Equine motor neuron disease; a preliminary report

A spontaneous motor neuron disease or neuronopathy was identified in 10 horses from the northeastern United States. Signs of generalized weakness, muscle fasciculations, muscle atrophy and weight loss progressed over 1 to several months in young and old horses of various breeds. Pathologic studies revealed that degeneration and loss of motor neurons in the spinal cord and brain stem resulted in axonal degeneration in the ventral roots and peripheral and cranial nerves and denervation atrophy of skeletal muscle. Many spinal neurons were swollen, chromatolytic and contained neurofilamentous accumulations. Other cell bodies were shrunken and undergoing neuronophagia and some were lost and replaced by glia. This fatal equine motor neuron disease has not been reported previously and its cause has not been determined. The progressive weakness and wasting and the neuronal degenerative changes in these horses were similar to those described in people with sporadic amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.     

Spinal cord medulloepithelioma in a dog

Medulloepithelioma, an embryonal neural tumor, was diagnosed in a 6-month-old Bullmastiff. Clinical signs of incomplete, upper motor neuron, transverse myelopathy involving the hindlimbs were observed on examination. Myelography was required to identify the cause of clinical abnormality. The tumor involved the L1 spinal cord segment.     

Developmental delay in islet-1-positive motor neurons in chick spina bifida

Spina bifida aperta (SBA) is a congenital malformation of the spinal cord with complications such as spinal ataxia and bowel and bladder dysfunction. We have developed a chick model with surgery-induced SBA that shows spinal ataxia after hatching. In the present study, motor neurons in the early stages in chicks with and without SBA were observed by immunohistochemical staining with a monoclonal antibody against Islet-1, a motor neuron marker. Delay in migration and maturation of motor neurons was observed in SBA. Although the final numbers of Islet-1-positive neurons in these two groups were not different, a defect in the production and elimination of excess motor neurons in the early developmental stages in the SBA group may be involved in the pathological mechanism of the motor complications of this disease.     

Gliomatosis cerebri in two dogs

A 3.5 yr old Saint Bernard was evaluated for nonambulatory tetraparesis and cranial nerve dysfunction, and a 7 yr old rottweiler was evaluated for progressive paraparesis. Clinical signs of left-sided vestibular and general proprioceptive ataxia and cranial nerve VII dysfunction in the Saint Bernard suggested a lesion affecting the brain stem. Signs in the rottweiler consisted of general proprioceptive/upper motor neuron paraparesis, suggesting a lesion involving the third thoracic (T3) to third lumbar (L3) spinal cord segments. MRI was normal in the Saint Bernard, but an intra-axial lesion involving the T13-L2 spinal cord segments was observed in the rottweiler. In both dogs, the central nervous system (CNS) contained neoplastic cells with features consistent with gliomatosis cerebri (GC). In the Saint Bernard, neoplastic cells were present in the medulla oblongata and cranial cervical spinal cord. In the rottweiler, neoplastic cells were only present in the spinal cord. Immunohistochemistry disclosed two distinct patterns of CD18, nestin, and vimentin staining. GC is a rarely reported tumor of the CNS. Although GC typically involves the cerebrum, clinical signs in these two dogs reflected caudal brainstem and spinal cord involvement.     

Intraoperative use of ultrasonography during continuous dorsal laminectomy in two dogs with caudal cervical vertebral instability and malformation ("Wobbler syndrome")

OBJECTIVE: To describe an intraoperative ultrasound imaging technique during dorsal laminectomy in 2 dogs with caudal cervical vertebral instability and malformation (CCVIM, "Wobbler syndrome"). STUDY DESIGN: Clinical case report. SAMPLE POPULATION: Two dogs with CCVIM. RESULTS: On neurologic examination there was tetraparesis with upper motor neuron signs in the thoracic limbs and lower motor neuron signs in the pelvic limbs in dog 1, and hyperreflexia of the rear limbs, normoreflexia of the right front limb, and hyporeflexia of the left front limb of dog 2. Both dogs had signs of marked cervical pain and radiographic signs of cervical spinal cord compression. Intraoperative ultrasonography of the spinal cord revealed protruding intervertebral disc at C5-6 (dog 1) and C6-7 (dog 2), and the parallel borders of the spinal cord and central canal after decompression. Continuous dorsal laminectomy (CDL) resulted in improvement over 16 months (dog 1) and 20 months (dog 2). CONCLUSIONS: Intraoperative ultrasonographic imaging of the cervical spinal cord after CDL was helpful in determining adequate decompression (postlaminectomy) of the spinal cord in relation to the ventral and lateral compressive component(s) and to image the protruding intervertebral disc. CLINICAL RELEVANCE: Intraoperative ultrasonography can be used to provide valuable information on the spinal cord and surrounding soft tissues for the neurosurgeon.     

