Transverse Myelitis - UpToDate

Transverse myelitis - UpToDate 01-05-18 15'35
Official reprint from UpToDate®

www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.
Transverse myelitis
Authors: Chitra Krishnan, MHS, Benjamin Greenberg, MD, MHS

Section Editor: Francisco González-Scarano, MD
Deputy Editor: John F Dashe, MD, PhD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Mar 2018. | This topic last updated: Nov 17, 2016.
INTRODUCTION — Acute transverse myelitis (TM) is a rare acquired neuro-immune spinal cord disorder that
can present with the rapid onset of weakness, sensory alterations, and bowel or bladder dysfunction. TM can
occur as an independent entity, usually as a postinfectious complication, but TM also exists on a continuum of
neuro-inflammatory disorders that includes acute disseminated encephalomyelitis, multiple sclerosis, and
neuromyelitis optica. The clinical features, diagnostic work-up, and acute and chronic therapies differ between
these forms of TM. It is important in the evaluation of patients with acute myelopathies to exclude compressive
and noninflammatory causes of myelopathy as well as to distinguish various types of TM, since the prognosis,
risk of recurrence, and treatment options may differ among these distinct entities.
This topic will review transverse myelitis. Related conditions are discussed elsewhere. (See "Acute disseminated
encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis" and "Acute disseminated
encephalomyelitis in adults" and "Pathogenesis, clinical features, and diagnosis of pediatric multiple sclerosis"
and "Clinical features of multiple sclerosis in adults" and "Clinically isolated syndromes suggestive of multiple
sclerosis" and "Neuromyelitis optica spectrum disorders" and "Disorders affecting the spinal cord".)
IMMUNOPATHOGENESIS — The immunopathogenesis of TM is varied and reflects the rather diverse spectrum
of this disease from idiopathic to disease-associated myelitis (see 'Associated conditions' below). There is
evidence of perivascular infiltration by monocytes and lymphocytes in the lesion [1]. Axonal degeneration is also
reported [1]. This pathologic heterogeneity and the involvement of both gray and white matter suggest that TM is
not a pure demyelinating disorder but rather a mixed inflammatory disorder that affects neurons, axons, and
oligodendrocytes and myelin. Postvaccination TM has been reported [2,3], and autopsy reports have described
lymphocytic infiltration with demyelination and axonal loss [4]. Although these case reports describe TM
occurring after vaccination, causation has not been established based on the timing and sequence of events
alone. In a US database with 64 million vaccine doses administered among children and adults from 2007
through 2012, there were only seven subjects with TM who were vaccinated during the primary exposure interval
of 5 to 28 days prior to TM onset [5]. Comparing each TM case with all matched subjects in the exposure interval
who received the same vaccination, there was no association of TM with prior vaccination.
In 30 to 60 percent of the idiopathic TM cases, there is an antecedent respiratory, gastrointestinal, or systemic
https://www.uptodate.com/contents/transverse-myelitis/print?search=…earch_result&selectedTitle=1~104&usage_type=default&display_rank=1 Página 1 de 27
illness [6-12]. In parainfectious TM, the injury may be associated with direct microbial infection of the central
nervous system, or with the systemic response to infection by a variety of agents such as varicella zoster virus,
herpes virus, and Listeria monocytogenes. Molecular mimicry and super antigen-mediated disease have also
been described as potential mechanisms of autoimmunity [4]. Molecular mimicry in TM was postulated to be the
cause of injury following infection with Enterobius vermicularis (pinworm) in a patient who had elevated titers of
cross-reacting antibodies [13]. Microbial super antigens such as staphylococcal enterotoxins A through I, toxic
shock syndrome toxin-1, and streptococcus pyogenes exotoxin, have also been purported to stimulate the
immune system and are known to be capable of activating T-lymphocytes without costimulatory molecules [4,14-
17], thereby triggering autoimmune disease by activating auto-reactive T cell clones [18,19].
The diverse pathology of disease-associated TM is evident from studies showing that lupus-associated TM could
be associated with central nervous system vasculitis or thrombotic infarction of the spinal cord [4,20-24]. Other
studies have also described the role of autoantibodies in patients with neuromyelitis optica and recurrent TM [25-
28]. Autoantibodies have been implicated in activating other components of the immune system by crossing the
blood-brain barrier. The high prevalence of various autoantibodies seen in such patients suggests polyclonal
derangement of the immune system. It may also be that some autoantibodies initiate a direct and selective injury
of neurons that express antigens that cross-react with antibodies directed against infectious pathogens [4].
ASSOCIATED CONDITIONS — Idiopathic TM usually occurs as a postinfectious complication that appears to

result from an autoimmune process. Alternatively, TM can be directly associated with infectious, systemic
inflammatory, or multifocal central nervous system disease.
Acquired central nervous system autoimmune disorders that can cause TM include multiple sclerosis,
neuromyelitis optica, and acute disseminated encephalomyelitis.
● TM can occur as part of the spectrum of multiple sclerosis. In some cases, TM is the initial demyelinating
event (a clinically isolated syndrome [CIS]) that precedes clinically definite multiple sclerosis. (See "Clinical
features of multiple sclerosis in adults" and "Clinically isolated syndromes suggestive of multiple sclerosis".)
● TM manifesting as a longitudinally extensive spinal cord lesion spanning three or more vertebral segments
is one of the characteristic manifestations, along with bilateral optic neuritis, of neuromyelitis optica.
However, neuromyelitis optica can also cause TM involving fewer segments. (See "Neuromyelitis optica
spectrum disorders", section on 'Transverse myelitis'.)
● TM may be seen in patients with acute disseminated encephalomyelitis, a demyelinating disease of the
central nervous system that typically presents as a monophasic disorder with multifocal neurologic
symptoms and encephalopathy. (See "Acute disseminated encephalomyelitis in adults" and "Acute
disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis".)
Other central nervous system conditions that can cause TM are as follows:
● Infections including but not limited to West Nile virus, herpes viruses, HIV, HTLV-1, Zika virus [29], Lyme,
Mycoplasma, and syphilis. In general, infectious causes of spinal cord dysfunction are rare.
● Neurosarcoidosis (see "Neurologic sarcoidosis").
● Paraneoplastic syndromes (see "Paraneoplastic syndromes affecting the spinal cord and dorsal root
ganglia").
Systemic inflammatory autoimmune disorders that are associated with TM include the following:
● Ankylosing spondylitis [30]
● Antiphospholipid antibody syndrome [31]
● Behçet disease
● Mixed connective tissue disease [32]
● Rheumatoid arthritis [33]
● Scleroderma [34]
● Sjögren syndrome [31,35,36]
● Systemic lupus erythematosus [31,37,38]
CLASSIFICATION — Transverse myelitis is an inflammatory disorder that presents with acute or subacute spinal
cord dysfunction resulting in weakness, sensory alterations, and autonomic impairment (eg, bowel, bladder, and
sexual dysfunction) below the level of the lesion [39]. Idiopathic TM is defined by its occurrence without a
definitive etiology despite a thorough work-up. Secondary (disease-associated) TM is most often related to a
systemic inflammatory autoimmune condition.
Subtypes of TM are differentiated on the basis of the clinical severity and radiologic extent of the spinal cord
lesion. These include acute partial TM, acute complete TM and longitudinally extensive TM (LETM).
● Acute partial TM refers to spinal cord dysfunction that is mild or grossly asymmetric with an MRI lesion
extending one to two vertebral segments.
● Acute complete TM refers to spinal cord dysfunction that causes symmetric, complete or near complete
neurologic deficits (paresis, sensory loss, and autonomic dysfunction) below the level of the lesion with an
MRI lesion extending one to two vertebral segments.
● Longitudinally extensive transverse myelitis (LETM) refers to complete or incomplete spinal cord dysfunction
with a lesion on MRI that extends three or more vertebral segments.
These subtypes of TM, while imperfect, imply distinct differential diagnoses and prognoses.
EPIDEMIOLOGY — Although TM is a rare disorder, the reported incidence between one to eight new cases per
million people per year [40] is probably an underestimate. These numbers would imply that approximately 1400
new cases occur in the United States per year and that about 34,000 people have chronic morbidity from TM at
any time [6-8]. There is no gender or familial predisposition to TM, although women predominate among the
cases that are associated with multiple sclerosis [41]. A bimodal peak between the ages of 10 to 19 years and 30
to 39 years has been reported [6-8]. Approximately 20 percent of cases are under the age of 18 years [42]. One
report found a bimodal distribution by age even among patients younger than 18 years of age, and a higher peak
under the age of 3, with no gender predisposition [42]. In the 30 days prior to the onset of TM symptoms, there
was a preceding illness in 47 percent of the children and vaccination in 28 percent. There were no patterns in the
illness or vaccine history that correlated with the acute onset of disease.
In one series of 354 patients with TM, approximately 64 percent of cases were idiopathic and 36 percent were
disease-associated (secondary TM) [1]. In other reports, idiopathic TM accounts for 15 to 30 percent of cases
[39,43,44]. These wide discrepancies in the frequency of idiopathic TM may reflect variation in catchment area
populations, disease definitions, and the evolution of diagnostic methods.
CLINICAL FEATURES — The onset of TM is characterized by acute or subacute development of neurologic

