FDG‐PET IN HEALTHY AND EPILEPTIC LAGOTTO ROMAGNOLO DOGS AND CHANGES IN BRAIN GLUCOSE UPTAKE WITH AGE

Regional cerebral metabolism and blood flow can be measured noninvasively with positron emission tomography (PET). 2‐[¹⁸F]fluoro‐2‐deoxy‐D‐glucose (FDG) widely serves as a PET tracer in human patients with epilepsy to identify the seizure focus. The goal of this prospective study was to determine whether juvenile or adult dogs with focal‐onset epilepsy exhibit abnormal cerebral glucose uptake interictally and whether glucose uptake changes with age. We used FDG‐PET to examine six Lagotto Romagnolo dogs with juvenile epilepsy, two dogs with adult‐onset epilepsy, and five control dogs of the same breed at different ages. Three researchers unaware of dog clinical status visually analyzed co‐registered PET and magnetic resonance imaging (MRI) images. Results of the visual PET analyses were compared with electroencephalography (EEG) results. In semiquantitative analysis, relative standard uptake values (SUV) of regions of interest (ROI) drawn to different brain regions were compared between epileptic and control dogs. Visual analysis revealed areas of hypometabolism interictally in five out of six dogs with juvenile epilepsy in the occipital, temporal, and parietal cortex. Changes in EEG occurred in three of these dogs in the same areas where PET showed cortical hypometabolism. Visual analysis showed no abnormalities in cerebral glucose uptake in dogs with adult‐onset epilepsy. Semiquantitative analysis detected no differences between epileptic and control dogs. This result emphasizes the importance of visual analysis in FDG‐PET studies of epileptic dogs. A change in glucose uptake was also detected with age. Glucose uptake values increased between dog ages of 8 and 28 weeks and then remained constant.     

Cerebellar cortical abiotrophy in Lagotto Romagnolo dogs

This case report documents two pathological variations of potentially inherited, cerebellar cortical abiotrophy in two unrelated Lagotto Romagnolo breed dogs. The first dog had an atypical lesion in the cerebellar cortex with depletion of cerebellar granular cell layer and sparing of the Purkinje cell layer. The second case had degenerative changes in both Purkinje and granular cell layers. The clinical picture was similar in both cases presented, although the severity of the signs of cerebellar dysfunction varied.     

Multifocal retinopathy of Great Pyrenees dogs

Forty-four related Great Pyrenees dogs were examined ophthalmoscopically. Focal retinal elevations, multiple gray-tan-pink subretinal patches, and discrete areas of tapetal hyper-reflectivity were seen in 19 dogs, ranging from 13 weeks to 10 years of age. These lesions varied in size from focal spots that were barely visible with the indirect ophthalmoscope to areas that were larger than the optic disc. Complete blood cell counts, serum biochemical profiles, urinalyses, and blood pressure measurements were completed on four affected dogs and all were within normal reference ranges. Photopic and scotopic electroretinography was completed and the a-wave and b-wave amplitudes and latencies were similar for affected and age-matched nonaffected Great Pyrenees and other normal dogs. Electroretinograms that were examined twice during a 3-year period on three affected adult dogs did not reveal significant progressive deterioration of the a or b-wave parameters. Fluorescein angiography was completed on four affected dogs of ages 1 (n = 2), 5, and 6 years. These angiograms were repeated in three of these dogs 1 year later. The blood ocular barrier was intact in these dogs but there was blocked choroidal fluorescence. Postmortem examination, light microscopy, scanning and transmission electron microscopy were performed on three affected puppies and two affected adult dogs. These examinations revealed that the lesions in the puppies were limited to bilateral multiple areas of retinal pigment epithelial vacuolation, hypertrophy, and apparent separation from Bruch's membrane, and multiple serous retinal detachments. The affected adult dogs had focal retinal degeneration and retinal pigment epithelial hypertrophy, hyperplasia and pigmentation. Pedigree analysis and test mating confirm that this condition is inherited, probably as an autosomal recessive trait. This condition develops at approximately 13 weeks of age and the focal areas of retinal detachment and retinal pigment epithelial vacuolation progress to permanent and stable focal areas of retinal degeneration, and retinal pigment epithelial hypertrophy and pigmentation.     

Behavioral Abnormalities in Lagotto Romagnolo Dogs with a History of Benign Familial Juvenile Epilepsy: A Long‐Term Follow‐Up Study

Background

Lagotto Romagnolo (LR) dogs with benign juvenile epilepsy syndrome often experience spontaneous remission of seizures. The long‐term outcome in these dogs currently is unknown. In humans, behavioral and psychiatric comorbidities have been reported in pediatric and adult‐onset epilepsies.

