scholarly journals Hereditary myelopathies in dogs

2021 ◽  
Vol 72 (1) ◽  
pp. 2583
Author(s):  
RD Baka ◽  
ZS POLIZOPOULOU

The purpose of the current review is to summarize data regarding hereditary myelopathies in dogs. Canine degenerative myelopathy (DM) is a progressive disease prevalent in senior (≥8 years old), large breed-dogs, predominating in German shepherd dog. Neurolocalization indicates a thoracolumbar, upper motor neuron, lesion; it can progress to the thoracic limbs and later to lower motor neurons of all limbs resembling human amyotrophic lateral sclerosis (ALS). Tentative diagnosis is based on ruling out other progressive myelopathies. Clinical similarities between ALS and DM made superoxide dismutase 1 gene (SOD1) a viable candidate gene as an etiopathogenic factor. Α E40K missense mutation of the SOD1 has been linked to DM. A genetic test for DM exists, which will aid breeding programs to eliminate the disease. Exercise and physiotherapy are important to slow the progression of DM. Long-term prognosis is poor as dogs become non-ambulatory within 4-6 months from the time of diagnosis. Dystrophic myelopathies include Afgan hound myelopathy/hereditary necrotizing myelopathy in Kooikerhondje dogs, leukoencephalomyelopathy and neuroaxonal dystrophy in Rottweilers and spongiform leukoencephalomyelopathy. A similar myelopathy is Jack Russell and Fox terrier hereditary ataxia. Their etiology is suspected to be hereditary and they appear at a young age. They are diagnosed solely postmortem with histopathological examination. There is no etiologic treatment and the prognosis is poor except for Rottweiler neuroaxonal dystrophy and Jack Russel ataxia due to the extremely slow progression of symptoms. Syringomyelia is characterized by the formation of fluid filled cavities within the spinal cord and outside the central canal that may communicate with the central canal and it is caused mainly due to Chari-like malformation (CLM). The most important clinical sign is neuropathic pain and is localized in the cervical region of the spinal cord. Progression of the disease varies, there are severe disabilities due to pain or it can be an incidental finding. CT and MRI are the preferable ways to detect the lesions. For CLM, therapy includes surgical and medical management. Drugs can be divided into analgesics (non-steroidal anti-inflammatory, gabapentin, pregabalin, tramadol), drugs reducing cerebrospinal fluid (CSF) production (omeprazole, acetazolamide, furosemide) and corticosteroids. Medical therapy diminishes the severity of clinical signs but never succeeds full resolution.

2008 ◽  
Vol 53 (No. 11) ◽  
pp. 636-640 ◽  
Author(s):  
C.C. Perez-Marin ◽  
L. Molina ◽  
J.M. Dominguez ◽  
Y. Millan ◽  
J. Martin de las Mulas

Uterine adenomyosis, a disease not widely addressed in dogs, is characterised by the progressive penetration of endometrial glands and stroma into the myometrium, together with smooth-muscle hyperplasia. This report describes a case of adenomyosis in an 8-year-old German Shepherd bitch with mammary tumours, concomitant with cystic ovarian disease and endometrial cystic hyperplasia. Clinical signs included presence of small nodules and enlargement of mammary glands, and bloody uterine discharge. Ultrasonography confirmed the uterine and ovarian abnormalities, while the diagnosis was later confirmed by histopathological examination. The findings are discussed as possibly related to the reproductive disorders observed, and a hypothetical participation of hormonal factors, as has been described in woman, is suggested. However, further studies must be realized.


