Vol.18, No.
10 October 1996
Continuing Education Article
FOCAL POINT
★ Knowledge of spinal cord
diseases in specific canine breeds
can aid the clinician in diagnosis
of neurologic disorders and
formulation of a therapeutic plan.
KEY FACTS
■ Inborn errors of metabolism
result from enzyme deficiencies
that prevent adequate production
or elimination of biochemical
products.
■ Uninterrupted flow of axoplasm
between the cell body and distal
axon is important to maintain
viability of the axon and to supply
trophic factors to target organs.
■ Diseases of both the spinal
cord and cerebellum cause ataxia,
and clinical differentiation can be
difficult.
■ Appropriate myelinization
requires normal myelin
biochemistry as well as
appropriate interaction between
axons and myelin-producing
cells.
Signalment: An
Aid for Recognition
of Uncommon
and Diagnostically
Challenging Neurologic
Diseases in Dogs
Veterinary Specialists of Rochester University of Pennsylvania
Rochester, New York Betsy Dayrell-Hart, VMD
John Speciale, DVM
M
any spinal cord diseases of dogs are prevalent only in specific
breeds.1 Some diseases (e.g., dermoid sinus in Rhodesian ridgebacks
and spina bifida in English bulldogs) can be diagnosed appropriately
by physical examination, radiography, or both, but many diseases are not easily
diagnosed before death. Because routine ancillary tests are often unrewarding
and clinical signs are not pathognomonic, familiarity with predisposing signal-
ment can be helpful (Table I).
This article presents a classification scheme for breed-specific spinal cord dis-
eases that cause neurologic signs in dogs. Specific diseases are categorized
according to suspected pathophysiology and include abnormal neuron
metabolism, disorders of axoplasmic transport, myelin disorders, astrocyte pro-
liferation, abnormalities of gross structure of the spinal cord, and disorders of
unknown pathology.
ABNORMAL NEURON METABOLISM
Three types of breed-specific metabolic disease are considered here: inborn
errors of metabolism, abiotrophy, and neuroendocrine imbalance. Inborn er-
rors of metabolism result from enzyme deficiencies that prevent adequate pro-
 TABLE I
Classification of Breed-Specific Spinal Cord Disorders
Small Animal

Breed Affected
Type of Disorder Subgroup Disease (Age of Onset) Inheritance Structure Clinical Signs

Metabolic Abiotrophy Cerebellar Kerry blue terriers (< 6 mo) AR Cerebellum Ataxia (primarily pelvic),
diseases abiotrophy Gordon setters (6–30 mo) AR tremor (head),
Bullmastiffs (1–7 mo) AR hypermetria
Border collies (1–2 mo) Unknown
Spinal Brittany spaniels (1–2 or 2–12 mo) D Spinal cord, Muscle atrophy,
muscle English pointers (5 mo) Unknown motor neurons diminished myotatic
atrophy Rottweilers (4–6 wk) Unknown reflexes, paralysis,
Swedish Laplands (5–7) AR cerebellar
German shepherds (1–2 mo) Unknown
Mixed breeds, collies

Inborn Globoid Cairn terriers (3–6 mo) AR White matter of Cerebellar, paresis
errors of leukodystrophy West Highland white terriers (3–6 mo) AR peripheral and
metabolism Miniature poodles (3–6 mo) Unknown central nervous
Beagles (3–6 mo) Unknown system
Basset hounds (3–6 mo) Unknown
Pomeranians (mature) Unknown

Neuroendocrine Scotty cramp Scottish terriers (usually < 6 mo) AR No morphologic Muscle spasm
imbalance (hyperkinesis) lesion

Diseases of Axoplasm Boxer Boxers (< 6 mo) Unknown Axons in cervical Ataxia
axons transport neuropathy spinal roots and
ventral columns
Sensory Long-haired dachshunds (birth) Unknown Distal axons Ataxia, incontinence,
neuropathy diminished reflexes

Giant axonal Alsatians (16 mo) AR Motor neurons Ataxia, posterior paralysis,
neuropathy diminished myotatic
reflexes
Neuroaxonal Rottweilers (adult) AR Distal axons in Hypermetria (fore),
dystrophy central nervous diminished reflexes, tremor
system
Axon Hereditary ataxia Smooth-haired fox terriers (2–6 mo) AR White matter of Ataxia, posterior paralysis
degeneration Jack Russell terriers (2–6 mo) AR cervical and
thoracic spinal
cord
The Compendium October 1996
 TABLE I (continued)
Breed Affected
Type of Disorder Subgroup Disease (Age of Onset) Inheritance Structure Clinical Signs

