Veterinary Dermatology 2005, 16, 137146

Review

Blackwell Publishing, Ltd.

Cutaneous manifestations of neurological diseases: review of

neuro-pathophysiology and diseases causing pruritus
KIRSTEN GNIRS* and PASCAL PRLAUD
*3 rue du Dauphin, F-94100 Saint Maur des Fosss, France
17 rue Fernet, F-94700 Maisons-Alfort, France
(Received 16 June 2004; accepted 10 January 2004)

Abstract Pruritus does not always originate from stimulation to the skin associated with primary dermatological disorders. It may be caused by neurological or behavioural disorders. The essential role of the nervous system
in the control (enhancement and inhibition) of pruritus and its pathophysiology are presented. In order to allow
differentiation between dermatological and neurological disorders, inherited or acquired peripheral neuropathies
and central nervous disorders (ArnoldChiari syndrome, seizure-related disorders, central nervous system
tumours) that may induce itch are discussed.

IN TRO D U CT ION
The majority of pruritic skin diseases in dogs and cats
are caused by ectoparasitic infestations, bacterial and
fungal infection or hypersensitivities. However, pruritus
does not always originate from external stimulation to
the skin, but can be caused by neurological or behavioural disorders. The essential role of the peripheral
and central nervous system in the control (enhancement and inhibition) of pruritus has been described.1,2
The goal of this paper is to review the basic pathophysiology of pruritus, predominantly that of neurological
origin. It is hoped that this information will be useful
in the clinical setting, allowing differentiation of
patients with skin disease due to neurological causes
from those with primary dermatological disorders.

Pathophysiology of pruritus
Pruritus or itching is defined clinically as an unpleasant
sensation that causes an animal to rub, lick, chew, or
scratch at its skin.3 It is a physiological self-protective
mechanism and, similar to other cutaneous sensations
such as pain, touch, perception of vibration, cold or
heat, it helps to defend the skin against harm from the
environment. Additionally, pruritus is a major sign of
skin and some systemic diseases. Pruritus can be localized (acral lick dermatitis), regional (pedal pruritus) or
generalized. Pruritus can be evoked in the skin directly
by mechanical and thermal stimuli or indirectly through
chemical mediators. It may also occur as a result of
peripheral nerve disease or be generated from the
spinal cord or brain, independent of peripheral stimulation. Pruritus is not a disease, but a sign of an underlying dermatological condition or neuro-psychological
Correspondence: Kirsten Gnirs, 3 rue du Dauphin, F-94100 Saint
Maur des Fosss, France, E-mail: kgnirs@hotmail.com
2005 European Society of Veterinary Dermatology

disorder. Establishing what initiates the pruritus in an

individual case requires evaluation of multiple internal
and external factors4 that vary from condition to condition and animal to animal.5

Pruritus vs. pain

Pruritus and pain are both defined as signs resulting
from an unpleasant sensation. Pain and itch are transmitted predominantly through slow-conducting, unmyelinated C-fibres.6 One theory for pruritus was that it is
a subliminal version of pain (intensity theory). Accordingly, weak noxious stimuli would cause minor activation of nociceptors, which would be felt as itch,
whereas stronger noxious stimuli would activate nociceptors more intensely and provoke pain.6 However,
recent studies have clearly shown that there is a distinct
neuronal pathway for itch, functionally separate from
the pain pathway. Primary afferent nerve fibres that
carry histamine-induced itch have been found in
humans7 and, more recently, spinal neurones have been
identified that respond specifically to histamine.8 The
combination of dedicated peripheral and central neurones, with a unique response pattern to pruritogenic
mediators and anatomically distinct projections to the
thalamus, provides the basis for a specific neuronal
pathway for itch. Unfortunately, it has not yet been
possible to morphologically differentiate neurones that
mediate itch from those that mediate pain.
However, there are complex interactions between pain
and itch. There is not a complete distinction between
pruritic and pain-producing (algogenic) substances
in the activation of pruriceptors and nociceptors. The
inhibition of itch by pain is well known and can explain
the antipruritic effect of scratching. The opposite effect
also exists and has clinical implications: inhibition of
pain processing (e.g. by spinal opioids) can generate
itch.3 Conversely, blockade of spinal opioid receptors
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K Gnirs and P Prlaud