signs and symptoms consistent with motor, sensory and/or autonomic dysfunction. Motor symptoms include a
rapidly progressing paraparesis that can involve the upper extremities, with initial flaccidity followed by spasticity
[1,4,10]. Most patients have a sensory level. In one series of 170 patients with idiopathic TM, spinal MRI T2-
weighted imaging showed a cervical signal abnormality in 44 percent and a thoracic signal abnormality in 37
percent [45]. A thoracic clinical sensory level was identified in 63 percent of the patients. Typical sensory
symptoms are pain, dysesthesia, and paresthesia, although paresthesia are uncommon in children [1,46].
Autonomic symptoms include increased urinary urgency, bladder and bowel incontinence, difficulty or inability to
void, incomplete evacuation and bowel constipation, and sexual dysfunction [1,47-49]. The onset of urinary
retention may be the first sign of myelitis and should always raise the possibility of a myelopathy.
In a series of 47 children with TM, the mean time to nadir from the onset of acute symptoms was about two days
[42]. Common symptoms included sensory loss or numbness, weakness, urinary dysfunction, and pain, as
reported in 91, 89, 85, and 75 percent of children, respectively [42]. Most (89 percent) of the children were bed
bound or wheelchair bound in the initial phase of TM. Also noted was the high proportion of children (53 percent)
that had a thoracic clinical sensory level [42].
In an adult case series of 170 patients with idiopathic TM [45], the rostral-caudal extent of the lesion ranged from
one vertebral segment in many to the entire spinal cord in two patients. A similar pattern was seen in children
[42], with the average lesion spanning six segments.
MRI of the spinal cord typically shows a gadolinium-enhancing signal abnormality (image 1), usually extending
over one or more cord segments [6,42,50,51]. The cord often appears swollen at the affected levels.
Cerebrospinal fluid (CSF) is abnormal in approximately one-half of patients, with a moderate lymphocytosis
(typically <100/mm3) and an elevated protein level (usually 100 to 120 mg/dL). Glucose levels are normal.
Oligoclonal bands are usually not present in isolated TM, and when present suggest a higher risk of subsequent
MS [6,52]. In a case series of 170 adult patients with idiopathic TM, the mean CSF white blood cell count was
38/mm3 and mean protein level was 75 mg/dL (0.75 g/L) [45]. However, higher levels were observed in a
pediatric case series, where the mean white cell count was 136/mm3 and the mean protein level was 173 mg/dL
(1.73 g/L) [42].
DIAGNOSIS — The diagnosis of TM is suspected when there are acute or subacute signs and symptoms of
motor, sensory, and/or autonomic dysfunction that localize to one or more contiguous spinal cord segments in
patients with no evidence of a compressive cord lesion. Thus, the diagnosis of TM requires exclusion of a
compressive cord lesion, usually by MRI, and confirmation of inflammation by either gadolinium-enhanced MRI
or lumbar puncture.
Diagnostic criteria — Diagnostic criteria for TM (table 1) include the following requirements [43,53]:
● Sensory, motor, or autonomic dysfunction attributable to the spinal cord
● Bilateral signs and/or symptoms
● Clearly defined sensory level
● No evidence of compressive cord lesion
● Inflammation defined by cerebrospinal fluid pleocytosis, elevated IgG index, or gadolinium enhancement on
MRI
● Progression to nadir between 4 hours and 21 days
These criteria are useful for defining populations for clinical trials, but some patients presenting with TM may not
fulfill all of the above criteria. As an example, a significant percentage of individuals with a clinical pattern that
otherwise resembles TM do not meet the inflammatory features; therefore, the absence of inflammatory markers
does not rule out TM [31].
Evaluation — The diagnostic approach to acute myelopathy proposed here (algorithm 1) is based upon earlier
work [43,53] and emphasizes the determination of distinct entities that are likely to have different treatment
options, risk of recurrence and prognoses. The first step is to determine from the history and examination (see
'Clinical features' above) whether the condition is likely to be a myelopathy.
When myelopathy is suspected, emergent spinal imaging is warranted to exclude a compressive etiology. An
MRI of the spine is the preferred diagnostic study, but a spine CT or CT myelogram are reasonable alternatives if
an MRI cannot be obtained immediately.
If a compressive myelopathy is ruled out, the clinician should determine whether the myelopathy is inflammatory
or noninflammatory. The best surrogate markers for inflammation are a 'hot' cerebrospinal fluid (ie, with
pleocytosis and/or an elevated IgG index) or a 'hot' spinal MRI (ie, with positive gadolinium enhancement). When
inflammation is present in the absence of cord compression, then the criteria for TM (table 1) have been met,
and it is necessary to evaluate for the presence of infection, systemic inflammation, and the extent and sites of
central nervous system inflammation (table 2).
Investigations — The following investigations are recommended for the evaluation of all patients with suspected
TM [39]:
● MRI of the entire spine, with and without gadolinium, to evaluate for compressive versus noncompressive
cord lesion(s)
● Brain MRI with and without gadolinium to evaluate for the presence of brain lesions suggestive of multiple
sclerosis
● Cerebrospinal fluid analysis including cell count and differential, protein, glucose, VDRL, oligoclonal bands,
immunoglobulin G index, and cytology
● Serum NMO-IgG antibodies (anti-aquaporin-4 IgG), serum B12, methylmalonic acid, human
immunodeficiency antibodies, syphilis serologies, serum ANA, Ro/SSA, and La/SSB antibodies, and thyroid
stimulating hormone
For patients with longitudinally extensive spinal cord lesions, the following tests are additionally recommended
[39]:
● Serum erythrocyte sedimentation rate, C reactive protein, antinuclear antibodies, antibodies to extractable
nuclear antigen, rheumatoid factor, antiphospholipid antibodies, and antineutrophil cytoplasmic antibodies
● Chest CT to evaluate for evidence of sarcoidosis
Select patients may need additional testing [39]:
● Neuro-ophthalmologic evaluation
● Paraneoplastic panel
● Infectious serologies and cerebrospinal fluid studies
● Serum copper and ceruloplasmin
● Serum vitamin E level
● Spinal angiogram
● Prothrombotic evaluation
● Electrodiagnostic evaluation (somatosensory evoked potentials, nerve conduction studies,