Hound ataxia Beagles (2–7 yr) E? Spinal cord tracts, Posterior paralysis
Foxhounds (2–7 yr) E? especially
Harriers (2–7 yr) E? midthoracic
Degenerative German shepherds (5–11 yr) Unknown Funiculi of Posterior paralysis
myelopathy Siberian huskies (10–12 yr) Unknown midthoracic
The Compendium October 1996

spinal cord
Myelin Demyelinization Dalmatian Dalmatians (3–6 mo) AR Degeneration of Blindness, posterior
disorders leukodystrophy myelin in optic paralysis
nerve and
cerebrum
Demyelination Miniature poodles (2–12 mo) Unknown Spinal cord Posterior paralysis
white matter
Afghan hound Afghan hounds (6–9 mo) AR Spinal cord Cerebellar, posterior
myelopathy white matter paralysis
(especially
midthoracic)
Leukoencephalo- Rottweilers (2–3 yr) Unknown Dorsal and lateral Cerebellar, posterior
myelopathy funiculi of spinal paralysis
cord and
cerebellar white
matter
Abnormal Defective Chow chows (< 1 mo) Unknown Central nervous Tremor
myelinization myelinization Springer spaniels (2–3 days) X-linked system white
Dalmatians (2–3 days) Unknown matter
Samoyeds Unknown
Astrocyte Fibrinoid Labrador retrievers (6–9 mo) Unknown Central nervous Posterior paralysis
proliferation leukodystrophy system white
matter
Abnormalities Spinal Weimaraners (6 mo) CD Spinal cord Bunny-hopping gait,
of gross dysraphism Dalmatians (3 mo) medial structures proprioceptive deficits
structure Siberian huskies (3 mo)
Mixed breeds (18 mo)
Disorders of Dancing Doberman pinschers (mature) Unknown Prolonged limb flexion
pathophysiology doberman
disease
Small Animal
Small Animal The Compendium October 1996

duction or elimination cerebellar dysfunction are most frequently observed in

of biochemical prod- patients with globoid cell leukodystrophy; however,
Muscle spasm, paralysis
with extensor rigidity
ucts. In several metabolic paresis or ataxia related only to involvement of the
diseases of dogs, clinical spinal cord may be seen early in the disease process.4 In
Clinical Signs

Pain, posterior signs relate to dysfunc- breeds that are affected most often (e.g., cairn and West
paralysis tion of the spinal cord; Highland white terriers,5–14 miniature poodles,15 coon-
in most cases, signs of hounds, 16 and beagles 17), signs manifest by 3 to 6
extraspinal disease pre- months of age. In other breeds (e.g., basset hounds18
dominate. and Pomeranians19), signs are not obvious until maturi-
The underlying caus- ty. Regardless of the breed or age of onset, the disease
es of abiotrophy are progresses relentlessly in all cases.
poorly understood. Clinical signs result from the inability to metabo-
Meningitis

lize β-galactocerebroside, which accumulates in the

Unknown

Abiotrophy may be a
Structure
Affected

consequence of prema- Schwann cells and oligodendrocytes of the white mat-

ture aging, which is ter of the central nervous system. The inability to me-
caused by metabolic or tabolize β-galactocerebroside results from decreased ac-
nutrient deficiencies, tivity of the enzyme β-galactocerebrosidase. Because it
or it may result from is a disease of white matter, globoid cell leukodystrophy
Inheritance

Unknown

Unknown

deficient enzyme ac- can also be classified as a demyelinating disease.

tivity. Evidence has in- On rare occasions, abnormal storage of β-galacto-
criminated abnormal cerebroside can be demonstrated by positive results
activity of N-acetyl- from periodic-acid Schiff after staining of the cyto-
ated-α-linked acidic plasm of cells recovered from cerebrospinal fluid. Mea-
dipeptidase3 and super- surement of decreased β-galactocerebrosidase activity
TABLE I (continued)

oxide dismutase as a in peripheral leukocytes of dogs with globoid cell

cause of abiotrophy.2 If leukodystrophy is a possible method of antemortem di-
Labrador retrievers (< 1 mo)

enzyme deficiency is agnosis. Because lesions occur in the peripheral nerves

ultimately recognized as well as in the central nervous system, biopsy of pe-
as the cause of the ripheral nerves can also be diagnostic.20–22
(Age of Onset)