can be used as an antipruritic therapy. Moreover, the

spinal processing of pain and itch can be modulated,
resulting in hypersensitivity or hyposensitivity to
pain or itch. In chronic painful or pruritic conditions,
ongoing activity of peripheral receptors can induce a
spinal hypersensitivity to pain or itch in patients. At
present, the specific stimuli that would sensitize only
neurones mediating pruritus, but not affect other Cfibres, have not been identified. Peripheral mechanisms
alone might not be sufficient to explain why some
inflammatory disorders cause pain, whereas others are
characterized by intense pruritus. Central sensitization
contributes to chronic pruritus or pain.3 Assessment of
pain or pruritus in an animal is difficult because both
entities are perceptions. Additionally, the relative
reaction to pruritus or pain varies considerably within
the same species.9 Evaluation of the pathophysiology
of pruritus in veterinary species requires concurrent
review of the pathophysiology of pain.

Skin innervation and neuronal processing

of pruritus
All layers of the skin have a dense network of highly
specialized afferent sensory and efferent autonomic
nerves. Afferent sensory nerve fibres transmit noxious
information and cold via thinly myelinated A-fibres
(conduction velocity 1030 m s1). Pain, heat and itch
are transmitted through slow-conducting unmyelinated
C-fibres (conduction velocity 0.52 m s1). Recent
studies using histamine stimulation in humans and cats
suggest a neuronal system dedicated to itch. Single fibre
recordings in humans have shown that the sensation of
histamine-induced itch is transmitted by a very slowconducting (0.5 m s1) subpopulation of C-neurones
(pruriceptors) that also respond to other pruritic
substances such as prostaglandin E2.10 However, it
is doubtful that this particular type of pruriceptor is
responsible for all types of the itch sensation.
Sensory afferent fibres from the skin of limbs, neck
and trunk enter the spinal cord through the dorsal root
and synapse on relay neurones in the dorsal horn of the
spinal cord (Fig. 1). At the spinal cord level, spinothalamic projection neurones are selectively excited by
histamine and thus probably participate in the transmission of pruriceptive information in a dedicated
neuronal pathway.8 In addition, positron-emission
tomography studies in humans have shown possible

Figure 1. Sensory afferent fibres from the skin of limbs, neck and
trunk enter the spinal cord through the dorsal root and synapse on
relay neurones in the dorsal horn of the spinal cord.

Figure 2. Domestic animals have multisynaptic, bilateral

nociceptive pathways, in the propriospinal system. These thin fibres,
located deep in the spinal cord are resistant to injury. The spinal
tracts relay in the thalamus and then travel to the sensory cerebral
cortex.

activation of the primary sensory cortex after cutaneous histamine application.11 Motor-associated areas
that correlate with the desire to scratch were activated.
In contrast to pain, itch does not provoke a spinal
reflex. The scratch movement is governed from an area
in the distal medulla near the bottom of the fourth ventricle which is, in turn, controlled by the midbrain.12 The
nociceptive spinal cord pathways include other tracts
like the spinoreticular, spinomesencephalic and spinocervical tracts. The relative significance and anatomical
location of these tracts within the spinal cord differ
between species. Domestic animals have multisynaptic,
bilateral nociceptive pathways in the propriospinal system composed of thin fibres located deep in the spinal
cord, thus are resistant to destruction and injury. The
spinal tracts relay in the thalamus and then travel to
the sensory cerebral cortex (Fig. 2). Nociception of the
head is carried in branches of trigeminal (V), facial
(VII), glossopharyngeal (IX), and vagus (X) nerves.
Fibres synapse in the caudal nucleus of the trigeminal
nerve, then follow routes to the cerebrum, similar to
those of the spinal pathway. Once a pruritic neural
impulse is in the dorsal horn of the spinal cord or in the
sensory cortex of the brain, the signal can be modified
by emotional, biochemical and other central factors.