electromyography)
● Salivary gland biopsy
DIFFERENTIAL DIAGNOSIS — The main considerations in the differential diagnosis of idiopathic TM are
conditions that cause other types of myelopathy (eg, compressive or noninflammatory), the various disorders that
cause secondary TM, and nonmyelopathic disorders that may mimic TM (eg, Guillain-Barré syndrome).
Other types of myelopathy — Patients with acute myelopathy may be recognized by a constellation of motor,
sensory, and autonomic dysfunction, usually with myelopathic signs such as changes in urinary function and/or a
sensory level (table 3). MRI of the entire spine is important in the evaluation and diagnosis of acute myelopathy
and in the distinction between different types of myelopathy (table 4), particularly for distinguishing a
noncompressive inflammatory lesion from a structural abnormality or mass lesion causing spinal cord
compression. Imaging of the entire spinal cord is indicated, even in patients with paraplegia, due to the potential
for cervical cord lesions to cause isolated lower extremity symptoms.
Disc herniations, epidural masses or blood, vertebral body compression fractures, and spondylosis are among
the most common causes of compressive myelopathy. They may present without overt evidence or history of
trauma. Identifying these disorders is critical since immobilization to prevent further injury, neurosurgical
intervention, and/or high dose methylprednisolone may be warranted in certain cases. Therefore, emergent
spinal cord imaging is indicated. Tuberculosis of the spine is another potential cause of compressive myelopathy,
and is more common in developing countries than in the United States.
Noninflammatory conditions that may mimic TM include the following [39]:
● Vascular myelopathies
• Anterior spinal artery infarction
• Spinal-dural arteriovenous fistula
• Fibrocartilaginous embolism
● Metabolic and nutritional myelopathies
• Vitamin B12 deficiency
• Vitamin D deficiency
• Vitamin E deficiency
• Copper deficiency
• Nitrous oxide toxicity
• Neurolathyrism and neurocassavism
● Neoplasms
• Intramedullary primary spinal cord tumor
• Primary central nervous system lymphoma
• Intravascular lymphoma
● Radiation myelitis
Secondary transverse myelitis — When an inflammatory myelopathy is confirmed, it is important to distinguish