Beagles (4–24 mo)

disorder, it may be re- Clinical descriptions of globoid cell leukodystrophy

Breed

classified as an inborn are similar to those of another congenital metabolic dis-

error of metabolism. order, Gaucher’s disease; however, this rare disease of
AR = autosomal recessive, CD = codominant, D = dominant, E = environmental.

Neuroendocrine im- silky terriers is seldom, if ever, reported to occur outside

balance does not nec- of Australia.23,24
essarily cause destruc-
tive or morphologic Abiotrophy
changes in nervous tis- Spinal Muscle Atrophy
Polyarteritis
myoclonus

sue; therefore, only ab- Similar to amyotrophic lateral sclerosis (Lou Gehrig’s
Familial
Disease

normal motor func- disease) in humans, the signs of spinal muscle atrophy
tion is observed. in Brittany spaniels 25–28 (inherited as an autosomal
dominant trait); English pointers,29,30 mixed breeds,31,32
Inborn Errors of rottweilers,33,34 collies,35 and German shepherds36 (un-
Metabolism: known mode of inheritance); and Swedish Lapland
Subgroup

Globoid Cell dogs37,38 (inherited as an autosomal recessive trait) result

Leukodystrophy from degeneration of motor neurons. In addition to the
Globoid cell leuko- various patterns of inheritance, the cytopathologic ap-
dystrophy, an infre- pearance also tends to differ. Therefore, the disease is
quently diagnosed probably caused by a metabolic disorder that varies
Type of Disorder

metabolic disease that from breed to breed. In all seven breeds, signs begin
causes spinal cord during puppyhood and consist of weakness that relent-
signs, is inherited as a lessly progresses to atrophy and paralysis. Distinct syn-
simple autosomal re- dromes of spinal muscle atrophy have occurred in Brit-
cessive trait. Signs of tany spaniels and, in one syndrome, signs do not