Peripheral and central sensitization with

chronic pruritus
It is well established that in painful inflammatory
conditions, peripheral nociceptors increase their sensitivity (peripheral sensitization) and further augment
the perception of pain. Simultaneously, the spinal
processing of noxious signals can be facilitated (central
sensitization) and further contribute to the sensation
of pain.13 Noxious stimuli can be perceived as itch in
patients with chronic pruritus (e.g. atopic dermatitis).14
Scratching itself may intensify pruritus by enhancing
local inflammation (production of endogenous pruritogenic substances such as prostaglandins, leukotrienes,15
prostaglandins16 and histamine4 by inflammatory cells
and release into the dermis) and inducing a higher Cfibre responsiveness.10 When neuronal depolarization
occurs, neuropeptides such as substance P may be
released from nerve fibres and produce itch sensation.4
There is also evidence that central sensitization occurs

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with chronic pruritus. Electrical, mechanical, chemical
and heat stimuli may evoke pruritus rather than pain in
the chronically affected pruritic skin areas.14,15 Ikoma
et al. have suggested that the marked increase in pruritus with chronicity may be due to sensitization in the
spinal cord rather than at the primary afferent neurones.14,17 This sensitization does not appear to be
simply based on skin inflammation.17 Chronic pruriceptive input may elicit central sensitization for itch
and nociceptive input no longer inhibits itch but on the
contrary is perceived as itch.14

Cutaneous manifestations of peripheral nervous

system diseases
There are numerous potential neurological causes of
pruritus or self-mutilation in dogs and cats. Most occur
because of stimulation of the peripheral nervous system. Central nervous system involvement is uncommon.
Damage to the peripheral nervous system by trauma,
nerve compression or neoplasia can result in localized
sensory neuropathy and secondary skin lesions.

SEN SO RY NE UROPAT HY
Inherited sensory neuropathies
English pointers. A sensory neuropathy, inherited as an
autosomal recessive trait in English pointers in the
United States and short-haired pointers in Europe has
been documented.18,19 Various descriptive terms have
been used in the literature: toe necrosis, hereditary
neurotrophic osteopathy and ulceromutilating acropathy.20
Affected dogs typically show signs of marked paw licking
and chewing at 3 to 5 months of age. However, diminution of pain perception can be detected in younger
puppies by detailed physical examination. As affected
puppies age, further multilation of the limbs occurs
with swelling, erythema, ulceration and laceration of
the distal limbs and paws. With time, painless fractures
and autoamputation may occur. Neurological examination reveals analgesia of the digital area of the hind
paws and hypoalgesia of the forepaws. The loss of pain
sensation may extend proximally up the limbs and onto
the trunk. There is no ataxia or proprioception deficits.
Stretch reflexes and muscle tone are normal. Electrodiagnostic evaluation shows normal motor nerve conduction velocity and there is no spontaneous activity.
Histopathological evaluation reveals that this disorder
is a slowly progressive, postnatal degeneration of sensory neurones of primarily, but not exclusively, the
nociceptive neurones.20 Paradoxically, however, there is
an absolute increase in the smaller neurones, and it was
speculated that this might be due to retarded differentiation or growth of sensory neurones.21 Cummings
et al. subsequently reported a reduction in substance P,
the excitatory neuropeptide proposed to mediate nociception, at the first synapse of sensory neurones in the
central nervous system (CNS), in the spinal cord of
affected pointers.18

139

There is no treatment for this neuropathy, and

despite bandaging the feet and muzzling the dog to prevent self-mutilation, infection of mutilated areas and
osteomyelitis develop. The prognosis is poor. Euthanasia should be recommended for severely affected dogs.
Long-haired dachshunds. In the UK, a sensory neuropathy, postulated to be inherited as an autosomal
recessive trait, has been reported in long-haired dachshunds.22,23 Subtle ataxia can be noted in affected dogs
as early as 812 weeks of age. Additional signs are selfmutilation of the penis, intermittent dribbling of urine
and vomiting. Neurological evaluation reveals hindlimb ataxia, although this may not be noticed by the
owner. There is also loss of proprioception and placing
reactions, and reduction or loss of pain sensation
(nociception) over the whole body in response to
superficial and deep pain stimulation. There is neither
paresis nor muscle atrophy. Cranial nerve function is
normal, with the exception of diminution or loss of
facial sensation.
Electromyographic and motor nerve conduction
velocities are normal. Sensory nerve potentials may be
reduced or absent. Diagnosis can be made from histopathological evaluation of peripheral sensory nerve
biopsy, which shows degenerative changes in both
larger calibre myelinated and unmyelinated fibres.
In the CNS, lesions suggested a distal axonopathy.
Affected dogs that do not develop significant problems
apart from chronic vomiting and/or self-mutilation
apparently live fairly normal lives. It may be necessary
to muzzle dogs when not under direct supervision.
There is no treatment. Affected dogs and all firstdegree relatives should not be bred.