idiopathic TM from secondary (disease-associated) TM, such as a rare infection of the nervous system (eg, West
Nile virus, herpes, central nervous system Lyme disease, mycoplasma), a systemic rheumatologic disease (eg,
systemic lupus erythematosus, Sjögren syndrome), a paraneoplastic syndrome, or a multifocal neurologic
disease (eg, multiple sclerosis, neuromyelitis optica, acute disseminated encephalomyelopathy).
Although rare, a central nervous system infection, such as herpes virus, mycoplasma, brucella or Lyme disease,
may be suspected with certain clinical or paraclinical factors, such as fever, meningismus, vesicular rash,
adenopathy, or serologic or molecular identification of an infectious agent (table 2).
A systemic inflammatory disorder, such as systemic lupus erythematosus, sarcoidosis, or Sjögren syndrome,
should be considered if the patient has certain clinical or paraclinical findings such as malar rash, livedo
reticularis, serositis, night sweats, oral ulcers, or autoimmune antibodies (table 2). Clinicians should remember
that conditions that cause systemic inflammation can present with TM as an initial event.
The importance of defining whether the TM is idiopathic or disease-associated is that the prognosis, acute and
chronic treatments, and risks of recurrence are different between those groups. As an example, TM associated
with active systemic lupus erythematosus could be associated with antiphospholipid antibodies or active
vasculitic lupus, requiring treatment with anticoagulation or immunosuppressive therapy, respectively.
Clinical and imaging evidence of multifocal involvement within the central nervous system raise suspicion for TM
associated with multiple sclerosis, neuromyelitis optica, or acute disseminated encephalomyelitis (table 2).
Neuromyelitis optica involves primarily, though not exclusively, the optic nerve and the spinal cord; patients with
neuromyelitis optica typically have longitudinally extensive spinal cord lesions [54].
Guillain-Barré syndrome — Patients with acute inflammatory demyelinating polyneuropathy (AIDP, Guillain-
Barré syndrome) may also present with progressive sensory and motor dysfunction [55]. However, several
clinical and paraclinical features may be used to rapidly discriminate patients with AIDP from those with acute
myelopathies (table 5). First, patients with AIDP often have both upper and lower extremity involvement, though
the lower extremity involvement is usually more severe. By contrast, patients with myelopathy may have only
lower extremity involvement if the myelopathy is thoracic, or equivalent upper and lower extremity involvement if
the myelopathy is cervical. Similarly, autonomic involvement differs between patients with AIDP and myelopathy.
Patients with myelopathy are more likely to have urinary or bowel urgency or retention, while those with AIDP are
more likely to have cardiovascular instability. Every attempt should be made to determine a neuropathic versus
myelopathic pattern of sensory loss, since a sensory level is often definable in patients with acute myelopathy
but is never present in AIDP. Cerebrospinal fluid analysis in AIDP usually shows an elevated protein with few
white cells (ie, cyto-albuminologic dissociation) whereas patients with TM may have an inflammatory
cerebrospinal fluid with an elevated number of white blood cells and IgG index. Spinal MRI imaging often shows
a discrete lesion in myelopathy, whereas spinal MRI is normal in AIDP. Finally, electrodiagnostic studies may
show conduction block or slowed conduction of peripheral nerves in AIDP and are usually (though not always)
normal in myelopathies.
TREATMENT — Intravenous glucocorticoids have long been considered the standard of care and first-line
therapy in acute idiopathic TM. Even without placebo-controlled trials evaluating glucocorticoids specifically in
TM [41], there is good evidence that intravenous glucocorticoids are effective in acute inflammatory central
nervous system diseases like TM, such as multiple sclerosis. (See "Treatment of acute exacerbations of multiple
sclerosis in adults", section on 'Glucocorticoids'.)
Therefore, we suggest high-dose intravenous glucocorticoid treatment for adult and adolescent patients with
acute idiopathic TM. Our preferred regimens are methylprednisolone (1000 mg daily) or dexamethasone (200 mg
daily) for three to five days. Continued treatment with glucocorticoids or more aggressive regimens is based
upon the clinical course and radiologic parameters.
Plasma exchange may be effective for acute central nervous system demyelinating diseases that fail to respond
to high-dose glucocorticoid treatment [56-58]. Thus, in addition to high-dose glucocorticoid therapy, we suggest
treatment with plasma exchange for patients who have acute TM with motor impairment [59]. Our preferred
regimen is five treatments, each with exchanges of 1.1 to 1.5 plasma volumes, every other day for 10 days [60].
For patients with significant deficits, waiting until glucocorticoid treatments are completed is not necessary.
Clinical judgment must be used and some patients may benefit from earlier intervention with plasma exchange.
In the clinical experience of the authors, intravenous cyclophosphamide (800 to 1200 mg/m2 administered as a
single pulse dose) for patients with aggressive TM has been associated with good outcomes. In a retrospective
study, all 122 patients with idiopathic TM were treated with three to five days of intravenous methylprednisone,
either alone (n = 66) or in combination with plasma exchange (n = 32), cyclophosphamide (n = 13), or both
plasma exchange and cyclophosphamide (n = 11) [61]. In a subset of patients with systemic autoimmune
disease (eg, systemic lupus erythematosus), the addition of cyclophosphamide to the treatment regimen
improved outcomes. In the absence of systemic autoimmune disease, plasma exchange plus methylprednisone
was superior to methylprednisone alone. Given the methodologic limitations of this small, uncontrolled
observational study, these findings require confirmation in randomized controlled trials.
For patients with recurrent disease, chronic immunomodulatory therapy must be considered. We most commonly
treat patients with azathioprine (150 to 250 mg/daily), methotrexate (15 to 20 mg/week) or mycophenolate (2 to 3
gm/daily), though oral cyclophosphamide (2 g/kg per day) may also be used in patients with systemic
inflammatory disease [4].
Ultimately, it may be possible both to specifically and effectively modulate the inflammation causing TM and to
provide neuroprotection against the immune-mediated assault that occurs with TM.
PROGNOSIS — Most patients with idiopathic TM have at least a partial recovery, which usually begins within
one to three months, and continues with exercise and rehabilitation therapy [62]. Recovery can proceed over
years. Some degree of persistent disability is common, occurring in about 40 percent [42,62]. A very rapid onset
with complete paraplegia and spinal shock has been associated with poorer outcomes [31,62,63].
Research is needed to define biomarkers of disease that predict outcome and risk of recurrence.
Monophasic versus recurrent disease — The majority of patients with TM experience monophasic disease.
Recurrence has been reported in approximately 25 to 33 percent of patients with idiopathic TM [64-66]. In