1993 ◽  
Vol 5 (4) ◽  
pp. 585-590 ◽  
Author(s):  
K. Paige Carmichael ◽  
Elizabeth W. Howerth ◽  
John E. Oliver ◽  
Kurt Klappenbach

A syndrome resembling previously described feline hereditary neuroaxonal dystrophy (FHND) was diagnosed in a litter of cats. The disorder was characterized by a sudden onset of hind limb ataxia that slowly progressed to hind limb paresis and paralysis. The cats were between 6 and 9 months old when clinical signs were first noted. Histologically, there was marked ballooning of axonal processes, with spheroid formation and vacuolation in specific regions of the brain and spinal cord. Some dystrophic axons contained a central periodic acid-Schiff (PAS)-positive core. Neuronal loss and gliosis were seen in certain brain stem nuclei, spinal cord nuclei, and the cerebellum. Ultrastructurally, there was hypomyelination and dysmyelination of affected axons. The PAS-positive core in dystrophic axons corresponded ultrastructurally with accumulations of electrondense, flocculent, amorphous material. In addition, these axons contained membrane-bound osmiophilic bodies and large nonmembrane-bound vacuoles. The syndrome in this report differs from the previously described FHND in that no inner ear involvement was seen and onset of clinical signs occurred at a later age. In addition, although some of the affected cats did have diluted coat colors, abnormal coat color was not always associated with clinical disease. This disease is similar to juvenile neuroaxonal dystrophy in children and to neuroaxonal dystrophies described in horses, dogs, cattle, and sheep.


1906 ◽  
Vol 45 (1) ◽  
pp. 105-131 ◽  
Author(s):  
Alexander Bruce

The term intermedio-lateral tract was introduced in 1859 (Phil. Trans., 1859, p. 445) by Lockhart Clarke to designate a tract or column of nerve cells in the spinal cord, which he had previously described in 1851 (Phil. Trans., 1851, ii. p. 613) as occupying that portion of the lateral margin of the grey matter which is intermediate between the anterior and posterior cornua. According to Clarke's original account, the column in question was very transparent in appearance, and resembled somewhat the substantia gelatinosa of the posterior horn. It was found in the upper part of the lumbar enlargement, extended upwards through the dorsal region, where it distinctly increased in size, to the lower part of the cervical enlargement. Here it disappeared almost entirely. In the upper cervical region it was again seen, and could be traced upwards into the medulla oblongata, where, in the space immediately behind the central canal, it blended with its fellow of the opposite side. In the more complete account of the tract published in 1859 (p. 446), its component cells are described as in part oval, fusiform, pyriform, or triangular, and as being smaller and more uniform in size than those of the anterior cornua. In the mid-dorsal region, where they are least numerous, they are found only near the lateral margin of the grey matter, with the exception of some cells which lie among the white fibres beyond the margin of the grey substance. In the upper dorsal region the tract is larger, and not only projects further outwards into the lateral column of the white fibres, but also tapers inwards across the grey substance, almost to the front of Clarke's column. In the cervical enlargement it gradually disappears, although it seems to contain, in part at least, a few scattered cells resembling those of the intermedio-lateral tract of the dorsal region. In the upper cervical region, as already stated, it is again seen occupying a lateral horn similar to that found in the dorsal region. It is composed of the same kind of cells, and can be followed up into the medulla, where it is said to give origin to some of the fibres of the vagus and the spinal accessory.


1994 ◽  
Vol 6 (1) ◽  
pp. 62-71 ◽  
Author(s):  
Donal O'Toole ◽  
James Ingram ◽  
Val Welch ◽  
Katie Bardsley ◽  
Tom Haven ◽  
...  