AC QU IR E D S E N S O RY
N E U RO PAT H IE S
Canine ganglioradiculitis
This nonsuppurative inflammatory disease of the cranial and spinal ganglia and nerve roots affects adult
dogs of both sexes and various breeds.24 Affected dogs
acutely or subacutely develop sensory deficits that
progress despite medical therapy. Siberan huskies seem
to be over-represented among affected dogs.21 Clinical
signs are variable and include mutilation /hyperaesthesia
associated with hindlimb ataxia, difficulty in eating,
regurgitation or dysphagia. On neurological examination, there is no hindlimb paresis, but rather hypermetria
and base-wide stance. Proprioceptive positioning is
impaired and tendon reflexes depressed or absent.
Other findings include masticatory muscle atrophy,
megaoesophagus, dysphonia, unilateral Horners syndrome, facial hypalgesia, facial paraesthesia and head
tilt. Pain perception is decreased or disappears in some
dogs. The generalized dysesthesia is proposed to be
the cause of the self-induced skin lesions.21 The cause
of this neuritis is unknown, although toxic, viral, immunemediated, or genetic factors have been proposed. In

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K Gnirs and P Prlaud

humans, similar ganglioradiculitis CNS inflammatory

changes have been documented in association with
carcinomas, typically of the lung. Demonstration in
human patients of a cross-reacting antibody between
tumour and neurone nucleoprotein suggests that this
paraneoplastic syndrome has an immunopathogenic
basis in some patients.2

Pseudorabies
Pseudorabies (also known as Aujeskys disease, mad
itch, infectious bulbar paralysis) is an infectious disease caused by an alpha-herpes virus, with potent
neurotropism. The primary reservoir of the virus is
domestic and wild pigs. In companion animals, viral
transmission can occur by ingestion or via a bite
wound of an infected pig. After inoculation, the
pseudorabies virus is transported via sensory nerves
to the spinal cord and the brain.25 Incubation lasts
36 days.25 Nerve irritation and inflammatory changes
cause unrelenting pruritus that is not relieved by
scratching. Skin lesions are secondary to self-mutilation
and the virus is not present in the skin. However,
the site of pruritus is dependent on the location of viral
inoculation.26 In dogs and cats, the first clinical signs
are changes in behaviour. Hypersalivation is often
noticed, especially in cats. The primary clinical feature
of this disease is maniacal pruritus, most commonly
of the face and head. Infected animals scratch and rub
their faces, and severe self-trauma and excoriation
rapidly develop (Fig. 3). Pruritus is frantic and may
end in convulsions. Neurological deficits progress
because of increasing inflammation of the lower brain
stem and cranial nerves. Deficits are often unilateral
leading to anisocoria (oculomotor nerve III), masticatory muscles paralysis (trigeminal nerve V), head
tilt (vestibulocochlear nerve VIII), inability to swallow (glossopharyngeal and vagus nerves IX and X).
In the cat, a hoarse voice and anisocoria is highly suggestive of pseudorabies.25 Once clinical signs appear,
most animals die within 48 h.
Diagnosis is based on history, clinical signs and confirmation of pseudorabies infection by virus isolation on
frozen brain tissue. The primary differential diagnosis
is rabies and based on viral isolation from brain tissue.