A lower motor neuron disease in newborn Romney lambs

AIM: To determine the cause and nature of a disease in newborn New Zealand Romney lambs characterised by progressive weakness and premature death. METHODS: Affected lambs were examined clinically, humanely killed and submitted to necropsy. Selected fonmalin-fixed tissues were examined histologically. Data on the parentage of the lambs were collected. RESULTS: The principle lesions found were degeneration and loss of neurons in ventral horns of the spinal cord and brain stem and Wallerian degeneration of motor nerves and denervation atrophy of skeletal muscles fibres. CONCLUSION: The lesions are those of a lower motor neuron disease which appeared to have a genetic cause.     

A progressive neuronopathy in the young cairn terrier

The clinical signs and neuropathological changes of a nervous disease of pedigree cairn terriers are described. Three animals, of both sexes, between five and seven months of age showed hind leg weakness, quadriparesis, ataxia, loss of superficial and deep reflexes and tremor of the head. Pathologically there was chromatolysis of neurons in the spinal cord and brain stem. Although Wallerian-type degeneration was present in the spinal cord and peripheral nerves, its severity did not correlate with the intensity of the neuronal changes. This suggests that the chromatolysis observed may reflect a primary neuronal abnormality rather than represent a secondary change resulting from a primary degeneration of peripheral axons. Although the age of onset and some of the clinical signs are similar to those seen with globoid cell leucodystrophy (an inherited disease which also affects cairn terriers), the presence of lower motor neuron paralysis and the absence of signs of severe brain involvement in progressive neuronopathy should enable the conditions to be distinguished clinically.     

Syringohydromyelia in Cavalier King Charles spaniels

Syringohydromyelia secondary to foramen magnum overcrowding is described in seven Cavalier King Charles spaniels. Clinical signs were consistent with a central spinal cord lesion. The most common signs were persistent scratching at the shoulder region with apparent neck, thoracic limb, or ear pain and thoracic limb lower motor neuron deficits. The diagnosis was made by magnetic resonance imaging. The syringohydromyelia is postulated to be a consequence of an occipital bone malformation resulting in a small caudal fossa and cerebellar herniation. Clinical signs improved but did not completely resolve when the dogs received treatment with corticosteroids or nonsteroidal anti-inflammatory drugs.     

出生前山羊颈段脊髓运动神经元的发育

应用HE染色和超薄切片技术研究第6周到出生前山羊脊髓腹角运动神经元胞体的发育变化。结果表明：1．出生前山羊脊髓腹角运动神经细胞发育包括未分化期、不成熟期、发育成熟期和成熟期4个阶段。2．发育过程中，山羊脊髓运动神经元细胞核的体积持续增长；常染色质增加，异染色质减少且趋边分布；核仁数量减少，中央核仁在第8周形成，以后逐渐发育成熟；核膜渐趋成熟。3．山羊脊髓运动神经元胞体内膜系统的发育变化的规律与神经元的发育阶段相适应。     

Thoracolumbar spinal cord compression due to vertebral process degenerative joint disease in a family of Shiloh Shepherd dogs

Five young Shiloh Shepherd Dogs (4 males and 1 female) related by a common sire were studied because of progressive pelvic limb weakness and incoordination. All dogs had a spastic paraparesis and pelvic limb ataxia consistent with an upper motor neuron and general proprioceptive lesion between spinal cord segments T3 and L3. Proliferative lesions involving one or more of the articular processes from the 11th thoracic vertebrae to the 2nd lumbar vertebra were observed on radiographs of the thoracolumbar vertebrae. Dorsal compression of the spinal cord was identified during imaging studies at these sites. Abnormalities of the synovial joints and bony proliferation of the involved articular processes were identified at postmortem examination in 2 dogs. The articular processes and associated vertebral arches protruded into the vertebral canal, indenting the dorsal surface of the spinalcord. Degenerative joint disease (DJD) was identified histologically. A compressive myelopathy was diagnosed in the spinal cord. These dogs were affected by a compressive myelopathy as a consequence of vertebral process DJD that likely has a geneticcomponent. The DJD could have been caused by a primary vertebral malformation or an injury to the processes at a young age causing malarticulation.     

