dysfunction. 5 2 9
Fabry's Disease
Fabry's disease is a multisystem X-linked recessive disorder. An inborn error involving glycosphingolipid
metabolism causes the accumulation of ceramide trihexose in various tissues. The enzymatic defect of
ceramide trihexosidase affects the skin, blood vessels, cornea, and the cell bodies of the dorsal ganglia.
Both the central and peripheral nervous systems show lipid depositions in endothelial and perithelial cells
Page 23 of 72
of the vessel walls or perikaryon. 7 7 0 Axonal degeneration primarily involves small myelinated and
unmyelinated fibers. 2 7 0 , 4 5 1 The presenting clinical features include severe burning sensations of the
hands and feet. Nerve conduction studies, although ordinarily normal, may show some slowing in
affected men and occasionally in female carriers. 7 6 1 Electromyographic studies reveal no abnormalities in
most cases.
Familial Amyloid Neuropathy

Signs and symptoms of amyloidosis result from deposits of amyloid around blood vessels and connective
tissues in multiple organ systems. Clinical features depend on the organs involved, which commonly
include the heart, tongue, gastrointestinal tract, skeletal muscles, and kidney. Amyloid deposits in the
flexor retinaculum may cause carpal tunnel syndrome in about one fourth of the patients. Familial
amyloid neuropathies, unlike primary or nonfamilial amyloid neuropathies associated with
paraproteinemia (see this chapter, part 2), have relentless progression of neurologic and cardiac
impairment, lending to death within 7-15 years after disease onset. Compared to hereditary sensory and
autonomic neuropathy, familial amyloid neuropathy shows a greater motor and autonomic involvement
with an early loss of sympathetic skin responses. 7 6 5 Liver transplantation may offer hope for arrest of
progression and improvement of sensory motorneuropathy. 6 4 Neurologic symptoms rarely develop in
secondary amyloidosis seen in chronic debilitating inflammatory processes.
A form of autosomal dominant amyloidosis prevalent in northern Portugal produces progressive

neuropathy involving the legs in young adults. Another, milder form of autosomal dominant amyloidosis
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with neuropathy of the upper limbs primarily affects Swiss families with the onset later in life. Familial
amyloid neuropathy has also involved kinships of German, 6 2 1 Japanese, 2 4 , 3 4 1 , 3 7 7 , 7 8 8 Northwest Ireland, 8 0 1
Taiwanese, 9 2 1 and English ancestries. 4 4 5 Transthyretin gene mutations, found in some of these hereditary
cases, 8 4 9 have also affected British and French patients without a family history. 6 6 The most common
familial amyloid polyneuropathy, type I, has a variant transtlyretin with a single amino acid
substitution. 7 0 These include a most frequent methionine-for-valine substitution reported from Portugal,
Italy, Sweden and Japan, and alanine-for-value substitution found in a family of German origin and a
leucine-for-valine substitution seen in Japanese pedigrees. 8 5 5 The familial amyloid polyneuropathy type
IV phenotype in Finnish as well as Japanese kinships 8 5 results from a single base substitution, guanine to
adenine at nucleotide position 654 in the gelsolin gene located on chromosome 9q32-q34.
Other Neuropathies
Other rare inherited systemic disorders associated with peripheral neuropathy include sialidosis type I,
or the cherryred spot myoclonus syndrome, 8 0 3 cerebrotendinous xanthomatosis, 4 7 3 a variety of
extrapyramidal syndromes, 1 0 4 multiple endocrine neoplasias, 2 1 6 neurofibromatosis, 4 3 6 , 8 3 6 Cockayne's
syndrome, 3 2 1 congenital hypomyelination polyneuropathy, 8 2 , 3 2 7 , 3 4 2 chorea acanthocytosis, 5 0 7
adrenomyeloneuropathy (see Fig. 188), 6 9 6 , 8 7 9 , 9 0 3 infantile neuroaxonal dystrophy, 5 9 4 mitochondrial
disorders, 5 8 3 hereditary tyrosinemia, 5 7 6 hereditary motor and sensory neuropathy with treatable
extrapyramidal features, 3 9 8 and lethal neonatal autosomal recessive axonal sensory motor
polyneuropathy. 8 7 8
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2 Neuropathies Associated With General Medical Conditions

some of the affected patients, neuropathy is caused by an inflammatory vasculopathy. 4 6 3 , 7 3 0 , 9 2 9