A chronic progressive neurodegeneration, called hereditary porcine neuronal system degeneration (HPNSD), was recognized in a swine herd in Devon, England. Adult pigs that were presumed carriers of the dominantly inherited trait for HPNSD were transferred from England, where a breeding colony was maintained for 9 years, to the Wyoming State Veterinary Laboratory (WSVL) for study. Two litters of affected piglets were born to 2 carrier sows at the WSVL. Clinical signs of muscular tremors, paresis, or ataxia developed at 12–59 days of age in 4 of 6 liveborn pigs. Three other pigs were stillborn. In the 4 affected livebom pigs, clinical signs progressed and included symmetrical (3 pigs) or asymmetrical (1 pig) posterior paresis, bilateral knuckling of metatarsal-phalangeal or carpal joints, poor exercise tolerance, and in 1 pig, marked hind limb hypermetria. A 34-kg gilt exhibiting clinical signs of muscular tremors and posterior paresis and clinical signs for 22 days was euthanized and examined postmortem at 83 days of age. Apart from decubitus ulcers, gross lesions were absent. Microscopically, perikaryal vacuolation and osmiophilic lipid droplets were observed in atrophic alpha motor neurons in the spinal cord. There was axonal (Wallerian) degeneration in sulcomarginal and dorsal spinocerebellar tracts. Axonal degeneration also involved ventral but not dorsal spinal nerve roots, and was present in eight peripheral nerves sampled for histopathology. Changes in skeletal muscles were consistent with denervation atrophy and were most pronounced in M. tibialis cranialis of the 6 muscles sampled. Immunohistochemical staining of spinal cord for phosphorylated and nonphosphorylated neurofilaments did not reveal abnormal patterns, unlike some well-characterized inherited motor neuron diseases in other species.


2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Gerlinde J. Wunderink ◽  
Ursula E. A. Bergwerff ◽  
Victoria R. Vos ◽  
Mark W. Delany ◽  
Dorien S. Willems ◽  
...  

Abstract Background This case report describes the clinical signs of a calf with focal diplomyelia at the level of the fourth lumbar vertebra. Magnetic resonance imaging (MRI) images and histological findings of the affected spinal cord are included in this case report. This case differs from previously reported cases in terms of localization and minimal extent of the congenital anomaly, clinical symptoms and findings during further examinations. Case presentation The calf was presented to the Farm Animal Health clinic, Faculty of Veterinary Medicine, Utrecht University, with an abnormal, stiff, ‘bunny-hop’ gait of the pelvic limbs. Prominent clinical findings included general proprioceptive ataxia with paraparesis, pathological spinal reflexes of the pelvic limbs and pollakiuria. MRI revealed a focal dilated central canal, and mid-sagittal T2 hyperintense band in the dorsal part of the spinal cord at the level of the third to fourth lumbar vertebra. By means of histology, the calf was diagnosed with focal diplomyelia at the level of the fourth lumbar vertebra, a rare congenital malformation of the spinal cord. The calf tested positive for Schmallenberg virus antibodies, however this is not considered to be part of the pathogenesis of the diplomyelia. Conclusions This case report adds value to future clinical practice, as it provides a clear description of focal diplomyelia as a previously unreported lesion and details its diagnosis using advanced imaging and histology. This type of lesion should be included in the differential diagnoses when a calf is presented with a general proprioceptive ataxia of the hind limbs. In particular, a ‘bunny-hop’ gait of the pelvic limbs is thought to be a specific clinical symptom of diplomyelia. This case report is of clinical and scientific importance as it demonstrates the possibility of a focal microscopic diplomyelia, which would not be evident by gross examination alone, as a cause of hind-limb ataxia. The aetiology of diplomyelia in calves remains unclear.


2018 ◽  
Vol 44 (1) ◽  
pp. 10
Author(s):  
Larissa Garbelini Valentim ◽  
Rogério Anderson Marcasso ◽  
Ana Paula Frederico Loureiro Bracarense ◽  
Mônica Vicky Bahr Arias