Hypothesis/Objectives

The objectives of this study were to investigate possible neurobehavioral comorbidities in LR with a history of benign familial juvenile epilepsy (BFJE) and to assess the occurrence of seizures after the remission of seizures in puppyhood.

Animals

A total of 25 LR with a history of BFJE and 91 control dogs of the same breed.

Methods

Owners of the LR dogs in the BFJE and control groups completed an online questionnaire about each dog''s activity, impulsivity, and inattention. Principal component analysis (PCA) served to extract behavioral factors from the data. We then compared the scores of these factors between the 2 groups in a retrospective case–control study. We also interviewed all dog owners in the BFJE group by telephone to inquire specifically about possible seizures or other neurological problems after remission of seizures as a puppy.

Results

Lagotto Romagnolo dogs with BFJE showed significantly higher scores on the factors Inattention and Excitability/Impulsivity than did the control group (P = .003; P = .021, respectively). Only 1 of the 25 BFJE LR exhibited seizures after remission of epilepsy in puppyhood.

Conclusions and Clinical Importance

Although the long‐term seizure outcome in BFJE LR seems to be good, the dogs exhibit behavioral abnormalities resembling attention deficit hyperactivity disorder (ADHD) in humans, thus suggesting neurobehavioral comorbidities with epilepsy.     

Postattenuation neurologic signs after surgical attenuation of congenital portosystemic shunts in dogs: A review

The development of postattenuation neurologic signs (PANS) is a poorly understood and potentially devastating complication after surgical attenuation of congenital portosystemic shunts in dogs. Postattenuation neurologic signs include seizures but also more subtle neurologic signs such as depression, behavioral changes, tremors, and twitching. They most commonly occur within 7 days postoperatively and are typically unrelated to hyperammonemia, hypoglycemia, or electrolyte disturbances. This narrative review summarizes the findings of 50 publications from 1988-2020 that report occurrence of PANS. While most published reports included only dogs affected by postattenuation seizures (PAS), others included dogs with any form of PANS. Overall, PANS (including PAS) affected 1.6%-27.3% of dogs, whereas incidence of PAS ranged from 0%-18.2%. The etiology of PANS remains unknown; however, several theories have been proposed. Risk factors include preoperative hepatic encephalopathy, increasing age, and possibly certain breeds and extrahepatic shunt morphology. There is increasing evidence that prophylactic antiepileptic drugs do not prevent PANS. Treatment is centered around controlling neurologic signs with antiepileptic drugs and providing supportive intensive care. The 30-day survival rate in studies that included a minimum of four dogs affected by PANS was 0%-100% (median, 50.0%) and 0%-75.0% (median, 37.5%) for those with PAS. Mortality associated with PANS was typically related to occurrence of generalized seizure activity. Prognostic factors positively associated with short-term survival included having a history of preoperative seizures and development of focal seizures only. If affected dogs survived to discharge, survival for several years was possible, and the majority of neurologic signs manifested as part of the phenomenon of PANS appeared to resolve.     

Dilated cardiomyopathy in juvenile Portuguese Water Dogs

Dilated cardiomyopathy recently has been recognized in juvenile Portuguese Water Dogs. The purpose of this study was to evaluate unaffected and affected puppies by physical examination, electrocardiogram (ECG), echocardiogram, specific biochemical assays, and ultrastructure to document disease progression and to develop a method of early detection. Results of segregation analysis were consistent with autosomal recessive inheritance. Of 124 puppies evaluated clinically and echocardiographically, 10 were affected. No significant differences were found between unaffected and affected puppies for blood and myocardial carnitine or taurine concentrations, serum chemical variables, results of ophthalmological examinations, ECGs, or measurement of urine metabolites. Ultrastructural examination of myocardium from affected dogs revealed myofibrillar atrophy and small regions of myofibrillar degeneration, most prominently at the region of the intercalated discs. Only echocardiography allowed detection of affected puppies before clinical signs became evident. Echocardiography revealed a significant difference in the shortening fraction, E point to septal separation, and the end systolic and diastolic left ventricular internal diameters. Affected puppies were detected 1-4 weeks before the development of acute congestive heart failure.     