Diabetic thoracic radiculopathy produces a distinct syndrome characterized by radicular involvement,

Electrophysiologic studies have revealed a number of abnormalities in diabetic neuropathies. 1 5 0 , 2 1 8 , 5 2 4

impairment of peripheral as well as central afferent transmission. 1 6 8 , 3 2 5 Increased interpeak latencies of

Neuropathies in Malignant Conditions

Paraneoplastic neuropathies, as an autoimmune disorder, 2 1 1 result from the distant effects of

Neuropathies Associated with Paraproteinemia

Primary systemic amyloidosis or the lightchain type of amyloidosis affects multiple

Table 251 Main Features of Monoclonal Protein-Peripheral Neuropathy Syndromes

Sensory Autonomic CSF

Benign Distal, rarely ++ ++ + Chronic ++ Mild SD + AD

monoclonal (IgG, proximal progressive slowing

Benign Distal, ++ ++ 0 Chronic ++ Very

Amyloidosis, light Distal, +/++ +++ ++ Chronic + Mild

Osteosclerotic Distal, +++ + 0 Chronic +++ Very SD(+AD)

Waldenstrom's Distal, ++ ++ 0 Chronic ++ Very SD(occ'l

From Kelly,425 with permission.

spinal ganglion and postganglionic sympathetic ganglion cells. 4 6 9

3 Inflammatory, Infective, And Autoimmune Neuropathies

lipopolysaccharide. 9 3 2 In vitro demyelination by serum antibody from patients with Guillain-Barr

Miller Fisher Syndrome

Electrophysiologic studies usually show characteristics of an axonal neuropathy or neuronopathy with

Chronic Inflammatory Demyelinating Polyneuropathy

Multifocal Motor Neuropathy with Conduction Block

Table 252 Characteristics of MMN and CIDP

Pure motor manifestation Frequen Rare

Multiple mononeuropathy Yes No

Remission and exacerbation No Yes

Generalized areflexia No Yes

CSF protein level Often normal Elevated

Elevated Anti-GMl antibody Frequent Rare

Choice of therapy Immunosuppressants, immunoglobulin Steroids, plasma exchange

Acute Motor Axonal Neuropathy in China

Acquired Immunodeficiency Syndrome

neuropathy 4 3 , 2 9 3 , 3 0 4 , 3 2 4 , 3 3 1 , 3 7 2 , 9 1 3 as evidenced by nerve conduction studies. 1 8 0 , 2 7 1 , 4 8 4 , 7 8 5 In this entity,

In contrast to the autoimmune basis of demyelinative neuropathy, 8 1 0 less clearly established

A tick (Ixodes) bite may result in meningoradiculoneuritis with electromyographic evidence of

4 Metabolic And Toxic Neuropathies

Genetic Classification of Hereditary Motor and Sensory

Table 253 Genetic Classification of Hereditary Motor and Sensory Neuropathy

Locus Gene Mechanism

CMT1 (HMSN type I)

CMT1A 17pll.212 PMP22 Duplication/point mutation

CMT1B lq2123 Po Point mutation

CMT1C Unknown Unknown Unknown

CMT2 (HMSN type II)

CMT2A lp3536 Unknown Unknown

CMT2B 3ql322 Unknown Unknown

CMT2C Unknown Unknown Unknown

CMTX (X-linked HMSN)

CMTX1 Xq13.1 CX32 Point mutation

CMTX2 Xp22.2 Unknown Unknown

CMTX3 Xq26 Unknown Unknown

Dejerine-Sottas disease (HMSN type III)

DSD type A 17pll.212 PMP22 Point mutation

DSD type B lq2223 Po Point mutation

Hereditary neuropathy with pressure

HNPP type A 17pll.212 PMP22 Deletion/point mutation

HNPP type B Unknown Unknown Unknown