METABOLIC DISEASE ■ β-GALACTOCEREBROSIDASE ■ ABIOTROPHY

Small Animal
appear until adulthood. Of thoroughly studied breeds,
Brittany spaniels and rottweilers (but not English
pointers or Swedish Lapland dogs) have marked dilata-
tion of neurons. In well-studies breeds such as Brittany
spaniels, proximal axons are swollen with excessive neu-
rofilaments, an indication of defective axon transport.
Cerebellar Abiotrophy
Although the signs of abiotrophy of the cerebellum
predominantly result from the effects of brain disease,
this disorder is included in the discussion of spinal cord
disease because it creates spinal lesions in collies and be-
cause the clinical signs are similar to hereditary ataxia
(to be discussed later). Abiotrophy of the cerebellum,
which is inherited as an autosomal recessive trait, re-
sults in degeneration of the white matter of the ventral
spinal cord in rough-coated39 and Border40 collies but
does not cause spinal cord lesions in other affected
breeds, such as Kerry blue terriers,41 Gordon setters,42–47
and bullmastiffs.48 Ataxia, the principal sign, usually
manifests when affected dogs are younger than 6
months of age; in Gordon setters, however, signs are of-
ten inapparent until the dog is 1 to 2 years of age.
Neuroendocrine Imbalance: Scotty Cramp
An autosomal recessive gene in Scottish terriers49–54
and dalmatians55 is the cause of Scotty cramp (or hy-
perkinesis), a benign, episodic disorder characterized by
various clinical signs, including muscle spasms of the
limbs, back, and sometimes face. Signs are first ob-
served between 2 and 18 months of age. The disease re-
sults from abnormal serotonin metabolism in the spinal
cord. Exercise, excitement, and administration of am-
phetamines exacerbate the clinical signs. Signs are usu-
ally controlled by administration of promazine or diaz-
epam. No morphologic defects are associated with this
disorder.
AXOPLASMIC TRANSPORT DISORDERS
Uninterrupted flow of axoplasm between the cell
body and distal axon is important to maintain viability
of the axon and to supply trophic factors to target or-
gans. Both orthograde flow (from the cell body to the
distal axon) and retrograde flow (from the distal axon
to the cell body) must be maintained for normal func-
tion. Two distinct systems of orthograde flow (one des-
ignated as fast and the other as slow) and one of ret-
rograde flow are currently recognized. Malfunction
of either system is associated with a characteristic
cytopathology. For example, degeneration of distal
axons56,57 or accumulation of matter in proximal axons
results from malfunction of orthograde transport,
whereas accumulation of organelles or swelling in the
SCOTTY CRAMP ■ AXONOPATHY ■ SENSORY NEUROPATHY
The Compendium October 1996
distal axons indicates failure of retrograde transport.
Axoplasmic transport is compromised by exposure to
neurotoxins; however, in the disease considered here,
the cause of defective axoplasmic transport is unknown.
Such defects may even result from metabolic or nutri-
tional disorders, in which case they could also be con-
sidered abiotrophies.
Transection of an axon causes complete failure of ax-
oplasmic transport. The distal axon is deprived of all
nourishment, which results in wallerian degeneration
(distal axonal degeneration and nuclear chromatolysis).
The separated axon degenerates and may even swell,
but, in contrast with impaired retrograde transport,
organelles do not accumulate.
Accumulation of membranes, neurofilaments, and
neurotubules and the resultant swollen axons caused by
malfunction of axoplasmic transport presumably occur
in the following five spinal cord diseases: axonopathy in
boxers, sensory neuropathy in long-haired dachshunds,
giant axonopathy, neuroaxonal dystrophy, and spinal
muscle atrophy of Brittany spaniels (as discussed previ-
ously). Axon degeneration is the hallmark of hereditary
ataxia, hound ataxia, and degenerative myelopathy.
Axon Swelling
Axonopathy in Boxers
A type of axonopathy that is seen in young boxers is
presumably inherited as an autosomal recessive trait.
This disorder causes ataxia but not muscle atrophy.
Recovery from this progressive disease has not been
reported. Lesions are located primarily in the ventral
and dorsolateral columns of the spinal cord (including
the spinal roots), but the dorsal columns are spared.
Because affected axons are swollen by accumulated
membranes and organelles, it is likely that this disorder
results from defective axoplasmic transport. Abnor-
malities of myelin may, however, also play a role in the
disease process.58
Sensory Neuropathy in
Long-Haired Dachshunds
The signs of sensory neuropathy in long-haired
dachshunds include ataxia, urinary incontinence, and
decreased nociceptive responses.59 Signs become appar-
ent within the first few months of life. Although flexor
reflexes are usually absent, patellar reflexes are often nor-
mal. This combination of signs seems to indicate that
the disease process specifically and most severely affects
nociceptive axons.60 Electrodiagnostic studies have
shown decreased velocity of sensory conduction but not
of motor nerve conduction. Distal axons become dilated
with organelles; such dilatation presumably results from
disrupted flow of retrograde axoplasm.
The Compendium October 1996
Giant Axonopathy
Giant axonopathy in German shepherds first affects
dogs at 14 to 16 months of age. This disorder is proba-
bly inherited as an autosomal recessive trait. Hindlimb
paresis and, eventually, distal muscle atrophy are noted
along with fecal incontinence and regurgitation (which
results from esophageal hypomotility). This last com-
plication often results in aspiration pneumonia, which
worsens an already poor prognosis.61–63 Distal portions
of affected axons from the spinal cord and peripheral
nervous system become swollen with accumulated neu-
rofilaments; such accumulation presumably results
from defects of slow axon transport.64–66
Neuroaxonal Dystrophy
Neuroaxonal dystrophy in rottweilers is most likely
inherited as an autosomal recessive trait. Signs include
ataxia; head tremor; forelimb hypermetria; and, less fre-
quently, nystagmus. These signs appear in the first year
of life but are marked by such a slow progression that
some dogs do not become obviously affected until
adulthood. Signs of neuroaxonal dystrophy are consis-
tent with lesions in the cerebellar and vestibular nuclei;
however, the most severe lesions are in the dorsal horn
of the spinal cord and the cuneate and gracile nuclei.
Therefore, early signs often resemble cervical spinal
cord disease. Lesions are most severe in distal axons,
which become swollen with accumulated membranes.
Such accumulation provides evidence of compromised
retrograde transport.67–69
Axon Degeneration
Hereditary Ataxia
Jack Russell terriers and smooth-haired fox terriers
with hereditary ataxia (inherited as an autosomal reces-
sive trait) begin to show signs of disease at 10 to 16
weeks of age. Hereditary ataxia is remarkably similar to
the ataxia associated with abiotrophy of the cerebellum;
however, the lesions of hereditary ataxia occur predomi-
nantly in the dorsolateral and ventromedial spinal cord.
Of note is that the spinal lesions progress symmetrical-
ly. Ataxia progresses slowly, and the signs, which first
become obvious in the hindlimbs, progress to the fore-
limbs but may stabilize to the extent that some animals
experience minimal loss of function.70–73
Hound Ataxia
Beagles, harriers, and foxhounds are affected with
hound ataxia beginning at 2 to 7 years of age, and the
signs progress for as long as 18 months. The midtho-
racic spinal cord is most severely affected, and only the
dorsal funiculi are spared from severe axon degenera-
tion.74 Ultrastructural studies have presented solid evi-
NEUROAXONAL DYSTROPHY ■ ATAXIA ■ DEGENERATIVE MYELOPATHY
