Distal peripheral polyneuropathy in a great dane

A spayed female five year old Great Dane dog was diagnosed as having a chronic, progressive, symmetrical distal polyneuropathy and concurrent hypothyroidism. Axonal degeneration and segmental demyelination were evident in teased nerve fiber preparations. Clinical signs included hindlimb weakness and muscle atrophy of the head and distal limbs. Diagnosis was based on clinical, electrophysiological, and nerve and muscle biopsy findings. Thyroxine supplementation for one month was of no benefit. The etiology of the polyneuropathy was not established but several causes were considered. The extent of demyelination in our case was of greater magnitude than described in a previous report of a similar idiopathic distal symmetrical polyneuropathy in a Great Dane.     

Myotonic dystrophy-like disease in a dog

A mature female Rhodesian Ridgeback was determined to have a progressive, degenerative myopathy associated with myotonia, dysphagia, and marked muscle wasting. Clinical findings revealed a diffuse muscular disease with percussion dimpling, dysphagia, and creatine kinase elevation. A paroxysmal atrial tachycardia was found. Electromyography revealed a diffuse myopathy with high-frequency bizarre waves, myotonic discharges especially in the masticatory, laryngeal, and pharyngeal muscles. A few positive sharp waves were found in some of the appendicular muscles. Histopathologic and histochemical stains on skeletal muscle biopsy specimens demonstrated moderate fiber-size variation, myofiber architectural changes, muscle-fiber splitting, focal necrosis and phagocytosis, high percentage of internal nuclei, and atrophy of type-2 muscle fibers. A review of myotonic myopathies in the dog is presented. The clinical, electrophysiologic, and histochemical findings are similar to those for myotonic muscular dystrophy in man.     

Tongue atrophy as a neurological finding in hereditary polyneuropathy in Alaskan malamutes

BackgroundTongue atrophy with wrinkling as a clinical sign of inherited polyneuropathies has not been reported in dogs.ObjectivesClinically describe tongue atrophy as well as morphology of the tongue and hypoglossal nerve in Alaskan malamute polyneuropathy (AMPN).AnimalsSix client‐owned Alaskan malamute dogs diagnosed with AMPN, all homozygous for the causative mutation in the N‐myc downstream‐regulated gene 1 (NDRG1) and 1 neurologically normal control Alaskan malamute.MethodsProspective case study. Clinical and neurological examinations were performed on affected dogs. Necropsy samples from the tongue muscle and hypoglossal nerve were examined by light and electron microscopy.ResultsAll affected dogs had abnormal wrinkles and grooves on the dorsal surface of the tongue, a clinical sign not described previously in dogs with AMPN. Electromyography of the tongue performed in 2 dogs showed spontaneous activity. Five affected dogs underwent necropsy studies. Histopathology of the tongue showed groups of angular atrophic myofibers and changes in the hypoglossal nerve included thinly myelinated fibers, small onion bulbs, folded myelin, and axonal degeneration.Conclusion and Clinical ImportanceHistopathologic changes in the tongue and hypoglossal nerve were consistent with previously reported changes in skeletal muscle and other nerves from dogs with AMPN. Therefore, we conclude that macroscopic tongue atrophy is part of the disease phenotype of AMPN and should be considered a potential clinical sign in dogs with polyneuropathies.     

Hereditary encephalomyelopathy and polyneuropathy in an Alaskan husky

An Alaskan husky puppy was examined for a neurologic disease which began at six weeks of age with generalised paresis that progressed resulting in recumbency by 18 weeks. Thoracic limbs primarily exhibited lower motor neuron signs that included distal muscle atrophy and persistent elbow and carpal flexion that resisted manual extension. Pelvic limb signs primarily exhibited upper motor neuron and general proprioceptive deficits, but also included lower motor neuron signs. Abnormal vocalisation suggested a laryngeal paresis. Histopathologic lesions included a diffuse axonopathy and secondary demyelination in the nerves of the limbs and larynx and a similar bilaterally symmetrical degeneration in the spinal cord white matter suggestive of a dying back axonopathy. In addition, a degenerative process was present in nuclei in the brain stem and cerebellum. Recognition of this disease through clinical and pathologic examination in other related Alaskan Huskies suggested an autosomal recessive inherited disorder.     