Myelopathy caused by a histiocytic sarcoma in a cat

An eight-year-old, female spayed, domestic shorthair cat presented with a three-week history of progressive general proprioceptive ataxia and upper motor neuron paresis of the hindlimbs. Computed tomography revealed a mediastinal mass invading the vertebral canal with the T1 spinal nerve and roots, causing extramedullary compression of the cranial thoracic spinal cord. Histopathological and immunohistochemical studies of the mass during postmortem examination disclosed a neoplasm, later determined to be a poorly differentiated histiocytic sarcoma. Feline histiocytic tumours are rare, with only two prior reports existing in the veterinary literature. This report details a case work-up and reviews the literature on feline histiocytic diseases and tumours affecting the feline spinal     

Reduced tongue tone associated with degeneration of the hypoglossal nerve nucleus in a horse with equine motor neuron disease

Equine motor neuron disease (EMND) is a condition characterised by generalised weakness and muscle atrophy associated with degeneration of motor neurons in the ventral horns of the spinal cord. Despite the frequent detection of cranial nerve nuclei pathology during post mortem examination, associated clinical signs are rarely reported. This report describes a case of EMND in a pony gelding that presented with clinical signs of diffuse neuromuscular weakness associated with marked flaccidity of the tongue, making differentiation from similar neuromuscular conditions, particularly botulism, extremely challenging.     

Peripheral nerve biopsy for diagnosis of globoid cell leukodystrophy in a dog

Saphenous nerve biopsy was performed to diagnose globoid cell leukodystrophy in a 3-month-old West Highland White Terrier. The dog had progressive neurologic disease that appeared to involve the cerebellum, spinal cord, and lower motor neurons to the hind limbs. Light and transmission electron microscopic findings of the nerve biopsy specimen established the diagnosis. Peripheral nerve biopsy may provide a reliable antemortem method for diagnosis of globoid cell leukodystrophy.     

Comparison of arylalkylamine N-acetyltransferase and melatonin receptor type 1B immunoreactivity between young adult and aged canine spinal cord

Melatonin affects diverse physiological functions through its receptor and plays an important role in the central nervous system. In the present study, we compared immunoreactivity patterns of arylalkylamine N-acetyltransferase (AANAT), an enzyme essential for melatonin synthesis, and melatonin receptor type 1B (MT2) in the spinal cord of young adult (2~3 years) and aged (10~12 years) beagle dogs using immunohistochemistry and Western blotting. AANAT-specific immunoreactivity was observed in the nuclei of spinal neurons, and was significantly increased in aged dog spinal neurons compared to young adult spinal neurons. MT2-specific immunoreactivity was found in the cytoplasm of spinal neurons, and was predominantly increased in the margin of the neuron cytoplasm in aged spinal cord compared to that in the young adult dogs. These increased levels of AANAT and MT2 immunoreactivity in aged spinal cord might be a feature of normal aging and associated with a feedback mechanism that compensates for decreased production of melatonin during aging.     

Weakness associated with spinal subpial myelopathy in a weimaraner puppy

Clinical and pathological features are described in an eight-week-old weimaraner puppy which showed hindleg weakness and subpial myelin depletion in the spinal cord. The myelopathy was accompanied by pial thickening, vacuolation of sensory neurons in the spinal cord and of neurons in the medulla. Swollen axons occurred in the spinal cord, cerebellum and internal capsule. Comparisons are made between this case and a recently recorded form of hypomyelination in weimaraner dogs.     

A novel movement disorder in related male Labrador Retrievers characterized by extreme generalized muscular stiffness

Objectives: To describe the clinical phenotype of a new motor disorder in Labrador Retrievers. Animals and Methods: Case series study. Seven young male Labrador Retrievers presented for evaluation of stiff gait. Results: All affected dogs had generalized muscular stiffness, persistent at rest and resulting in restricted joint movements. They showed a forward flexed posture, festinating gait, and bradykinesia. Signs developed between 2 and 16 months of age and tended to stabilize in adulthood. Needle electromyogram in the conscious state showed continuous motor unit activity in resting epaxial and proximal limb muscles. This activity was abolished by general anesthesia. Muscle and nerve histopathology was normal. In 2 dogs necropsied, astrocytosis was evident throughout the spinal cord gray matter, reticular formation and caudate nuclei. Decreased neuronal counts were selectively found in the spinal cord Rexed's lamina VII, but not in VIII and IX. Pedigree analysis showed that the affected dogs were from 5 related litters. Conclusions and Clinical Importance: This new hypertonicity syndrome in Labrador Retrievers is unique because of the selective distribution of the histological lesions, the lack of progression in adulthood, and its exclusive occurrence in male dogs. Pedigree analysis suggests an X‐linked hereditary disease, although other modes of inheritance cannot be ruled out with certainty. We hypothesize that altered output from basal nuclei and reticular formation together with motor neuron disinhibition caused by a decreased number of spinal cord interneurons leads to the muscular stiffness.     