Peripheral nerve tumours

Tumours of the peripheral nerve are uncommon in
dogs and cats. They can occur at any level, from the
proximal origin of the nerve sheath to the distal ending.
Classification, according to location along the nerve,
has been suggested (Fig. 4).27 Tumours of the distal
aspect of the nerve will present as cutaneous or subcutaneous soft tissue masses difficult to differentiate, on
routine morphological examination, from fibrosarcoma
or haemangiopericytoma.28 Most peripheral nerve sheath
tumours (PNSTs) occur in the caudal cervical area,
originating peripherally and then extending proximally
to involve the spinal cord.2932 Tumours of the brachial
plexus (C6-T2) typically cause thoracic limb hyperaesthesia and lameness (nerve root signature). Moisson-

nier proposed another classification of these tumours

according to the anatomical and clinical characteristics.27
In this scheme, type 1 tumours are characterized by
hyperaesthesia and pruritus of a limb but without
noticeable neurological deficit. The progressive compression of nerve fibres by the growing tumour irritates
the nerve, causing hyperaesthesia that follows the distribution of the dermatomes. The dog will lick the limb
on a focal area innervated by the affected sensory nerve
(Fig. 5). PNSTs occur more commonly in the caudal
cervical nerve roots, particularly C6 to C8,30 thus the
dorsal aspect of the paw and cranial region of the limb,
innervated by the radial nerve, will often be where
neurologically derived skin lesions will develop (Fig. 6).
Type 2 and 3 of Moissonniers classification show
neurological deficits with monoparesis (proprioceptive
deficits, hyporeflexia, muscle atrophy, decreased sensation) characteristic of a lower motor neurone lesion.
Type 4 tumours extend into the spinal cord and may
cause neurological deficits of the opposite limb and/or
the pelvic limbs.
The nomenclature of peripheral nerve tumours is
confusing and numerous designations have been used:
schwannoma, neuroma, neurinoma, neurilemmoma,
neurofibroma and neurofibrosarcoma. In dogs, many
PNS tumours are malignant. The cell origin schwannian, perineurial or fibroblastic is often impossible to
determine in routine histological sections, and therefore immunohistochemistry, electron microscopy or
tissue culture is needed. Without these, the preferred
designation of the neoplasm should be malignant
peripheral nerve sheath tumour.33
Diagnosis of PNST can be made very early on by
electrodiagnosis. When a dog is presented with localized pruritus, without obvious dermatological or
behavioural cause, electromyography should be considered, even if no neurological deficit is evident. If the
electromyography demonstrates spontaneous activity
(fibrillation, positive sharp waves) this is evidence of
denervation and localizes the lesion. Further tests
assessing nerve conduction velocities and amplitudes
of the muscle-evoked potential can add information
about the extention, type and severity of the tumour.
Imaging techniques (myelography, ultrasonography,
tomography and MRI) may be required to evaluate
proximal extension of the nerve and spinal cord
involvement.34,35
Prognosis depends on the tumour type and extention. Focal PNSTPs may be resected. However,
tumours are often locally invasive and entrap other
nerves. PNSTs invading the spinal canal have a poor
prognosis as there is a high rate of recurrence, despite
aggressive treatment. Radiation and chemotherapy
have not been found to be beneficial in managing these
tumours in dogs and cats.

Paraneoplasic peripheral neuropathy associated

with malignant neoplasms
Peripheral neuropathy in cancer patients is attributed
to nutritional deficiencies, metabolic disturbances or

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Figure 3. Alopecia and excoriation of the face secondary to

self-mutilation in a dog suffering from pseudorabies infection
(courtesy of Dr B. Hubert).

Figure 4. Classification according to the site of the peripheral nerve

tumour:Type 1: tumour located at the nerve ending; Type 2: tumour
located along a peripheral nerve; Type 3: tumour involving the
plexus, thus, emergence of several peripheral nerves; Type 4: tumour
extending into the vertebral canal and compressing the spinal cord.

treatment effects. Less commonly, peripheral neuropathy

is a paraneoplastic syndrome. The exact mechanism of
neuronal injury is not known, but the pathogenesis is
presumed to be immunological. In humans, there is a
distinct paraneoplatic syndrome, which may develop in
women with breast cancer. This syndrome is characterized by upper and lower extremity paraesthesias and

141

Figure 6. Acral lick dermatitis located on the dorsal paw secondary

to radial nerve tumour (dog; courtesy Dr E. Guagure).