Prednisolone-responsive neuropathy in a cat

Chronic relapsing polyneuropathy was diagnosed in a 15-month-old cat with a 12-week history of limb weakness. The clinical course was punctuated by spontaneous remissions and relapse. There were two striking physical findings, weak withdrawal reflexes and atrophy of the proximal and distal limb muscles. Electrophysiological findings typical of a demyelinating motor neuropathy were present, namely small, dispersed compound muscle action potentials, markedly slow motor conduction and denervation potentials that were more prominent dis-tally. Muscle biopsies showed changes consistent with denervation and a paucity of myelinated axons in intramuscular nerve bundles. The neuropathy responded rapidly and completely to prednisolone administration, which was slowly tapered over several months.     

Bilateral Iliopsoas Muscle Contracture and Spinous Process Impingement in a German Shepherd Dog

Objective— To report diagnosis and treatment of bilateral iliopsoas muscle contracture in a dog with spinous process impingement. Study design— Case report. Animals— German Shepherd dog. Methods— A dog with chronic progressive lameness, flexion contracture of the coxofemoral joints, severe pain, and decreased femoral reflexes had severe spondylosis bridging the vertebral bodies from L1 to L4 and enlarged dorsal spinous processes from T8 to L6 with impingement and bony proliferation. Ultrasonographic and magnetic resonance imaging (MRI) findings were consistent with fibrosis, mineralization, and atrophy of the iliopsoas muscles bilaterally which was treated by staged tenectomy of the insertions of the iliopsoas muscles. Results— Because of severe perivascular fibrosis, the femoral vessels required ligation. Bilateral iliopsoas muscle tenectomy improved gait and provided pain relief. Histologic findings were consistent with fibrotic myopathy. Conclusions— Slow progression of severe clinical signs observed bilaterally in this dog differs from previous reports of iliopsoas myopathy. Findings were similar to the fibrotic myopathy of the gracilis or semitendinosus muscles described in dogs. Clinical Relevance— Iliopsoas muscle abnormalities should be considered in dogs with limited hip extension and pain. MRI is useful for diagnosing muscle fibrosis. Iliopsoas tenectomy may improve clinical function in dogs with fibrotic myopathy.     

Dystrophin‐deficient muscular dystrophy in a Norfolk terrier

A six‐month‐old male entire Norfolk terrier was presented with a 3‐month history of poor development, reluctance to exercise and progressive and diffuse muscle atrophy. Serum creatine kinase concentration was markedly elevated. Magnetic resonance imaging of the epaxial muscles revealed asymmetrical streaky signal changes aligned within the muscle fibres (hyperintense on T2‐weighted images and short‐tau inversion recovery with moderate contrast enhancement on T1‐weighted images). Electromyography revealed pseudomyotonic discharges and fibrillation potentials localised at the level of the supraspinatus, epaxial muscles and tibial cranialis muscles. Muscle biopsy results were consistent with dystrophin‐deficient muscular dystrophy. The dog remained stable 7 months after diagnosis with coenzyme Q10 and l ‐carnitine; however after that time, there was a marked deterioration and the owners elected euthanasia. This case report describes the clinical presentation, magnetic resonance imaging, electrodiagnostic and histopathological findings with immunohistochemical analysis in a Norfolk terrier with confirmed dystrophin‐deficient muscular dystrophy, which has not been previously described in this breed.     

Clinical signs and pathology of accidental monensin poisoning in sheep

The clinical signs and postmortem findings in sheep from two flocks accidentally poisoned with monensin are described. Clinical signs began within 24 hours of exposure to monensin. In the acute stages they consisted of lethargy, stiffness, muscular weakness, a stilted gait and recumbency. Feed refusal was seen in one flock but not in the second. Subacute to chronic clinical signs were decreased muscle volume of the rump and thigh. When forced to run, chronically affected sheep had a stilted, stiff legged, rocking horse gait.

Gross postmortem changes were not always visible. Where visible, they affected skeletal muscles and consisted of pale streaking, with atrophy in the chronic stages. Lesions were most severe in muscles of the rump and hind limbs. Microscopically myofiber swelling and hyalinization were seen with interstitial mononuclear cell reaction and extensive sarcoplasmic mineralization in some cases. Chronic lesions consisted of fibrosis and myofiber atrophy. In lambs less than one month old, diffuse gastrointestinal hemorrhage was the only finding.