addition, TM can recur in a subset of patients with a history of systemic autoimmune disease. With disease-
associated TM, the recurrence rate may be as high as 70 percent [6,40,44].
Features present at the time of initial acute onset that may predict recurrence (table 6) include the following
[1,66]:
● Multifocal or longitudinally extensive lesions in the spinal cord on MRI
● Brain lesions on MRI
● Presence of one or more autoantibodies (ANA, dsDNA, phospholipid, c-ANCA)
● Underlying mixed connective tissue disease
● Presence of oligoclonal bands in the cerebrospinal fluid
● Seropositivity for NMO-IgG (anti-aquaporin-4) antibody
In patients with the severe complete acute transverse myelitis, brain MRI is considered to be negative in this
population, essentially by definition. These patients appear less likely to present with oligoclonal bands, less
likely to relapse with a second bout of myelitis, and have a low transition rate to clinically definite multiple
sclerosis. (See 'Progression to multiple sclerosis' below.)
Progression to multiple sclerosis — Patients presenting with acute complete transverse myelitis (complete or
near complete clinical deficits below the lesion) have a generally cited risk of multiple sclerosis of only 5 to 10
percent [63,67], although some reports suggest a higher conversion rate [68]. However, partial or incomplete
myelitis with mild or grossly asymmetric spinal cord dysfunction is a more common clinical entity and bears
greater relevance to multiple sclerosis. Patients who have acute partial myelitis as an initial presentation and
cranial MRI abnormalities showing lesions typical for multiple sclerosis have a transition rate to multiple sclerosis
over three to five years of 60 to 90 percent [68-70]. In contrast, patients with acute partial myelitis who have a
normal brain MRI develop multiple sclerosis at a rate of only 10 to 30 percent over a similar time period [71].
Cerebrospinal fluid (CSF) studies suggest that patients with monosymptomatic disease and positive oligoclonal
bands (OCBs) have a higher risk of evolution to multiple sclerosis than those without OCBs [72], although CSF
results do not help further in prognosis when compared with MRI alone.
SUMMARY AND RECOMMENDATIONS
● Acute transverse myelitis (TM) is a neuro-inflammatory spinal cord disorder that presents with the rapid
onset of weakness, sensory alterations, and bowel and bladder dysfunction.
● Idiopathic TM is defined by its occurrence without a definitive etiology despite a thorough work-up.
Secondary (disease-associated) TM is most often related to a systemic inflammatory autoimmune condition.
Idiopathic TM usually occurs as a postinfectious complication and presumably results from an autoimmune
process. Alternatively, TM can be associated with infectious, systemic inflammatory, or multifocal central
nervous system disease. Acquired central nervous system demyelinating disorders that can cause TM
include multiple sclerosis, neuromyelitis optica, and acute disseminated encephalomyelitis. (See 'Associated
conditions' above and 'Classification' above.)
● TM is rare, with an annual incidence of one to eight new cases per million. (See 'Epidemiology' above.)
● The onset of TM is characterized by acute or subacute development of neurologic signs and symptoms
consistent with motor, sensory, and/or autonomic dysfunction. Motor symptoms include a rapidly progressing
paraparesis that can involve the upper extremities, with initial flaccidity followed by spasticity. In most
patients, a sensory level can be identified. Sensory symptoms include pain, dysesthesia and paresthesia.
Autonomic symptoms involve increased urinary urgency, bladder and bowel incontinence, difficulty or
inability to void, incomplete evacuation and bowel constipation, and sexual dysfunction. (See 'Clinical
features' above.)
https://www.uptodate.com/contents/transverse-myelitis/print?search…earch_result&selectedTitle=1~104&usage_type=default&display_rank=1 Página 10 de 27
● The diagnosis of TM is suspected when there are acute or subacute signs and symptoms of motor, sensory
and/or autonomic dysfunction that localize to one or more contiguous spinal cord segments in patients with
no evidence of a compressive cord lesion. Thus, the diagnosis of TM requires exclusion of a compressive
cord lesion, usually by MRI, and confirmation of inflammation by either gadolinium-enhanced MRI or lumbar
puncture (algorithm 1). When inflammation is present in the absence of cord compression, then the criteria
for TM (table 1) have been met, and it is necessary to evaluate for the presence of infection, systemic
inflammation, and the extent and sites central nervous system inflammation (table 2).
● The main considerations in the differential diagnosis of idiopathic TM are conditions that cause other types
of myelopathy (eg, compressive or noninflammatory), the various disorders that cause secondary TM, and
nonmyelopathic disorders that may mimic TM (eg, Guillain-Barré syndrome). (See 'Differential diagnosis'
above.)
● For adult and adolescent patients with acute idiopathic TM, we suggest high-dose intravenous glucocorticoid
treatment (Grade 2C). Our preferred regimens are methylprednisolone (1000 mg daily) or dexamethasone
(200 mg daily) for three to five days. For patients with acute TM complicated by motor impairment, we
suggest additional treatment with plasma exchange (Grade 2C). Our preferred regimen is five treatments,
each with exchanges of 1.1 to 1.5 plasma volumes, every other day for 10 days. (See 'Treatment' above.)
● Most patients with idiopathic TM have at least a partial recovery, which usually begins within one to three
months and continues with exercise and rehabilitation therapy. Some degree of persistent disability is
common, occurring in about 40 percent. A very rapid onset with complete paraplegia and spinal shock has
been associated with poorer outcomes. Recovery can proceed over years. (See 'Prognosis' above.)
● The majority of patients with TM experience monophasic disease. Recurrence has been reported in
approximately 25 to 33 percent of patients with idiopathic TM. With disease-associated (secondary) TM, the
recurrence rate may be as high as 70 percent. (See 'Monophasic versus recurrent disease' above.)
● Patients presenting with acute complete transverse myelitis have a generally cited risk of multiple sclerosis
of only 5 to 10 percent. However, patients with partial myelitis as an initial presentation and cranial MRI
abnormalities showing lesions typical for multiple sclerosis have a transition rate to multiple sclerosis over
three to five years of 60 to 90 percent. In contrast, patients with acute partial myelitis who have a normal
brain MRI develop multiple sclerosis at a rate of only 10 to 30 percent over a similar time period. (See
'Progression to multiple sclerosis' above.)
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Douglas Kerr, MD, who
contributed to earlier versions of this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.
REFERENCES
1. Krishnan C, Kaplin AI, Pardo CA, et al. Demyelinating disorders: update on transverse myelitis. Curr Neurol
Neurosci Rep 2006; 6:236.
2. Patja A, Paunio M, Kinnunen E, et al. Risk of Guillain-Barré syndrome after measles-mumps-rubella