Small Animal
dence that the lesions of hound ataxia may result from
primary demyelinization and not from wallerian degen-
eration.75
Forelimb function remains normal, but the hind-
limbs become ataxic; the cutaneous trunci reflex is of-
ten lost. Because different breeds are affected in the
same manner, hound ataxia is probably an environmen-
tal and not an inherited disease. Substantial evidence
links hound ataxia with ingestion of ruminant stom-
achs, and preliminary evidence implicates deficiency or
abnormal metabolism (or both) of methionine as the
pathophysiologic mechanism.
Degenerative Myelopathy
Degenerative myelopathy is a progressive disorder
that is seen primarily in mature (5 to 11 years) German
shepherds76–78 and less frequently in Siberian huskies,79
Irish setters, and miniature poodles.80 The lesions are
most severe in the thoracic spinal cord and include
myelin and axon degeneration. Because degeneration of
axons and myelin occurs to a similar extent, it is un-
known in which structure the disease originates. Patho-
logic descriptions of degenerative myelopathy have in-
cluded proliferation of astrocytes; therefore, it is even
possible that astrocytes are responsible for destruction
of the myelin and axons. Classification of degenerative
myelopathy as a type of primary axonopathy is specula-
tive and may change as further investigation is done.
Evidence suggests that the primary cause of degen-
erative myelopathy is immunologic or nutritional in
origin, but neither cause has yet been clearly estab-
lished.81–83
Although the prognosis for survival of patients with
degenerative myelopathy is poor, there is evidence that
treatment with exercise, vitamin supplements, and ∈-
aminocaproic acid are beneficial. A detailed description
of such treatment can be found in the literature.84
Because the clinical signs of degenerative myelopathy
resemble many disorders of large-breed dogs (e.g., type
II disk disease and neoplasia), it is necessary to elimi-
nate other diseases from consideration. Published case
series have reported degenerative myelopathy to be
more consistent in mature than in geriatric dogs. Diag-
nosis of degenerative myelopathy should thus be made
with special caution when signs appear in very old dogs
(older than 12 years of age).
MYELIN DISORDERS
Myelin, the complex lipid substance that surrounds
large axons and allows for rapid transmission of neuro-
logic impulses, is the product of the Schwann cells in
the peripheral nervous system or oligodendrogliocytes
in the central nervous system. Appropriate myeliniza-
Small Animal
tion requires normal myelin biochemistry as well as
appropriate interaction between axons and myelin-
producing cells.85 Because disease processes that cause
demyelinization can result in axonopathy, and ax-
onopathy can also be associated with secondary de-
myelinization, it is often difficult to identify the prima-
ry pathophysiologic event.
Leukoencephalomyelopathy
Leukoencephalomyelopathy is a chronic degenerative
disorder of adult rottweilers that is inherited as an auto-
somal recessive trait. Clinical signs include ataxia, hy-
permetria, and, eventually, tetraparesis. Lesions, which
are bilaterally symmetric and most severe in the dorsal
and lateral funiculi (especially in the cervical area), are
limited to myelin. There is little morphologic damage
to axons. Because reactive astrocytes are present in the
lesions of leukoencephalomyelopathy, it is not clear
whether the origin of the disease is genetic, environ-
mental, or immunologic.86
Leukodystrophy in Dalmatians
Dalmatian leukodystrophy, a progressive disorder of
white matter of the central nervous system, begins to
manifest in 3- to 6-month-old dalmatians; the disorder
is characterized by ataxia and blindness. Affected dogs
are not reported to recover.87 Although the cause is
unknown, it is suspected that the disorder is inherited
as an autosomal recessive trait. Because lipid-laden
macrophages are a histologic feature of dalmatian
leukodystrophy, some investigators have classified this
as a lipid storage disease.88 Both myelin and axons are
damaged, but demyelinization seems to occur early in
the course of disease.
Demyelinating Myelopathy
Demyelinating myelopathy is a suspected hereditary
disease of young miniature poodles that causes spastic
paraplegia and tetraplegia. Axons are, for the most part,
spared. Lesions in the brainstem and cerebellum have also
been reported.89,90 The occurrence of demyelinating
myelopathy has not been reported for several decades.91
Myelopathy in Afghan Hounds
Young Afghan hounds (3 to 13 months of age) can
be affected with paralysis that progresses during a peri-
od of 10 to 40 days. In some cases, death results from
respiratory paralysis. This disease, which is inherited
as an autosomal recessive trait, most severely affects
the dorsal and ventral funiculi of the thoracic spinal
cord.92–94 Complete necrosis of the spinal cord has been
reported, but axons seemed to be spared early in the
course of disease. The initial pathologic event is de-
LEUKODYSTROPHY ■ MYELOPATHY ■ ASTROCYTE PROLIFERATION
The Compendium October 1996
myelinization. The cause of myelopathy in Afghan
hounds is unknown, but it seems that enzymatic95 and
not vascular abnormalities (as initially reported) are the
underlying cause of demyelinization. 92 Because of
scrupulous culling by breeders of Afghan hounds, this
disease is now very rare.
Defective Myelinization
Predisposition to defective myelinization can be
found in several breeds of dogs, including springer
spaniels (inherited as sex-linked recessive trait), chow
chows (inherited as an autosomal recessive trait),
Samoyeds, weimaraners, Bernese mountain dogs,96 and
dalmatians. This disorder is unlikely to be mistaken for
exclusive disease of the spinal cord because the most
prominent sign of defective myelinization is tremor in
very young dogs, and, less noticeably, hypermetria and
gait abnormalities. Histopathology often demonstrates
a marked lack of spinal cord myelin. Although neuro-
logic function is severely compromised early in life,
myelinization commonly progresses and signs often re-
lent after the first year of life. Springer spaniels, howev-
er (the most severely affected breed), cannot eat with-
out assistance and are frequently euthanatized before
recovery. Because there is no evidence of astrocyte pro-
liferation or of myelin destruction, defective myeliniza-
tion is considered to be distinct from demyelinization.85,96
ASTROCYTE PROLIFERATION:
FIBRINOID LEUKODYSTROPHY
Laboratory techniques to study the biological role of
astrocytes have only recently been developed, but it is
now known that astrocytes are important for normal
metabolic and immunologic function of the central ner-
vous system. Fibrinoid leukodystrophy is associated with
astrocyte proliferation, and although the cause of the dis-
order is unknown, it is likely that abnormal metabolism
of astrocytes plays a role in the pathophysiology.97
Fibrinoid leukodystrophy, which is similar in pathol-
ogy to Alexander’s disease in humans, is a rare disease
in dogs. This disorder has been reported in Labrador
retrievers and in a Scottish terrier. Signs of hindlimb
paresis; progressive ataxia; and, eventually, behavioral
changes are first noticed at approximately 6 months of
age. Prognosis for survival is poor.98–100
The diagnostic histopathologic finding in cases of
fibrinoid leukodystrophy in humans and dogs is the
observation of Rosenthal fibers. These fibers are large,
eosinophilic refractile bodies that are most prevalent
around blood vessels; these bodies are presumably rem-
nants of degenerated astrocytes.101 Astrocyte prolifera-
tion is a frequent but nonspecific feature of fibrinoid
leukodystrophy.
The Compendium October 1996 Small Animal