     

Spastic syndrome in a Holstein bull: a histologic study

A 4-year-old Canadian holstein bull developed the spastic syndrome, an episodic but progressive disorder causing pelvic limb muscular spasms. A post-mortem study, including morphometry of skeletal muscles and teased peripheral nerve fibers of the pelvic limb, revealed mild type II skeletal muscle fiber atrophy and minimal, focal segmental demyelination with remyelination, and axonal degeneration in peripheral nerves. Such alterations are probably incidental or age-associated. Idiopathic muscular cramps is the most probable explanation of the clinical disease and is consistent with the absence of significant morphologic pathologic lesions.     

Distal denervating disease: a degenerative neuropathy of the distal motor axon in dogs

A group of ten dogs affected by an apparently identical denervating disease, is described. There was no breed or age predisposition but females were preferentially affected (70%). The rate of onset of signs was variable from a week to greater than 1 month. Quadriparesis was present to varying severities and in two dogs the head and neck could not be supported. Mastication, swallowing, respiration and bladder function were unimpaired. Pain sensation was normal, tone was usually decreased and the local limb reflexes depressed or absent. Muscle atrophy was often prominent. All cases bar one recovered but another dog was also destroyed. Electrophysiology revealed diffuse spontaneous activity in the muscles and the motor nerve conduction velocities were at the lower end of or just below the normal range. The evoked muscle potentials were reduced in amplitude. Sensory nerve potentials were normal. The pathology showed a degeneration of the distal intramuscular axons with collateral axonal sprouting. The muscle changes were typical of neurogenic atrophy. The disease has been called distal denervating disease (DDD) until the precise aetiology can be determined.     

Familial motor neuron disease in Rottweiler dogs: neuropathologic studies

Two 6-week-old female Rottweiler littermates were evaluated for regurgitation, diminished growth, progressive ataxia, and pelvic limb weakness. Clinical examination indicated a progressive, diffuse, lower motor neuron disorder and megaesophagus. The pups were killed at 6 and 8 weeks of age. Lesions included central chromatolysis and swelling of the perikarya in many large motor neurons in the ventral gray matter of the spinal cord. Some involvement of red, oculomotor, trigeminal motor, and ambiguus nuclei of the brainstem was noted. Ultrastructurally, chromatolytic neurons had excess neurofilaments, and an increase in and enlargement of Golgi complexes. Wallerian-like degeneration was prominent in neuropil of spinal cord and in peripheral nerve. Clinical, histological, and ultrastructural findings were consistent with a progressive motor neuron disease.     

Comparison of the large muscle group widths of the pelvic limb in seven breeds of dogs

Orthopaedic diseases are common in the pelvic limbs of dogs, and reference values for large muscle groups of the pelvic limb may aid in diagnosis such diseases. As such, the objective of this study was to compare the large muscle groups of the pelvic limb in seven breeds of dogs. A total of 126 dogs from different breeds were included, and the widths of the quadriceps, hamstring and gastrocnemius muscles were measured from images of the lateral radiographies. The width of the quadriceps was not different between the breeds, but the widths of the hamstring and gastrocnemius muscles were significantly different between the breeds. The widest hamstring and gastrocnemius muscles were seen in the Rottweilers and the Boxers, respectively. The narrowest hamstring and gastrocnemius muscles were seen in the Belgian Malinois and the Golden retrievers, respectively. All ratios between the measured muscles differed significantly between the breeds. Doberman pinschers and Belgian Malinois had the highest ratio of gastrocnemius width:hamstring width. Doberman pinschers had also the highest ratio of quadriceps width:hamstring width. German shepherds had the highest ratio of gastrocnemius width:quadriceps width. The lowest ratios of quadriceps width:hamstring width were determined in the German shepherds. The ratios of the muscle widths may be used as reference values to assess muscular atrophy or hypertrophy in cases of bilateral or unilateral orthopaedic diseases of the pelvic limbs. Further studies are required to determine the widths and ratios of the large muscle groups of the pelvic limbs in other dog breeds.     