Figure 7. Bilateral acral lick dermatitis of the dorsal area of distal

part of hind limbs, plantigrade posture, secondary to sciatic nerve
lesion in a 10-year-old German shepherd dog.

numbness, itching, muscle weakness and cramps,

and, in some, radicular symptoms and signs. Generally,
paraneoplastic neuropathy does not cause pruritus.2 In
animals, cases of paraneoplastic sensorimotor neuropathy in association with malignant tumours (mainly
insulinoma) have been described, but pruritus was not
reported.36

Figure 5. Cutaneous innervation of

thoracic and pelvic limbs of the dog.
musculocutaneous nerve
ulnar nerve
radial nerve.

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K Gnirs and P Prlaud

Syringohydromyelia associated with Arnold Chiari

syndrome in Cavalier King Charles spaniels

Figure 8. Bilateral self-induced ulcers of flanks secondary to

bilateral myoclonic movement of hind limbs, mimicking scratching
in a dog affected by canine distemper.

Cauda equina syndrome

Canine cauda equina syndrome results from damage to
sensory and/or motor spinal nerve roots emanating
from the L7, S1-S3, and Cd1-Cd5 spinal segments
which form the cauda equina. This general term does
not specify cause (e.g. degenerative lumbo-sacral
stenosis, diskospondylitis, trauma, neoplasia, vertebral
malformation, or idiopathic stenosis), but aetiological
diagnosis is vital for proper therapy and prognosis.
The most common cause of cauda equina syndrome
is degenerative lumbosacral stenosis and/or stenosis
of the intervertebral foramen. A combination of processes (disk protrusion, osteophyte formation, facet
subluxation and interarcuate ligament hypertrophy or
prolapse) may encroach on the cauda equina at the
level of the L7-S1 disk and/or foramen.37 German
shepherd dogs have an increased risk.38 The most consistent finding in patients with chronic lumbo-sacral
disease is pain in the lumbo-sacral area. Lumbo-sacral
hyperaesthesia, pruritus and/or self-inflicted lesions of
the hindlimb are frequent.39 Irritation of the sensory
component of the sciatic nerve, resulting from the progressive compression within the spinal canal or in the
foramen may cause hyperaesthesia and intense lick
dermatitis at the level of the dermatome (Fig. 7).40,41
The owners complaint is typically of their dogs reluctance to jump or of hindlimb lameness but not of
neurological disease.42 In severely affected dogs there
is hindlimb paresis, muscle atrophy, tail paresis, and
sphincter impairment with faecal and/or urinary
incontinence. Lumbo-sacral neurological disorders are
often misdiagnosed as hip dysplasia. Electrodiagnosis
appears to be the most sensitive test to differentiate
these two entities and demonstrates a peripheral nerve
dysfunction. Definitive diagnosis of cauda equina syndrome requires other tests: radiography (survey and
contrast) or high-technology imaging techniques.37

Cutaneous manifestations of central nervous

system (CNS) diseases
Neurogenic pruritus is a rare symptom of CNS lesions,
such as idiopathic seizure-related behaviour, congenital
abnormalities, inflammation or neoplasia.

Occipital bone hypoplasia resulting in overcrowding of

the caudal fossa and secondary syringohydromyelia
has been identified in young Cavalier King Charles
spaniels in Europe, Australia and North America. The
first clinical signs are observed between 6 months and
2 years of age and both sexes are affected.43 Affected
dogs scratch persistently at the cervical and shoulder
region of one or both thoracic limbs. The scratching
becomes more intense if the dog is excited, when a
collar is worn and when the triceps and the shoulder
region is palpated. When walked on a leash, affected
dogs will often pause every few steps to scratch. There
may also be cervical pain with manipulation of the
neck, head tilt, cranial nerve dysfunction, specifically
cranial nerve VIII to XII peripheral neurological deficits, thoracic and/or pelvic limb neurological deficits,
ataxia and sensory deficits and cerebellar signs.44
The cause of this condition remains unclear.
Occipital bone hypoplasia with secondary hydrosyringomyelia (Chiari type I malformation) is supected.
Scratching and paraesthesia is presumed to be a result of
damage to the dorsal horn, decussating spinothalamic
fibres, and interference with the processing of sensory
information.45
The defect in the breed is similar to the Chiari I malformation in humans.45,46 As in humans47 a hereditary
origin most likely to be autosomal recessive is suspected in the Cavalier King Charles spaniel. Chiari
malformation and hydromyelia can be diagnosed by
myelography (enlarged central canal), computed tomography or MRI.48 Ectromyography may show subtle
functional disturbances in the motor unit.49