ABNORMALITIES OF GROSS
STRUCTURE: SPINAL DYSRAPHISM
Spinal dysraphism (also known as myelodysplasia
and spinal dysplasia) is a genetic disorder that is best
documented in weimaraners102–105 but has also been re-
ported in dalmatians,106 Siberian huskies, and mixed-
breed dogs.107 Inheritance in weimaraners is codom-
inant and of variable penetrance. Affected dogs are
heterozygotes because the homozygous genotype is fa-
tal.108 Clinical signs, which are varied, become apparent
when the dog is younger than 2 months of age; the
most frequent signs include a bunny-hopping gait and
proprioceptive deficits.
Spinal dysraphism causes skeletal and epidermal ab-
normalities in some dogs, although this finding is not
consistent. The spinal cord abnormality always involves
the central area of the cord where it cannot be detected
before death. Malformation of the spinal cord varies
and may involve dilatation, absence, or malformation
of the central canal. Clinical signs of disease, however,
probably result directly from arrested differentiation
and malformation of medial white matter.
DISORDERS OF UNKNOWN PATHOPHYSIOLOGY
Some diseases that affect the spinal cord have no de-
fined pathophysiologic mechanism. Such disorders
include dancing Doberman disease, polyarteritis, and
familial myoclonus.
roiditis and splenic amyloidosis. Affected dogs often re-
spond well to antiinflammatory drugs, although recur-
rence is reported.110
Familial Myoclonus
Three-week-old Labrador retriever puppies have been
reported to develop muscle spasms that progress to
paralysis with extensor rigidity. This disorder is unlikely
to be mistaken for focal spinal cord disease; the clinical
signs tend to resemble strychnine poisoning or tetanus.
No histopathologic lesions have been detected, and a
seizure disorder has not yet been entirely eliminated as
a cause of the clinical signs.111
CONCLUSION
With a thorough knowledge and understanding of
spinal cord disorders that affect various breeds, clin-
icians can narrow the list of diagnostic differentials
when faced with a dog experiencing neurologic deficits.
Although spinal cord disorders are difficult to diagnose,
the information presented here should increase aware-
ness of this important group of diseases and enable
clinicians to add such diseases to the differential list.