Pulverulent nuclear cataract in the Norwegian buhund

Pulverulent cataracts were diagnosed in 52 of 102 Norwegian buhunds, with both sexes being equally affected. All the dogs were otherwise considered clinically healthy. Initial lens changes were visible from six and a half weeks of age as small dots parallel to the suture lines behind the nucleus. Gradually, the opacities along the suture lines became more opaque until, by the age of four to five and a half years, they had progressed to involve the fetal nucleus which then resembled a ball of candy floss. The outer part, the adult nucleus, usually remained clear, and the cortex was not involved. The ages of the dogs in the present study ranged from three and a half weeks to 12 years at first examination. An outcross of an affected bitch to a mixed-breed dog resulted in three puppies, of which two were affected. The retinas could be evaluated in all the examined dogs, and revealed no abnormalities except for one case of focal retinopathy. In addition to the pulverulent cataracts, 10 cases of cortical cataracts were diagnosed, three of which also had pulverulent cataracts. Based on the pedigrees, an autosomal dominant mode of inheritance with a high degree of penetrance is suggested for pulverulent cataract.     

Quantitative urinalysis in healthy Beagle puppies from 9 to 27 weeks of age

To evaluate indices of renal function in healthy, growing Beagle puppies from 9 to 27 weeks of age and to determine whether indices change with age during this period. Animals-6 healthy Beagle puppies. PROCEDURE: Urine collections were performed at 2-week intervals in puppies 9 to 27 weeks old. Daily excretion of urinary creatinine, protein, sodium, potassium, chloride, phosphorus, and calcium were determined, as were quantitative urinalyses including endogenous creatinine clearance, urine protein-to-creatinine ratios (UPr/C), and fractional clearances of sodium (FNa), potassium (FK), chloride (FCI), calcium (FCa), and phosphorus (FP). RESULTS: Significant differences among age groups were detected for endogenous creatinine clearance, and daily urinary protein, potassium, calcium, and phosphorus excretion. Significant differences also existed among age groups for UPr/C, FNa, FK, FCI and FP. Age-related effects fit a linear regression model for FNa, UPr/C, daily phosphorus excretion, and daily protein excretion. Quadratic regression models were judged most appropriate for endogenous creatinine clearance, FK, daily chloride excretion, and daily potassium excretion. Endogenous creatinine clearance measurements higher than adult reference ranges were observed from 9 to 21 weeks of age. The FNa, FK, FCI, FCa, and FP were slightly higher than those reported for adult dogs. CONCLUSIONS AND CLINICAL RELEVANCE: Selected results of quantitative urinalyses in healthy 9- to 27-week-old Beagle puppies differ with age and differ from those measured in adult dogs. Diagnostic measurements performed in puppies of this age range should be compared with age-matched results when possible.     

Canine wobbler syndrome: A study of the Dobermann pinscher in New Zealand

The aim of this study was to investigate the prevalence of wobbler disease within a Dobermann pinscher population from three geographical locations in New Zealand. The study population consisted of 138 adults (aged 1–13 years) and 32 puppies (aged 6 weeks to 11 months). Data collected for each dog included age, sex, geographic location, if a choker chain was used or not and, in adults, the following body measurements: dimensions of head length, head circumference, width between shoulders, neck length, height at withers and withers to rump length. In addition, lateral radiographs were taken of the caudal cervical vertebrae of each dog and the radiological abnormalities associated with wobbler disease scored, so that each dog could be assigned to one of three radiological groupings. Based upon a neurological examination, each animal was also placed into one of three neurological groupings. The relationship between radiological and neurological groupings and the independent variables was initially compared using a univariate and subsequently a multivariate analysis.

It was found that 48.8% of the dogs investigated had some abnormal radiological sign associated with wobbler disease, and 32.0% of them showed neurological signs. Dogs with radiological signs of the disease were 5.56 times more likely to have neurological signs. Statistical analysis of the data indicated that more severe radiological and neurological abnormalities occurred in the older dogs. In addition, dogs located in Hawke's Bay region had less chance of showing radiological changes than dogs from the other two regions, Hamilton and Wellington. Twelve of the 32 puppies were examined for radiological and neurological changes over the first year of their life. No abnormalities were detected in puppies under 12 weeks of age, but 28% (n=9) of the 32 puppies over 3 months of age did show some radiological changes. Only 9%(n=3) of puppies showed any neurological signs. Although several pedigree lines were investigated, the lineage data were incomplete, and therefore there was no conclusive evidence that wobbler disease was an inherited trait.