SE IZ U R E -R E L AT E D D ISO R D E R S
Psychomotor or partial complex seizures
Episodic licking or self-mutilation may be the predominant clinical sign of partial complex seizures, currently
called psychomotor seizures, which are defined as
bizarre behavioural activity with or without motor
seizures.50 Dogs are often non responsive during these
episodes, which may last from minutes to hours. The
behavioural sign may be the only abnormality or may
be followed by a generalized (tonic-clonic) motor
seizure. The affected structures in animals are located
in the temporal lobe and are part of the limbic system,
which serves to modulate emotional expression. Etiopathogeny of this entity includes almost all the causes
of focal and diffuse encephalopathies. Focal cerebral
lesions arise from tumours, post-traumatic scars or
haemorrhagic foci. More commonly, diffuse lesions include
ischemia, anoxia, inflammatory disorders (infectious or
dysimmune) or the seizures themselves. Most cases of
psychomotor seizures reported in dogs are idiopathic.50
Abnormal findings on electroencephalography (EEG)51
brain imaging such as computed-tomography or MRI
together with cerebrospinal fluid analysis, may confirm

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Cutaneous manifestations of neurological diseases

the cerebral dysfunction, localize the lesion and suggest
the nature of the lesion.

Dermatosis induced by behaviour syndromes

Licking or self-mutilation may develop as behavioural
patterns: rituals, displacement behaviours, or stereotypical behaviours. In rituals, the dog or cat scratches
or licks and the owners reaction re-enforces this behaviour. This attention-seeking behaviour is only observed
when a family member is present. Displacement behaviour can occur in anxious or depressed dogs or cats.52,53
Such behaviour may include licking (limbs, tail) sucking (flank, tail), onychophagia or scratching (head,
neck). These displacement activities are arranged in a
behavioural sequence starting with a typical preparatory
phase. Stereotypical behaviours are acts or a small
collection of acts carried out in a predictable pattern
but without obvious purpose. In veterinary medicine,
the distinction between a behaviourally driven action
such as tail chasing or flank sucking from a true neurological defect can be difficult. If dermatological and
peripheral neuropathy are ruled out, it may be difficult
to differentiate a psychomotor seizure from a compulsive aetiology.
Tail chasing, which occurs principally in bull terriers, has been discussed by many authors.5457 This
behaviour may result in self-inflicted wounds to the distal portion of the tail, severe enough in some affected
dogs to expose the dorsal surface of the tail vertebrae.
It has been suggested that this behaviour is caused by
excessive endorphinergic tone.58 This behavioural
pattern has some features in common with lethal acrodermatitis of bull terriers, a dermatological disease that
can be associated with behavioural abnormalities or
neurological disorders due to a heritable defect of zinc
absorption.59 However, in previous reports, the behavioural changes of lethal acrodermatitis and tail chasing
in bull terriers were suggested to be attributed to complex partial seizures, perhaps localized in the temporal
lobe of the brain.54,55 Dodman showed EEG abnormalities (multiple epileptiform spikes with predominantly symmetric, moderate-to-large amplitude, low
frequency waves) in all the dogs affected, often associated with hydrocephalus (six out of seven dogs) and a
variable response to anticonvulsant drugs.55
Other entities, like flank sucking in Dobermans or
Nordic breeds are considered as behavioural disorders
and result in auto-inflicted lesions, but have no known
aetiology. A central nervous system cause has never
been investigated.53 Affected dogs rest for hours with
their head directed towards the flank or perhaps jaws
opened and resting on the skin in the flank region. The
target skin is often covered by saliva, may be discoloured or alopecic60 and may progress to lichenification. The behaviour typically begins around the time of
puberty. A genetic influence is suspected.61