vaccination. J Pediatr 2001; 138:250.
3. Schonberger LB, Bregman DJ, Sullivan-Bolyai JZ, et al. Guillain-Barre syndrome following vaccination in
the National Influenza Immunization Program, United States, 1976--1977. Am J Epidemiol 1979; 110:105.
4. Kaplin AI, Krishnan C, Deshpande DM, et al. Diagnosis and management of acute myelopathies.
Neurologist 2005; 11:2.
5. Baxter R, Lewis E, Goddard K, et al. Acute Demyelinating Events Following Vaccines: A Case-Centered
Analysis. Clin Infect Dis 2016; 63:1456.
6. Jeffery DR, Mandler RN, Davis LE. Transverse myelitis. Retrospective analysis of 33 cases, with
differentiation of cases associated with multiple sclerosis and parainfectious events. Arch Neurol 1993;
50:532.
7. Christensen PB, Wermuth L, Hinge HH, Bømers K. Clinical course and long-term prognosis of acute
transverse myelopathy. Acta Neurol Scand 1990; 81:431.
8. ALTROCCHI PH. ACUTE TRANSVERSE MYELOPATHY. Arch Neurol 1963; 9:111.
9. Lipton HL, Teasdall RD. Acute transverse myelopathy in adults. A follow-up study. Arch Neurol 1973;
28:252.
10. Ropper AH, Poskanzer DC. The prognosis of acute and subacute transverse myelopathy based on early
signs and symptoms. Ann Neurol 1978; 4:51.
11. Poulter MO, Payne KB, Steiner JP. Neuroimmunophilins: a novel drug therapy for the reversal of
neurodegenerative disease? Neuroscience 2004; 128:1.
12. PAINE RS, BYERS RK. Transverse myelopathy in childhood. AMA Am J Dis Child 1953; 85:151.
13. Brocke S, Hausmann S, Steinman L, Wucherpfennig KW. Microbial peptides and superantigens in the
pathogenesis of autoimmune diseases of the central nervous system. Semin Immunol 1998; 10:57.
14. Bohach GA. Staphylococcal enterotoxins B and C. Structural requirements for superantigenic and
entertoxigenic activities. Prep Biochem Biotechnol 1997; 27:79.
15. Bohach GA, Fast DJ, Nelson RD, Schlievert PM. Staphylococcal and streptococcal pyrogenic toxins
involved in toxic shock syndrome and related illnesses. Crit Rev Microbiol 1990; 17:251.
16. Betley MJ, Borst DW, Regassa LB. Staphylococcal enterotoxins, toxic shock syndrome toxin and
streptococcal pyrogenic exotoxins: a comparative study of their molecular biology. Chem Immunol 1992;
55:1.
17. Zhang J, Vandevyver C, Stinissen P, et al. Activation and clonal expansion of human myelin basic protein-
reactive T cells by bacterial superantigens. J Autoimmun 1995; 8:615.
18. Kotzin BL, Leung DY, Kappler J, Marrack P. Superantigens and their potential role in human disease. Adv
Immunol 1993; 54:99.
19. Vanderlugt CL, Begolka WS, Neville KL, et al. The functional significance of epitope spreading and its
regulation by co-stimulatory molecules. Immunol Rev 1998; 164:63.
20. PIPER PG. Disseminated lupus erythematosus with involvement of the spinal cord. J Am Med Assoc 1953;
153:215.
21. Andrianakos AA, Duffy J, Suzuki M, Sharp JT. Transverse myelopathy in systemic lupus erythematosus.
Report of three cases and review of the literature. Ann Intern Med 1975; 83:616.
22. Nakano I, Mannen T, Mizutani T, Yokohari R. Peripheral white matter lesions of the spinal cord with
changes in small arachnoid arteries in systemic lupus erythematosus. Clin Neuropathol 1989; 8:102.
23. Sinkovics JG, Gyorkey F, Thoma GW. A rapidly fatal case of systemic lupus erythematosus: structures
resembling viral nucleoprotein strands in the kidney and activities of lymphocytes in culture. Tex Rep Biol
Med 1969; 27:887.
24. WEIL MH. Disseminated lupus erythematosus with massive hemorrhagic manifestations and paraplegia. J
Lancet 1955; 75:358.
25. Haase CG, Schmidt S. Detection of brain-specific autoantibodies to myelin oligodendrocyte glycoprotein,
S100beta and myelin basic protein in patients with Devic's neuromyelitis optica. Neurosci Lett 2001;
307:131.
26. Pandit L, Rao S. Recurrent myelitis. J Neurol Neurosurg Psychiatry 1996; 60:336.
27. Tippett DS, Fishman PS, Panitch HS. Relapsing transverse myelitis. Neurology 1991; 41:703.
28. Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica:
distinction from multiple sclerosis. Lancet 2004; 364:2106.
29. Mécharles S, Herrmann C, Poullain P, et al. Acute myelitis due to Zika virus infection. Lancet 2016;
387:1481.
30. Oh DH, Jun JB, Kim HT, et al. Transverse myelitis in a patient with long-standing ankylosing spondylitis.
Clin Exp Rheumatol 2001; 19:195.
31. de Seze J, Lanctin C, Lebrun C, et al. Idiopathic acute transverse myelitis: application of the recent
diagnostic criteria. Neurology 2005; 65:1950.
32. Mok CC, Lau CS. Transverse myelopathy complicating mixed connective tissue disease. Clin Neurol
Neurosurg 1995; 97:259.
33. Harzheim M, Schlegel U, Urbach H, et al. Discriminatory features of acute transverse myelitis: a
retrospective analysis of 45 patients. J Neurol Sci 2004; 217:217.
34. Torabi AM, Patel RK, Wolfe GI, et al. Transverse myelitis in systemic sclerosis. Arch Neurol 2004; 61:126.
35. Anantharaju A, Baluch M, Van Thiel DH. Transverse myelitis occurring in association with primary biliary
cirrhosis and Sjogren's syndrome. Dig Dis Sci 2003; 48:830.
36. Rabadi MH, Kundi S, Brett D, Padmanabhan R. Neurological pictures. Primary Sjögren syndrome
presenting as neuromyelitis optica. J Neurol Neurosurg Psychiatry 2010; 81:213.
37. Lehnhardt FG, Impekoven P, Rubbert A, et al. Recurrent longitudinal myelitis as primary manifestation of
SLE. Neurology 2004; 63:1976.
38. Krishnan AV, Halmagyi GM. Acute transverse myelitis in SLE. Neurology 2004; 62:2087.
39. Beh SC, Greenberg BM, Frohman T, Frohman EM. Transverse myelitis. Neurol Clin 2013; 31:79.
40. Berman M, Feldman S, Alter M, et al. Acute transverse myelitis: incidence and etiologic considerations.
Neurology 1981; 31:966.
41. Scott TF, Frohman EM, De Seze J, et al. Evidence-based guideline: clinical evaluation and treatment of
transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American
Academy of Neurology. Neurology 2011; 77:2128.
42. Pidcock FS, Krishnan C, Crawford TO, et al. Acute transverse myelitis in childhood: center-based analysis
of 47 cases. Neurology 2007; 68:1474.
43. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute
transverse myelitis. Neurology 2002; 59:499.
44. de Seze J, Stojkovic T, Breteau G, et al. Acute myelopathies: Clinical, laboratory and outcome profiles in 79
cases. Brain 2001; 124:1509.
45. Krishnan C, Kaplin AI, Calabresi P, Kerr DA. Clinical characteristics and prognostic factors in 170 patients
with idiopathic transverse myelitis. Neurology 2004; 62 (Suppl 5):A231.
46. Dunne K, Hopkins IJ, Shield LK. Acute transverse myelopathy in childhood. Dev Med Child Neurol 1986;
28:198.
47. Sakakibara R, Hattori T, Yasuda K, Yamanishi T. Micturition disturbance in acute transverse myelitis. Spinal
Cord 1996; 34:481.
48. Burns AS, Rivas DA, Ditunno JF. The management of neurogenic bladder and sexual dysfunction after
spinal cord injury. Spine (Phila Pa 1976) 2001; 26:S129.
49. DasGupta R, Fowler CJ. Sexual and urological dysfunction in multiple sclerosis: better understanding and
improved therapies. Curr Opin Neurol 2002; 15:271.
50. Bakshi R, Kinkel PR, Mechtler LL, et al. Magnetic resonance imaging findings in 22 cases of myelitis:
comparison between patients with and without multiple sclerosis. Eur J Neurol 1998; 5:35.
51. Choi KH, Lee KS, Chung SO, et al. Idiopathic transverse myelitis: MR characteristics. AJNR Am J
Neuroradiol 1996; 17:1151.
52. Cordonnier C, de Seze J, Breteau G, et al. Prospective study of patients presenting with acute partial
transverse myelopathy. J Neurol 2003; 250:1447.
53. Berger JR, Cambi F, Di Rocco A, Farace J. Overview to approach to the patient with noncompressive
myelopathy. Continuum (Minneap Minn) 2005; 11:13.
54. Wingerchuk DM, Lennon VA, Pittock SJ, et al. Revised diagnostic criteria for neuromyelitis optica.
Neurology 2006; 66:1485.
55. Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse myelitis overview and differential diagnosis. J Child
Neurol 2012; 27:1426.
56. Weinshenker BG, O'Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central
nervous system inflammatory demyelinating disease. Ann Neurol 1999; 46:878.
57. Cortese I, Chaudhry V, So YT, et al. Evidence-based guideline update: Plasmapheresis in neurologic
disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American
Academy of Neurology. Neurology 2011; 76:294.
58. Bigi S, Banwell B, Yeh EA. Outcomes after early administration of plasma exchange in pediatric central
nervous system inflammatory demyelination. J Child Neurol 2015; 30:874.
59. Greenberg BM. Treatment of acute transverse myelitis and its early complications. Continuum (Minneap
Minn) 2011; 17:733.
60. Gwathmey K, Balogun RA, Burns T. Neurologic indications for therapeutic plasma exchange: an update. J
Clin Apher 2011; 26:261.
61. Greenberg BM, Thomas KP, Krishnan C, et al. Idiopathic transverse myelitis: corticosteroids, plasma
exchange, or cyclophosphamide. Neurology 2007; 68:1614.
62. Defresne P, Hollenberg H, Husson B, et al. Acute transverse myelitis in children: clinical course and
prognostic factors. J Child Neurol 2003; 18:401.
63. Bruna J, Martínez-Yélamos S, Martínez-Yélamos A, et al. Idiopathic acute transverse myelitis: a clinical
study and prognostic markers in 45 cases. Mult Scler 2006; 12:169.
64. Seifert T, Enzinger C, Ropele S, et al. Relapsing acute transverse myelitis: a specific entity. Eur J Neurol
2005; 12:681.
65. Kim KK. Idiopathic recurrent transverse myelitis. Arch Neurol 2003; 60:1290.
66. Borchers AT, Gershwin ME. Transverse myelitis. Autoimmun Rev 2012; 11:231.
67. Chen L, Li J, Guo Z, et al. Prognostic indicators of acute transverse myelitis in 39 children. Pediatr Neurol
2013; 49:397.
68. Gajofatto A, Monaco S, Fiorini M, et al. Assessment of outcome predictors in first-episode acute myelitis: a
retrospective study of 53 cases. Arch Neurol 2010; 67:724.
69. Morrissey SP, Miller DH, Kendall BE, et al. The significance of brain magnetic resonance imaging
abnormalities at presentation with clinically isolated syndromes suggestive of multiple sclerosis. A 5-year
follow-up study. Brain 1993; 116 ( Pt 1):135.
70. Ford B, Tampieri D, Francis G. Long-term follow-up of acute partial transverse myelopathy. Neurology
1992; 42:250.
71. Scott TF, Kassab SL, Singh S. Acute partial transverse myelitis with normal cerebral magnetic resonance
imaging: transition rate to clinically definite multiple sclerosis. Mult Scler 2005; 11:373.
72. Bashir K, Whitaker JN. Importance of paraclinical and CSF studies in the diagnosis of MS in patients
presenting with partial cervical transverse myelopathy and negative cranial MRI. Mult Scler 2000; 6:312.
Topic 14088 Version 11.0
GRAPHICS
Spinal cord MRI demonstrating transverse myelitis in a