Background: Various diseases can cause spinal cord disorders in dogs including neoplasia of the nervous system, which are more common in mature and older animals. Some breeds, as brachycephalics, are more predisposed for certain types of tumors. Spinal neoplasia can be categorized as primary or secondary tumors, and still can be grouped in extradural, intradural/extramedullary or intramedullary. The aim of this study was to investigate the signalment, neurological syndrome, type and source of the mass, presence of metastases, and clinical outcomes in 28 dogs diagnosed with spinal tumors.Materials, Methods & Results: This study included 28 dogs, and in 20 cases, spinal neoplasia was confirmed by necropsy, biopsy, histopathology, and immunohistochemistry. In the other eight animals, the presumptive diagnosis was based on clinical and neurological signs and by observing changes in plain radiographs of the spine, chest radiographs, and myelography. The location of the lesion was established as cervical, cervicothoracic, thoracolumbar, or lumbosacral, and the lesion was determined to be multifocal when clinical signs appeared in more than one location. Spinal cord injury was additionally classified as unilateral, bilateral, symmetrical, or asymmetrical. The following complementary examinations were performed based on the clinical suspicion, indications, and availability of the owner: complete blood count, serum biochemical profile, urinalysis, spinal radiography, cerebrospinal fluid analysis (CSF), myelography, abdominal ultrasound, thoracic X-ray to determine metastases, and Computed Tomography. CSF was collected and analyzed in seven animals, and pleocytosis with a predominance of lymphocytes was found in two cases. Decompression and exploratory surgeries were performed in six dogs to confirm the suspected diagnosis and collect tissues for histopathology. The final diagnosis was made by histopathological examination of the tissue obtained by excisional biopsy or necropsy after the death of the animal or euthanasia. In eight cases, the neoplasia type could not be confirmed as the owner did not provide consent to perform exploratory surgery or necropsy. The dogs most affected were poodles (17.85%) and boxers (17.85%), between the ages of 5 and 10 years, and 67.85% of the cases were female dogs. The thoracolumbar region was the most affected (50%). Metastatic spinal tumors predominated; especially those originating in the mammary gland (38.46%) and on the skin (34.61%), and among the primary neoplasms, meningiomas predominated. The clinical course in relation to the onset of symptoms of neurological dysfunction, until death or euthanasia, ranged from 1-170 days, with an average 42 days of survival.Discussion: The results differ from previous studies, in which the number of male and female dogs with neoplasms in the CNS was the same, as in the present study female dogs were more affected. Unlike other studies, metastatic spinal tumors predominated. After the diagnosis, the survival was short and this may be due to the fact that most dogs in this study already had more than one system affected, and consequently showed significant changes related to systemic metastases during the onset of the neurological symptoms. The surgical treatment presented variable results, and the results observed after surgical resection of neoplasms vary due to the location in relation to the spinal cord, neoplasm type, severity of the clinical signs, and possibility of removal along with margin without causing spinal instability. In dogs with spinal disorders, the veterinary practitioner should suspect cancer, especially if there is a history of excision of neoplasms in other systems.


Neurosurgery ◽  
1987 ◽  
Vol 21 (2) ◽  
pp. 235-238 ◽  
Author(s):  
Yuk-keung Lo ◽  
David Chao ◽  
Sui-hang Yan ◽  
Hsiu-chi Liu ◽  
Fu-li Chu ◽  
...  

Abstract A 43-year-old woman suffered from low back pain and bilateral footdrop. A cisternal myelogram unexpected revealed multiple filling defects in the spinal canal extending from the lower cervical region to the caudal equina. Diagnostic exploration revealed numerous cystic organisms adhering to the spinal cord and nerve roots. Histopathological examination showed these organisms to be proliferative sparganum cestode larvae. Although these cestode larval infections have been reported a dozen times in humans from various parts of the world, this is probably the first reported case of spinal cord infection.


2020 ◽  
Vol 9 (11) ◽  
pp. 3395
Author(s):  
Chang-Hoon Koo ◽  
Jung-Hee Ryu ◽  
Jin-Young Hwang ◽  
Jin-Hee Kim ◽  
Hyun-Jung Shin ◽  
...  