Suspected immune-mediated myositis in horses

BACKGROUND: Although immune-mediated myositis (IMM) is commonly reported in other species, this condition is poorly described in horses. HYPOTHESIS: IMM occurs in horses. ANIMALS: Thirty-seven horses with suspected IMM were included in the study. METHODS: The database of the Neuromuscular Diagnostic Laboratory was reviewed to identify 37 horses with muscle biopsies characterized by lymphocytic infiltrates. A retrospective standardized questionnaire regarding clinical signs and response to treatment was answered by horse owners. RESULTS: Horses with suspected IMM were predominantly of Quarter Horse bloodlines (33/37 horses) and primarily either < or =8 years or > or =17 years of age. Clinical signs included rapid atrophy, particularly of the epaxial and gluteal muscles, depression, and stiffness. Creatine kinase (CK) activity (mean 9746; range 260-139,183 U/L: reference range 119-287 U/L) and aspartate transaminase (AST) activity (mean 2880; range 350-9009 U/L: reference range 138-409 U/L) were high. Exposure to horses with infectious respiratory disease occurred in 39% (9/23) of horses before clinical signs and 47% (9/19) had recurrence of atrophy. Variation in dosage and time elapsed before administration of corticosteroids confounded assessment of treatment efficacy. Macrophages and CD4+ T lymphocytes were the prominent mononuclear cellular infiltrates with lesser numbers of CD8+ cells and small clusters of B lymphocytes in some samples. Myofibers did not stain for equine immunoglobulin G (IgG). CONCLUSIONS AND CLINICAL IMPORTANCE: IMM appears to be a distinct cause of rapid muscle atrophy, particularly in Quarter Horses that may be amenable to treatment with corticosteroids. Diagnosis is best achieved by identifying lymphocytic infiltrates in atrophied muscles.     

Clinical Phenotype of X‐Linked Myotubular Myopathy in Labrador Retriever Puppies

Background

Seven male Labrador Retriever puppies from 3 different litters, born to clinically normal dams and sires, were evaluated for progressive weakness and muscle atrophy. Muscle biopsies identified a congenital myopathy with pathologic features consistent with myotubular myopathy. Further investigations identified a pathogenic mutation in the myotubularin gene, confirming that these puppies had X‐linked myotubular myopathy (XLMTM).

Objective

To review the clinical phenotype, electrodiagnostic and laboratory features of XLMTM in this cohort of Labrador Retrievers.

Results

Male puppies with XLMTM were small and thin compared with their normal littermates. Generalized weakness and muscle atrophy were present by 7 weeks of age in some puppies and evident to most owners by 14 weeks of age. Affected puppies stood with an arched spine and low head carriage, and walked with a short, choppy stride. Muscle atrophy was severe and progressive. Patellar reflexes were absent. Laryngeal and esophageal dysfunction, and weakness of the masticatory muscles occurred in puppies surviving beyond 4 months of age. Serum creatine kinase activity was normal or only mildly increased. EMG findings were nonspecific and included positive sharp waves and fibrillation potentials. Clinical signs progressed rapidly, with most affected puppies unable to walk within 3–4 weeks after clinical signs were first noticed.

Conclusions and Clinical Importance

Although initial clinical signs of XLMTM are similar to the phenotypically milder centronuclear myopathy in Labrador Retrievers, XLMTM is a rapidly progressive and fatal myopathy. Clinicians should be aware of these 2 distinct myopathies with similar clinical presentations in the Labrador retriever breed.     

Recurrent demyelination and remyelination in 37 young Bengal cats with polyneuropathy

Background: With the exception of diabetic neuropathy, polyneuropathy associated with hyperchylomicronemia, and a few inherited polyneuropathies, peripheral neuropathies are poorly characterized in cats. A chronic polyneuropathy is described in a cohort of young Bengal cats. Objective: To characterize the clinical and histopathological features of a chronic‐relapsing peripheral neuropathy in young Bengal cats. Animals: Thirty‐seven young Bengal cats with clinical weakness consistent with peripheral neuropathy. Methods: Bengal cats were included in this study after a diagnosis of polyneuropathy was confirmed by muscle and peripheral nerve biopsy specimens. Pathological changes were characterized at the light and electron microscopic level and by morphometry. Clinical information and long‐term outcome from case records of Bengal cats with histologically confirmed peripheral neuropathy were then assessed. Results: Nerve fiber loss within distal intramuscular nerve branches was a consistent finding in young Bengal cats with polyneuropathy. The most common abnormalities in peripheral nerve biopsies included inappropriately thin myelin sheaths and thinly myelinated fibers surrounded by supernumerary Schwann cell processes, indicative of repeated cycles of demyelination and remyelination. Recovery was common. Response to treatment could not be determined. Conclusions and Clinical Importance: A chronic‐relapsing form of polyneuropathy associated primarily with episodes of demyelination and remyelination was identified in young Bengal cats. The prognosis for recovery is good, although relapses are possible and there can be residual motor deficits.     