Fecal incontinence and spinal cord abnormalities in seven dogs

Seven dogs with fecal incontinence and abnormal gaits were evaluated. Fecal incontinence was characterized as defecation of normal stools without posturing. Duration of clinical signs prior to evaluation ranged from 5 months to 3 years. Five dogs had upper motor neuron (UMN) paraparesis, and 2 dogs had UMN tetraparesis. With magnetic resonance imaging, spinal cord abnormalities primarily involving the dorsal aspect of the spinal cord were identified in all dogs. Five dogs had focal abnormalities, and 2 dogs had diffuse abnormalities of the spinal cord. Of the dogs with focal spinal cord lesions, 4 had cystic spinal cord abnormalities and 1 had a meningioma. Surgery was performed on all dogs with focal lesions; 4 of the 5 dogs had resolution of fecal incontinence after surgery. Results in these dogs suggest that fecal incontinence can be associated with spinal cord abnormalities and, depending on the characteristics of the lesion, can resolve after surgical treatment of the abnormality.     

The histopathogenesis of paralytic rabies in six-week-old C57BL/6J mice following inoculation of the CVS-11 strain into the right triceps surae muscle

A fatal encephalomyelitis was developed after intracerebral and hind limb inoculation of in 6-week-old C57BL/6J mice by the inoculation of fixed rabies virus (CVS-11 strain), intracerebrally and into hind. After the intracerebral inoculation, virus antigens were detected in the cerebral cortex and hippocampus at 2 days postinoculation (PI), and later spread centrifugally to thalamus, brain stem, cerebellum, spinal cord and spinal ganglia. At 4 days PI, severe apoptosis and DNA fragmentation were observed in the hippocampus and cerebral cortex. All mice infected intracerebrally were dead without limb paralysis at from 10 to 11 days PI. In contrast, mice infected with virus intramuscularly were persistently observed virus antigens in the myocytes at the site of inoculation from 2 days PI. At 4 days PI, the antigens were demonstrated in the spinal dorsal root ganglia, spinal cord and muscle spindles without their detection in the cerebrum and hippocampus. There were no apoptosis in the spinal cord and dorsal root ganglia, however hind limb paralysis was found in all infected mice. Hind limb paralysis was progressed to quadriparalysis, and mice were dead from 11 to 13 days PI. From 4 days PI, necrosis of neuron was observed in the the spinal and dorsal ganglia with infiltration of lymphocyte. This study suggested that the necrosis of spinal neurons was more important to cause the paralysis of hind limb rather than the severe cerebral infection and apoptosis in C57BL/6J mice infected with CVS-11 strain. The virus primarily replicated in the muscles was ascended the spinal cord via afferent fibers and retrogradely invaded the cerebrum, and with subsequent spread to muscle spindles.     

北京鸭迷走背核向脊髓的直接投射——HRP法研究

采用HRP逆行追踪法,对25例北京鸭迷走背核直接投射到脊髓的传导通路的起始部位进行了研究。乌拉坦(Urethane)静脉注射麻醉动物,分别在脊髓的颈中部(C7)、颈膨大部和腰膨大部注时30～50％HRP,灌流固定,取脑做冰冻连续切片,蓝色反应显色,中性红复染,镜检。实验结果:单侧脊髓注射HRP后,在延髓的闩后部分,双侧的迷走背核内发现了标记细胞,对侧的标记细胞数量多于同侧。此外,双侧的疑核和孤束核也有一些标记细胞。在颈中部脊髓引入HRP后,出现的标记细胞较多;在颈膨大部引入HRP后,出现的标记细胞较少;在腰膨大部引入HRP后,迷走背核内不出现标记细胞,而疑核和孤束核仍有少量标记细胞。本文对禽类迷走背核和疑核至脊髓的直接传导通路,结合哺乳类的有关资料进行了讨论。     

Fibrocartilaginous emboli.

Fibrocartilaginous embolism (FCE) is an acute ischemic myelopathy, primarily of large or giant breed dogs, which results from occlusion of blood vessels within the spinal cord parenchyma or the adjacent leptomeninges by masses of fibrocartilage. Lateralizing and asymmetric neurologic deficits are very suggestive of spinal cord infarction. The diagnosis of FCE is made by eliminating causes of acute compressive myelopathy such as trauma and intervertebral disc herniation. Patients with lower motor neuron deficits secondary to FCE have a more guarded prognosis than those with upper motor neuron deficits. In most instances, if recovery is to occur, improvement will be evident within the first 10 days after the onset of clinical signs.