Dancing Doberman Disease

Interested in
Mature Doberman pinschers can be affected by a
slowly progressing neurodegenerative disease that report-
submitting an
edly affects peripheral nerves and results in muscle atro-
phy. Clinical signs consist of prolonged flexion of
exotics article
hindlimbs. This posture is similar to that seen in animals
with painful musculoskeletal diseases, but dogs with
for Compendium?
dancing Doberman disease are apparently not in pain.109
Please contact Dr. Branson Ritchie
Polyarteritis for details (phone 706-542-6316;
Polyarteritis has been documented in young beagles
(younger than 2 years of age).110 Polyarteritis is an acute email britchie@vet.uga.edu;
progressive syndrome that may be the result of an au- fax 706-542-6460).
toimmune disorder; the disease is associated with in-
flammation of the meninges and of the spinal cord. Af-
fected dogs have a stiff gait, are reluctant to move, and
seem to guard their necks. Although the clinical signs
of polyarteritis resemble those of cervical disk disease
(also a common disorder of beagles), dogs with poly-
teritis are usually febrile and spinal fluid typically con-
tains numerous neutrophils (more than 1000 cells/
mm3). Signs referable to other organ systems are inap-
parent, but the disease may also affect coronary vessels
and, in some dogs, is associated with lymphocytic thy-

SPINAL DYSRAPHISM ■ POLYARTERITIS ■ FAMILIAL MYOCLONUS

Small Animal
About the Authors
Dr. Speciale, who is a Diplomate of the American Board
of Veterinary Practitioners and the American College of
Veterinary Internal Medicine, is affiliated with the Veteri-
nary Specialists of Rochester, Rochester, New York. Dr.
Dayrell-Hart, who is a Diplomate of the American College
of Veterinary Medicine, is affiliated with the Department of
Clinical Studies, School of Veterinary Medicine, University
of Pennsylvania, Philadelphia, Pennsylvania.
REFERENCES
1. Moore MP: Approach to the patient with spinal disease.
Vet Clin North Am Small Anim Pract 22:751–780, 1992.
2. Blakely RD, Robinson MB, Thompson RC, et al: Hydroly-
sis of the brain dipeptide N-acetyl-L-aspartyl-L-glutamate:
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