37-year-old man with multiple sclerosis
T2-weighted sagittal (A) and axial (B) images show a focus of hyperintensity
in the posterior columns of the cervical spinal cord at the C2 level. Post-
gadolinium T1-weighted sagittal (C) and axial (D) images demonstrate
enhancement consistent with an active plaque.
MRI: magnetic resonance imaging.
Graphic 73197 Version 7.0
Diagnostic criteria for transverse myelitis
Sensory, motor or autonomic dysfunction attributable to the spinal cord
Bilateral signs and/or symptoms
Clearly defined sensory level
No evidence of compressive cord lesion
Inflammation defined by cerebrospinal fluid pleocytosis or elevated IgG index or gadolinium enhancement
Progression to nadir between four hours and 21 days
Evaluation of suspected acute myelopathy
Modified with permission from: Berger JR, Cambi F, Di Rocco A, Farace J. Overview to approach to the patient
with noncompressive myelopathy. Continuum (Minneap Minn) 2005; 11:13. DOI:

10.1212/01.CON.0000293718.40497.96. Copyright © 2013 American Academy of Neurology. Unauthorized
reproduction of this material is prohibited.
Potential medical work-up for suspected acute transverse myelitis
Indicative signs and symptoms Suggested evaluation
Infectious etiology
Fever CSF Gram stain and bacterial culture
Meningismus CSF PCR: HSV-1, HSV-2, HHV-6, VZV, CMV, EBV,

enteroviruses
Rash CSF viral culture
Concurrent systemic infection CSF acid-fast bacilli smear and tuberculous culture
Immunocompromised state CSF HSV, VZV, and HTLV-1 antibodies
Recurrent genital infection CSF anti-Borrelia burgdorferi antibodies
Symptoms of zoster radiculopathy CSF VDRL
Adenopathy CSF India ink and fungal culture
Residence in area endemic for parasitic infections Chest radiograph
Lymphadenopathy Serology for antibodies to HIV, HSV, VZV, HTLV-1, B.

burgdorferi
Serology for hepatitis A, B, C, and Mycoplasma
Consider serology for parasites
Blood cultures
Systemic inflammatory disease (vasculitis, collagen vascular diseases, mixed connective tissue
disease)
Rash Serum ACE
Oral or genital ulcers Auto-antibodies: ANA, ds-DNA, Ro/SSA, La/SSB, Sm,

RNP
Adenopathy Complement levels
Livedo reticularis Urinalysis with microscopic analysis for hematuria
Serositis Lip/salivary gland biopsy
Photosensitivity Chest CT with intravenous contrast
Inflammatory arthritis Schirmer test
Erythema nodosum Chest radiograph
Xerostomia Gallium scan
Keratitis Antiphospholipid antibodies (anticardiolipin antibodies,

Russel viper venom time, partial thromboplastin time)
Conjunctivitis
Contractures or thickening of skin
Anemia/leukopenia/thrombocytopenia
Raynaud phenomenon
History of arterial and venous thrombosis
Multiple sclerosis
Previous demyelination event Brain MRI
Incomplete deficit clinically with MRI abnormality ≤2 Evoked potentials

spinal segments and <50 percent of cord diameter
CSF oligoclonal bands and IgG index
Neuromyelitis optica (Devic's disease)
Optic neuritis Evoked potentials
Clinical deficit with MRI abnormality ≥3 spinal Brain MRI (usually negative)
segments
NMO-IgG testing
Idiopathic transverse myelitis
No clinical or paraclinical features suggestive of Evoked potentials

another diagnostic category
Electromyography/nerve conduction velocity
ACE: angiotensin-converting enzyme; ANA: anti-nuclear antibodies; CMV: cytomegalovirus; CSF: cerebrospinal fluid;
EBV: Epstein-Barr virus; HHV: human herpes virus; HIV: humam immunodeficiency virus; HSV: herpes simplex virus;
HTLV-1: human T-cell lymphotropic virus 1; IgG: immunoglobulin G; NMO-IgG: neuromyelitis optica IgG autoantibody;
VDRL: Veneral Disease Research Laboratory; VZV: varicella zoster virus.
Modified with permission from: Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and
nosology of acute transverse myelitis. Neurology 2002; 59:499. Copyright © 2002 Lippincott Williams & Wilkins.
Clinical features suggestive of acute myelopathies
Clinical features Potential diagnosis
Acute paraparesis with sensory level and/or autonomic Acute myelopathy

involvement
History of thrombosis Arterial or venous ischemic infarct
Male, >40 years old with stuttering or downhill Venous hypertension, dural arteriovenous fistula
myelopathy over months
Known tuberculosis Vertebral compression fracture or central nervous

system tuberculosis
History of or concurrent optic neuritis Neuromyelitis optica
History of or concurrent systemic rheumatologic Transverse myelitis associated with rheumatologic

disorder disorder
Prior history of demyelination and mild, mainly sensory Transverse myelitis associated with multiple sclerosis
symptoms
Antecedent trauma (eg, hyperextension) Consider congenital or acquired spinal column stenosis
and compression
Suggestive MRI findings of acute myelopathies
MRI findings Potential diagnosis
Blood within the spinal cord (bright and dark T1 and T2 Vascular malformation such as cavernous angioma or
signal) dural arteriovenous fistula
Flow voids within spinal cord Dural arteriovenous fistula or arteriovenous

malformation
Central T2 signal abnormality Venous hypertension
Ring-enhancing lesion Infection or tumor (but consider course of intravenous

glucocorticoids to rule out inflammatory process before
progressing to biopsy)
Acute loss of vertical intervertebral disc height and Consider fibrocartilaginous embolism
corresponding T2 signal abnormality
Fusiform lesion extending over >3 spinal cord segments Consider neuromyelitis optica or disease-associated
transverse myelitis
T2 bright lesion in white matter occupying less than 2 Consider multiple sclerosis
spinal cord segments in rostral-caudal extent and less
than 50 percent of the cord diameter
T2 spinal cord lesion adjacent to disk herniation or Consider dynamic spinal cord compression only during
spondylitic ridge, but lack of spinal cord compression flexion or extension (flexion-extension x-ray to
determine the presence of abnormal spinal column
mobility; MRI in flexion or extended position instead of
in neutral position)
Distinguishing features between acute inflammatory demyelinating polyneuropathy