Occurrence, clinical features and outcome of canine pancreatitis (80 cases)

Medical records of 80 dogs diagnosed with acute pancreatitis during a 4-year period were evaluated regarding history, breed predilection, clinical signs and additional examination findings. Cases were selected if compatible clinical symptoms, increased serum activity of amylase or lipase and morphologic evidence of pancreatitis by ultrasonography, laparotomy or necropsy were all present. Like in other studies, neutered dogs had an increased risk of developing acute pancreatitis. Although breed predilection was consistent with earlier reports, some notable differences were also observed. Apart from Dachshunds, Poodles, Cocker Spaniels and Fox Terriers, the sled dogs (Laikas, Alaskan Malamutes) also demonstrated a higher risk for pancreatitis according to our results. Concurrent diseases occurred in 56 dogs (70%), diabetes mellitus (n = 29, 36%) being the most common. Clinical signs of acute pancreatitis were similar to those observed in other studies. The study group represented a dog population with severe acute pancreatitis, having a relatively high mortality rate (40%) compared to data of the literature. Breed, age, gender, neutering and body condition had no significant association with the outcome. Hypothermia (p = 0.0413) and metabolic acidosis (p = 0.0063) correlated significantly with poor prognosis and may serve as valuable markers for severity assessment in canine acute pancreatitis.     

Peripheral nerve pathology in two rottweilers with neuronal vacuolation and spinocerebellar degeneration

Neuronal vacuolation and spinocerebellar degeneration in young Rottweiler dogs is a neurodegenerative condition characterized by neuronal vacuolation of several nuclei in the central nervous system and degeneration of the spinal cord white matter. Here, we describe the morphologic and ultrastructural findings in laryngeal muscles and peripheral nerves of a 16-week-old female and a 32-week-old female Rottweiler dog affected by progressive ataxia and tetraparesis associated with laryngeal paralysis. Lesions were characterized by neurogenic muscle atrophy of the intrinsic laryngeal muscles, and a loss of large myelinated fibers in the recurrent laryngeal nerve, accompanied by demyelinating/remyelinating features affecting the small myelinated fibers. No significant changes were detected in the cranial laryngeal, vagus, phrenic, ulnar, or peroneal nerves. These findings were indicative of a selective distal neuropathy of the recurrent laryngeal nerve with early severe axonal degeneration, mainly of the large myelinated fibers.     

Gelatinous transformation of bone marrow in a rabbit

A 9-y-old, spayed female rabbit was presented for evaluation of hypoglycemia and lateral recumbency. The patient was hypothermic and had diffuse muscle wasting; weight loss since a previous visit was also noted. Hematologic abnormalities included progressive nonregenerative anemia and severe heteropenia. Evaluation of a bone marrow aspirate sample revealed active hematopoiesis with abundant pink matrix. The matrix material stained positively with periodic acid–Schiff and alcian blue, and a diagnosis of gelatinous transformation of the bone marrow (GTBM, serous atrophy of fat) was made. Although its precise prevalence remains to be determined, GTBM should be suspected in rabbits with persistent cytopenias following prolonged starvation or gastrointestinal disease.     

Neuronal vacuolation, myelopathy and laryngeal neuropathy in a mixed-breed dog

A bilateral and symmetrical neuronal vacuolation associated with spinal cord white matter degeneration and laryngeal neuropathy was observed in a 12-week-old male mixed-breed dog with a history of progressive pelvic limbs ataxia. On clinical examination, signs included inspiratory stridor, spinal ataxia, tetraparesis, and proprioceptive deficits more severe in the pelvic limbs. Examination of the larynx showed bilateral laryngeal paralysis and electromyography revealed fibrillation potentials restricted to the intrinsic laryngeal muscles. Clinical and pathological findings resembled the syndrome of neuronal vacuolation and spinocerebellar degeneration described in Rottweiler dogs. This is the first report of a similar disorder in a dog different from Rottweiler.