Abnormal movements
Abnormal movement, such as myoclonus, may mimick
continuous pruritus and creates cutaneous lesions by

143

scratching (Fig. 8). Myoclonus is almost pathognomonic for canine distemper encephalomyelitis62 but it has
been observed in other inflammatory CNS disorders63 and
sporadically with lead,64 chlorambucil65 or propofol
toxicity.66 Myoclonus may be associated with other disorders in humans.67 Canine distemper is common in
young unvaccinated dogs, but can occur in susceptible
adult dogs.68 The mechanism of myoclonus is not well
understood but experimental studies have shown focal
spinal cord lesions. The site of damage may be the
lower motor neurones of the spinal cord (limbs and
trunk) or cranial nerve nuclei (head). A spontaneous
depolarization and discharge of the alphamotor
neurones may result from an abnormal pacemaker
activity of the spinal cord motor neuronal pool.62

CNS (brain and spinal cord) tumours with

cutaneous manifestation
In humans, localized pruritus may indicate the presence of a CNS (spinal cord or intracranial) tumour.
Localized rash and pruritus in a dermatomal pattern
associated with spinal cord lesions (tumours or syringohydromyelia) have been described.6971 Reports of
other CNS lesions associated with localized pruritus
include brain abscesses,72 basilar artery aneurysms,73
stroke74,75 and tumours.76 Different pathological mechanisms may be involved according to the primary stimulus (stroke, tumour, etc.). To date, no case associating
a CNS tumour with localized pruritus has been
reported in animals.

C O N C LU SIO N
When evaluating pruritic disease, consideration should
be given to neurological causes. Age, breed, history and
physical findings are important diagnostic aids. A complete neurological examination may be an appropriate
step in the evaluation of a dog or cat with pruritic or
dermatological disease. Ataxia or paresis, postural deficits, modifications of medullary reflexes, cranial nerve
disorders, or conscience or behaviour alterations may be
seen. In veterinary medicine, pruritus or self-mutilation
may be the only clinical sign, and differentiation of
primary dermatological disease from a neurological
disorder may be difficult. The clinical approach to
pruritus should not be restricted to diseases of the skin.

AC K N OW L E D G E M E N T
The authors would like to thank Helen T. Power for her
assistance with this manuscript.

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Rsum Le prurit nest pas toujours li une stimulation cutane. Il peut tre associ un trouble neurologique
ou comportemental. Le rle essentiel du systme nerveux dans le contrle (stimulation et inhibition) du prurit
et sa pathophysiologie sont prsents. Afin de diffrencier les dermatoses prurigineuses primitives et celles lies
un trouble neurologique, les neuropathies priphriques congnitales ou acquises (syndrome d Arnold-Chiari,
syndromes convulsifs, tumeurs du systme nerveux central) sont prsentes.
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K Gnirs and P Prlaud

Resumen El prurito no siempre origina de una estimulacin cutnea asociada a una alteracin dermatolgica
primaria. Puede ser causada por alteraciones neurolgicas o del comportamiento. Presentamos el papel esencial
del sistema nervioso en el control (estmulo e inhibicin) del prurito y su patofisiologa. Para permitir la
diferenciacin entre alteraciones dermatolgicas y neurolgicas, se discuten neuropatas perifricas heredadas o
adquiridas y alteraciones nerviosas centrales (sndroma de Arnold-Chiari, alteraciones asociadas a ataques,
tumores del sistema nervioso central) que pueden inducir prurito.

Zusammenfassung Juckreiz entspringt nicht immer einer Stimulation der Haut in Verbindung mit primren
dermatologischen Strungen. Er kann durch neurologische oder Verhaltensstrungen hervorgerufen werden.
Die essentielle Rolle des Nervensystems bei der Kontrolle (Verstrkung und Hemmung) von Juckreiz und seine
Pathophysiologie wird vorgestellt. Um eine Differenzierung zwischen dermatologischen und neurologischen
Strungen zu erlauben, werden vererbliche oder erworbene periphere Neuropathien und zentralnervse Strungen
(Arnold-Chiari Syndrom, anfallsbedingte Strungen, Tumoren des ZNS), die Juckreiz hervorrufen knnen diskutiert.

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