(Guillain-Barré syndrome) and transverse myelitis
Guillain-Barré
Characteristics Acute myelitis Distinguishing features
syndrome
Motor findings Paraparesis or Ascending weakness Myelopathy: if UE involvement, often as

quadriparesis LE>UE in the early severe as LE; often no UE involvement
stages
GBS: there usually is UE involvement and it
is less severe than LE involvement early in
the disease
Sensory findings Usually can Ascending sensory Myelopathy: sensory level usually identified;
diagnosis a spinal loss LE>UE in the often no arm involvement
cord level early stages GBS: no sensory level; usually UE less
affected than LE early in the disease
Autonomic findings Early loss of bowel Autonomic Myelopathy: urinary urgency or retention
and bladder control dysfunction of the early and prominent; cardiovascular
cardiovascular instability only in severe cases higher than
system T6 spinal level
GBS: urinary urgency or retention less
common; cardiovascular instability is more
common
Cranial nerve None Extra-ocular muscle GBS: cranial neuropathies are more
findings palsies or facial common than in acute myelopathy
weakness
Electrophysiologic EMG/NCV findings EMG/NCV findings The lack of peripheral nerve abnormalities
findings may be normal or confined to the in a patient with progressive weakness and
may implicate the peripheral nervous sensory loss should suggest evaluation of
spinal cord system: motor the spinal cord for pathology
Prolonged central and/or sensory Conversely, patients with suspected acute
conduction on nerve conduction myelopathy but equivocal clinical,
somatosensory velocity reduced, laboratory, or radiologic findings may
evoked potential distal latencies warrant peripheral nerve examination
prolonged;
(SEP) latencies or
missing SEP in conduction block;
conjunction with reduced H reflex
normal sensory usually present
nerve action
potentials
MRI findings Usually a focal area Normal MRI abnormalities may be helpful in
of increased T2 diagnosing a patient who is suspected of
signal with or having GBS from acute myelopathy
without gadolinium
enhancement
CSF Usually, CSF Usually, elevated CSF pleocytosis and elevated IgG index may
pleocytosis and/or protein in the be helpful in diagnosing a patient who is
increased IgG index absence of CSF suspected of having GBS from acute
pleocytosis myelopathy
Features that predict recurrent transverse myelitis
Tests Monophasic Recurrent
Spinal MRI Single T2 lesion Multiple distinct lesions or fusiform lesion extending over three or
more spinal cord segments
Brain MRI Normal T2/FLAIR abnormalities
Blood serology Normal One or more autoantibodies (ANA, dsDNA, phospholipid, c-ANCA)
SS-A Negative Positive
NMO-IgG Negative Positive
Oligoclonal bands Negative Positive

(CSF)
Systemic disease None Connective tissue disorder
Optic nerve No Likely

involvement
Contributor Disclosures
Chitra Krishnan, MHS Nothing to disclose Benjamin Greenberg, MD, MHS Grant/Research/Clinical Trial
Support: MedImmune; Chugai Pharma [Neuromyelitis optica]; Acorda Therapeutics [Multiple sclerosis
(Dalfampridine)]; Genentech [Multiple sclerosis (ocrelizumab)]. Consultant/Advisory Boards: Novartis [Multiple
sclerosis (Fingolimod)]; Alexion [NMO (Eculizumab)]. Francisco González-Scarano, MD Equity
Ownership/Stock Options: Multiple, but traded by advisors without personal input [Pharmaceutical]. Other
Financial Interests: NeuroLink [Venture Capital]. John F Dashe, MD, PhD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform
to UpToDate standards of evidence.
Conflict of interest policy

Transverse Myelitis - UpToDate

Загружено:

Сведения о документе

Авторское право

Доступные форматы

Поделиться этим документом

Поделиться или встроить документ

Параметры публикации

Этот документ был вам полезен?

Это неприемлемый материал?

Авторское право:

Доступные форматы

Transverse Myelitis - UpToDate

Загружено:

Авторское право:

Доступные форматы

Transverse myelitis - UpToDate 01-05-18 15'35

Official reprint from UpToDate®

Authors: Chitra Krishnan, MHS, Benjamin Greenberg, MD, MHS

In 30 to 60 percent of the idiopathic TM cases, there is an antecedent respiratory, gastrointestinal, or systemic

ASSOCIATED CONDITIONS — Idiopathic TM usually occurs as a postinfectious complication that appears to

● Neurosarcoidosis (see "Neurologic sarcoidosis").

● Ankylosing spondylitis [30]

● Antiphospholipid antibody syndrome [31]

● Mixed connective tissue disease [32]

● Rheumatoid arthritis [33]

● Sjögren syndrome [31,35,36]

● Systemic lupus erythematosus [31,37,38]

CLINICAL FEATURES — The onset of TM is characterized by acute or subacute development of neurologic

● Sensory, motor, or autonomic dysfunction attributable to the spinal cord

● Bilateral signs and/or symptoms

● Clearly defined sensory level

● No evidence of compressive cord lesion

● Progression to nadir between 4 hours and 21 days

● Chest CT to evaluate for evidence of sarcoidosis

Select patients may need additional testing [39]:

● Infectious serologies and cerebrospinal fluid studies

● Serum copper and ceruloplasmin

● Serum vitamin E level

● Electrodiagnostic evaluation (somatosensory evoked potentials, nerve conduction studies,

● Salivary gland biopsy

Noninflammatory conditions that may mimic TM include the following [39]:

• Anterior spinal artery infarction

• Spinal-dural arteriovenous fistula

● Metabolic and nutritional myelopathies

• Vitamin B12 deficiency

• Nitrous oxide toxicity

• Neurolathyrism and neurocassavism

• Intramedullary primary spinal cord tumor

• Primary central nervous system lymphoma

Secondary transverse myelitis — When an inflammatory myelopathy is confirmed, it is important to distinguish

adenopathy, or serologic or molecular identification of an infectious agent (table 2).

● Multifocal or longitudinally extensive lesions in the spinal cord on MRI

● Brain lesions on MRI

● Presence of one or more autoantibodies (ANA, dsDNA, phospholipid, c-ANCA)

● Underlying mixed connective tissue disease

● Presence of oligoclonal bands in the cerebrospinal fluid

● Seropositivity for NMO-IgG (anti-aquaporin-4) antibody

SUMMARY AND RECOMMENDATIONS

Use of UpToDate is subject to the Subscription and License Agreement.

2. Patja A, Paunio M, Kinnunen E, et al. Risk of Guillain-Barré syndrome after measles-mumps-rubella

Topic 14088 Version 11.0

Spinal cord MRI demonstrating transverse myelitis in a

MRI: magnetic resonance imaging.

Graphic 73197 Version 7.0

Diagnostic criteria for transverse myelitis

Sensory, motor or autonomic dysfunction attributable to the spinal cord

Bilateral signs and/or symptoms

Clearly defined sensory level

No evidence of compressive cord lesion

Progression to nadir between four hours and 21 days

Graphic 88914 Version 2.0

Evaluation of suspected acute myelopathy

with noncompressive myelopathy. Continuum (Minneap Minn) 2005; 11:13. DOI:

Graphic 88920 Version 6.0

Potential medical work-up for suspected acute transverse myelitis

Indicative signs and symptoms Suggested evaluation

Fever CSF Gram stain and bacterial culture

Meningismus CSF PCR: HSV-1, HSV-2, HHV-6, VZV, CMV, EBV,

Rash CSF viral culture