This study showed that, although the radiological signs of wobbler disease were present throughout a wide age range, the associated neurological changes tended to appear at a later age. In both instances, the severity of these changes increased with age.     

Dermatomyositis in five Shetland sheepdogs in the United Kingdom

Five cases of dermatomyositis in four Shetland sheepdog puppies and one adult bitch are described. The dogs all had well-defined patches of scaling, crusting and alopecia over the muzzle, periorbital skin and distal limbs, and the tail, perineum and pinnae were affected in some of them. The affected puppies were all sired by the same stud dog. The affected adult bitch was unrelated to the puppies. Three of the four dogs tested had high serum creatine kinase concentrations and electromyographic abnormalities were detected in three of the four dogs tested. The histological changes observed in the skin of four of the dogs strongly supported the diagnosis of dermatomyositis, and in the fifth dog they were compatible with this diagnosis. Two of the puppies were euthanised shortly after being diagnosed. In the other two puppies and the adult the disease remains stable and non-progressive 15 to 18 months after diagnosis. The sire of the four affected puppies has been used extensively because it was considered to be genetically clear of collie eye anomaly.     

Canine wobbler syndrome: a study of the Dobermann pinscher in New Zealand

The aim of this study was to investigate the prevalence of wobbler disease within a Dobermann pinscher population from three geographical locations in New Zealand. The study population consisted of 138 adults (aged 1-13 years) and 32 puppies (aged 6 weeks to 11 months). Data collected for each dog included age, sex, geographic location, if a choker chain was used or not and, in adults, the following body measurements: dimensions of head length, head circumference, width between shoulders, neck length, height at withers and withers to rump length. In addition, lateral radiographs were taken of the caudal cervical vertebrae of each dog and the radiological abnormalities associated with wobbler disease scored, so that each dog could be assigned to one of three radiological groupings. Based upon a neurological examination, each animal was also placed into one of three neurological groupings. The relationship between radiological and neurological groupings and the independent variables was initially compared using a univariate and subsequently a multivariate analysis. It was found that 48.8% of the dogs investigated had some abnormal radiological sign associated with wobbler disease, and 32.0% of them showed neurological signs. Dogs with radiological signs of the disease were 5.56 times more likely to have neurological signs. Statistical analysis of the data indicated that more severe radiological and neurological abnormalities occurred in the older dogs. In addition, dogs located in Hawke's Bay region had less chance of showing radiological changes than dogs from the other two regions, Hamilton and Wellington. Twelve of the 32 puppies were examined for radiological and neurological changes over the first year of their life. No abnormalities were detected in puppies under 12 weeks of age, but 28% (n=9) of the 32 puppies over 3 months of age did show some radiological changes. Only 9% (n=3) of puppies showed any neurological signs. Although several pedigree lines were investigated, the lineage data were incomplete, and therefore there was no conclusive evidence that wobbler disease was an inherited trait. This study showed that, although the radiological signs of wobbler disease were present throughout a wide age range, the associated neurological changes tended to appear at a later age. In both instances, the severity of these changes increased with age.     

Changes in factor VIII activity and von Willebrand factor antigen concentration with age in dogs.

The factor VIII activity of 38 German shepherd puppies, 6-12 weeks old, submitted for diagnosis of haemophilia A was measured. Eight of these puppies had values higher than would be expected for haemophiliacs, but less than the reference range for adult dogs. A further sequential study of 21 puppies (6-26 weeks of age) indicated that the factor VIII activity of puppies is generally less than that of adult dogs until about 14 weeks of age. Changes in the concentration of von Willebrand factor antigen in the puppies were irregular. These variations are probably not sufficient to interfere with accurate diagnosis of haemophilia A in most affected young dogs, but may interfere with the detection of heterozygotes in young bitches.     

Systemic necrotizing vasculitis in nine young beagles.

A systemic necrotizing vasculitis of unknown etiopathogenesis may be termed juvenile polyarteritis syndrome (JPS). The syndrome has been recognized primarily in young Beagles used for toxicologic studies. We studied 9 young Beagles with JPS. Affected dogs had fever (40 to 41.5 C), anorexia, and signs of pain in the cervical area. They had a characteristic hunched stance, and were unwilling to move. Laboratory abnormalities in all dogs included nonregenerative anemia, hypoalbuminemia, and leukocytosis characterized by a mature neutrophilia. Analysis of CSF revealed a moderate to severe neutrophilic pleocytosis and a mildly high protein concentration in most dogs. Signs of disease resolved rapidly with high doses (2.2 mg/kg of body weight, PO) of prednisone. If untreated, clinical signs and laboratory abnormalities had a remitting and relapsing course in most dogs. Findings at necropsy included necrotizing arteritis with fibrinoid necrosis, periarteritis, thrombosis, and intimal proliferation that most frequently affected small- to medium-sized vessels in the cervical spinal cord, mediastinum, and heart. An immune-mediated pathogenesis for this disease is suspected.     