Spinal cord ischemia is one of the most serious complications of aortic repair in patients with acute aortic syndrome. However, the effect of hypotension before aortic clamping on spinal cord injury has not been documented. A total of 48 male Sprague-Dawley rats were randomly divided into four groups: the sham group; control group (mean arterial pressure (MAP) < 90% of baseline value before aortic clamping); mild hypotension group (MAP < 80%); and profound hypotension group (MAP < 60%). Spinal cord ischemia was induced using a balloon-tipped catheter placed in the descending thoracic aorta. Neurological function of the hind limbs was evaluated for seven days after reperfusion and recorded using a motor deficit index (MDI). The spinal cord was then harvested for histopathological examination and evaluation of oxidative stress and inflammation. The profound hypotension group demonstrated a significantly higher MDI 48 h post-reperfusion and lower number of normal motor neurons than the other groups (p < 0.001). The levels of tissue malondialdehyde and tumor necrosis factor-α (TNF-α) were also significantly increased in the profound hypotension group compared with other groups. Profound hypotension before aortic clamping can aggravate neurologic outcomes after aortic surgery by exacerbating neurologic injury and reducing the number of normal motor neurons.


2018 ◽  
Vol 37 (02) ◽  
pp. 140-144
Author(s):  
Palanisamy Seerangan ◽  
Aravinth Ashok ◽  
Jolarpettai Mahendran

Introduction Inclusion cysts of the spinal cord are rarely intramedullary. Such cysts are commonly located in the lumbar and thoracic regions and are usually associated with congenital spinal dysraphism and dermal sinus. Intramedullary dermoid cysts in the cervical region without spinal dysraphism are extremely rare. To our knowledge, only seven such cases are reported in the literature to date. Materials and Methods An 18-year-old female patient presented with weakness in all four limbs, more distal than proximal muscle weakness, that had been progressing for 3 years. The magnetic resonance imaging (MRI) showed an intramedullary lesion from C5– C7 with peripheral ring enhancement. “Whorls” were observed within the lesion on T2 weighted image, with associated excavation of vertebral bodies C5– C7. Operative procedure and findings: partial laminectomy of C5– D1was performed. The dura was opened. A small myelotomy was made in the root entry zone. About 1.5 ml of yellowish colored fluid was drained. White shiny debris with hair, whitish pultaceous content and teeth were removed. Complete excision of cyst and its wall was performed. Results The histopathological examination revealed that the cyst wall was lined by stratified squamous epithelium with underlying dermis showing hair follicles, sebaceous glands, adipose tissue and cyst filled with keratin debris suggestive of dermoid cyst. Conclusion The intramedullary location of the dermoid cyst in the cervical cord and the absence of any congenital spinal dysraphism make this case a very unique and rare entity and warrants its inclusion in the reported cases of rare intramedullary space occupying lesions.


2002 ◽  
Vol 97 (2) ◽  
pp. 161-165 ◽  
Author(s):  
Langston T. Holly ◽  
Ulrich Batzdorf

Object. The authors review their experience in the diagnosis and management of 32 patients with slitlike syrinx cavities. Methods. There were 18 men and 14 women with a mean age of 40 years. Presenting symptoms that prompted magnetic resonance (MR) imaging evaluation were mechanical spinal pain (13 patients), radicular pain (seven patients), paresthesia (six patients), numbness (five patients), and muscle spasm (one patient). In 12 patients neurological examination demonstrated normal status, and in the remainder only minimal sensory or motor abnormalities were found. The mean diameter of the syrinx cavity was 2 mm (range 1–5 mm), and on average it covered three vertebral levels. The cavities were limited to the cervical region in 16 patients, the thoracic in 12, and both regions in four patients. The mean follow-up time for changes in clinical condition and repeated MR imaging features were 38 and 32 months, respectively. Thirty-one patients were treated nonoperatively, and one was treated surgically. During the follow-up period clinical improvement was documented in six patients, worsened status in seven, and no change was demonstrated in the clinical status of 19 patients. None of the syrinx cavities changed in size. In 16 patients medical workup revealed alternative diagnoses that were determined to be the true causes of each patient's symptoms. Conclusions. Slitlike cavities likely do not represent true syringomyelia but rather remnants of the central canal detected in a small percentage of adults. Review of the authors' experience indicates that these cavities are asymptomatic and are unlikely to change in size. They can be considered an incidental finding, and in many of these patients another condition explaining the patient's symptoms may be found.


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