Uncommon acute neurologic presentation of canine distemper in 4 adult dogs

Four uncommon cases of canine distemper (CD) were diagnosed in vaccinated adult dogs. All dogs had acute onset of neurologic signs, including seizures, abnormal mentation, ataxia, and proprioceptive deficits. Polymerase chain reaction for CD virus was positive on cerebrospinal fluid in 2 cases. Due to rapid deterioration the dogs were euthanized and CD was confirmed by postmortem examination.     

Bilateral cavernous sinus syndrome in dogs: 6 cases (1999-2004)

OBJECTIVE: To determine clinical features, diagnostic imaging abnormalities, underlying disease, disease progression, and outcome in dogs with bilateral cavernous sinus syndrome. DESIGN: Retrospective study. ANIMALS: 6 dogs. PROCEDURE: Dogs were included if clinical signs consistent with bilateral cavernous sinus syndrome (i.e., deficits of the third, fourth, and sixth cranial nerves and at least 1 of the first 2 branches of the fifth cranial nerve) were present and a lesion of the cavernous sinus was identified by means of diagnostic imaging or postmortem examination. RESULTS: 5 dogs were evaluated because of problems referable to abnormal ocular motility or pupillomotor dysfunction, and 1 dog was evaluated because of partial motor seizures involving the face and bilateral mydriasis. Four dogs had neurologic signs referable to an extrasinusoidal lesion at the time of initial examination, and the remaining 2 dogs eventually developed extrasinusoidal signs. Besides neuroanatomic location, the only consistent neuroimaging feature was variably intense, heterogeneous enhancement of cavernous sinus lesions. Neoplasia was histologically confirmed as the underlying cause in 5 of the dogs and was suspected in the remaining dog. Median survival time for the 4 dogs that were treated was 199 days (range, 16 to 392 days). CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that bilateral cavernous sinus syndrome is rare in dogs but should be suspected in dogs with compatible clinical signs. Affected dogs have a poor prognosis, and dogs with clinical signs of bilateral cavernous sinus syndrome should be systematically evaluated for neoplastic disease.     

RESULTS OF MYELOGRAPHY IN SEVEN DOGS WITH MYELOMALACIA

Myelomalacia is a hemorrhagic infarction of the spinal cord that can occur as a sequel to acute spinal cord injury. Myelomalacia may be focal or diffuse; the diffuse form is typically associated with cranial migration of neurologic signs ("ascending syndrome") and is often fatal. In a retrospective study of seven affected dogs, diffuse myelomalacia was associated with intervertebral disc extrusion in five dogs, focal myelomalacia was associated with fibrocartilagenous embolus in one dog, and had no apparent cause in one dog. The myelographic signs included a variable degree of contrast medium infiltration into the spinal cord in six dogs (86%) and/or spinal cord swelling in six dogs (86%). In one dog with focal myelomalacia, the only myelographic sign was spinal cord swelling.     

Pituitary apoplexy-like disease in 4 dogs

BACKGROUND: Pituitary apoplexy in humans is a clinical syndrome resulting from sudden infarction, hemorrhage, or both in a normal or an adenomatous pituitary gland. OBJECTIVE: Describe a clinical syndrome in dogs similar to pituitary apoplexy in humans. ANIMALS: Four dogs exhibiting a sudden onset of neurologic signs. METHODS: A retrospective study was used, including clinical examination, computed tomography (CT), postmortem examination, and histopathology of the brain. Pituitary tissue from 3 of the dogs was subjected to immunocytochemistry. RESULTS: Four dogs (2 Mongrels, 1 Bordeaux Dog, and 1 Cocker Spaniel; median age, 11 years; median body weight, 20.5 kg) presented with acute neurologic signs including depression (n = 3), behavioral changes (n = 1), vision loss (n = 1), seizures (n = 1), and collapse (n = 1). CT disclosed suprasellar infarction, hemorrhage, or both associated with a pituitary macroadenoma in 3 dogs and a frank hemorrhage in a nonadenomatous pituitary gland in 1 dog. CT findings were correlated with postmortem findings, and pituitary apoplexy was confirmed by histopathology and immunocytochemistry of the pituitary tissue. CONCLUSIONS AND CLINICAL IMPORTANCE: This study provides histopathologic evidence of pituitary apoplexy in dogs. The results are relevant for future diagnosis and treatment of pituitary disease in dogs.     

Experimental infection of equine herpesvirus 9 in dogs

Equine herpesvirus 9 (EHV-9), a new neurotropic equine herpesvirus, was inoculated intranasally at 107 plaque-forming units in five dogs to assess its pathogenicity. Dogs showed weight loss, pyrexia, anorexia, and neurologic signs on the fourth day. The EHV-9 virus was recovered from the examined brains. Histologically, dogs had a fulminant nonsuppurative encephalitis characterized by severe neuronal degeneration and loss, with intranuclear inclusions, slight glial reactions, perivascular cuffing, and multifocal hemorrhage. The olfactory bulb and the frontal and temporal lobes were predominantly affected. Immunohistochemistry revealed reactivity for EHV-9 antigen in neurons. All dogs had mild bronchopneumonia and various degrees of lymphoid necrosis. These findings indicate that dogs are fully susceptible to EHV-9 and that EHV-9 can cause fulminant encephalitis with high mortality in dogs, as in gazelles and goats.     

Diagnostic imaging findings and endocrine test results in dogs with pituitary-dependent hyperadrenocorticism that did or did not have neurologic abnormalities: 157 cases (1989-2005)

OBJECTIVE: To compare imaging findings in dogs with pituitary-dependent hyperadrenocorticism (PDH) that did or did not have neurologic abnormalities. Design-Retrospective case series. ANIMALS: 157 dogs with PDH that did (n = 73) or did not (84) have neurologic abnormalities. PROCEDURES: Medical records were reviewed for the presence and nature of clinical signs of CNS disease, and computed tomographic and magnetic resonance images were reviewed for evidence of a pituitary tumor. RESULTS: 60 of the 84 (71%) dogs without neurologic abnormalities and 48 of the 73 (66%) dogs with neurologic abnormalities had a detectable pituitary tumor. However, 17 of the 84 (20%) dogs without neurologic abnormalities had a pituitary macrotumor (ie, a tumor > or = 10 mm in height), and 41 of the 73 (56%) dogs with neurologic abnormalities did not have a detectable pituitary tumor or had a pituitary microtumor. Vague signs of CNS dysfunction (ie, lethargy, inappetence, and mental dullness) were more specific for detection of pituitary macrotumors than were CNS-specific signs (ie, seizure or blindness). CONCLUSIONS AND CLINICAL RELEVANCE: Results suggested that there was no apparent relationship between a pituitary tumor and development of neurologic abnormalities in dogs with PDH. In addition, neurologic abnormalities in dogs with pituitary macrotumors were often vague (ie, lethargy, inappetence, and mental dullness).     

Spongy Degeneration with Cerebellar Ataxia in Malinois Puppies: A Hereditary Autosomal Recessive Disorder?

Background: There is a high incidence of hereditary degenerative diseases of the central nervous system in purebred dogs. Hypothesis: Cerebellar ataxia in Malinois puppies, caused by degenerative changes that predominate in cerebellar nuclei and the granular cell layer, is a hereditary disorder that is distinct from cerebellar cortical abiotrophies. Animals: Thirteen Malinois puppies with cerebellar ataxia. Methods: Retrospective study. Records of Malinois puppies with spongy degeneration of the cerebellar nuclei were analyzed including clinical signs, histopathological changes, and pedigree data. Results: Signs of cerebellar dysfunction were observed in puppies of both sexes from 5 different litters (1995–2009) of phenotypically normal parents. Clinical signs started before the age of 2 months and resulted in euthanasia of all puppies by the age of 13 weeks. Histopathology disclosed marked bilateral spongy degeneration of the cerebellar nuclei and vacuoles in the granular cell layer and foliate white matter of the cerebellum. In some puppies, discrete vacuoles in gray and white matter were present in other parts of the brain. Furthermore, spheroids and dilated myelin sheaths were observed. Pedigree data and segregation frequency support an autosomal recessive hereditary disorder. Conclusions and Clinical Importance: Malinois suffer from a hereditary spongiform degeneration that predominates in the cerebellum and causes an early onset of clinical signs with unfavorable prognosis. Future efforts should increase awareness among veterinarians and breeders and aim to identify underlying metabolic mechanisms and the affected genes.