Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A severe atrophy of the cerebellum was observed in a 7-month-old male Italian hound with a history of progressive ataxia and head tremor from the age of 3 months. On clinical examination, signs included severe hypermetric gait, head tremors and proprioception deficits in all limbs. At necropsy, a pronounced symmetrical reduction in size of the cerebellum was the only gross lesion observed. Histological examination of the cerebellum revealed marked thinning of the granular and molecular layers with almost complete loss of granule cells. Purkinje cells had normal morphology and distribution. These findings differ from those of previous reports of cerebellar cortical abiotrophy in dogs, which were mainly characterized by prominent Purkinje cell degeneration and loss.
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PMID:Cerebellar granuloprival degeneration in an Italian hound. 1254 31

Cerebellar abiotrophy, a premature degeneration of cerebellar neurons, has been described in most domestic animals. Affected animals typically present with progressive neurologic signs after a variable period of postnatal normalcy. This report describes the clinical, histopathologic, and immunohistochemical (IHC) findings of cerebellar abiotrophy in an alpaca. The alpaca developed intention tremors, hypermetria, and a wide-based stance at 1.5 years of age. Histologic lesions, confined to the cerebellar vermis, included marked absence of Purkinje cells, decreased granule cells, narrowing of the molecular layer, and thinning of white matter tracts consistent with abiotrophy. Increased cell processes in the molecular layer immunolabeled for glial fibrillary acidic protein, whereas immunoreactivity for neurofilament was reduced in the molecular layer and cerebellar folia white matter. To the investigators' knowledge, this is the first report of cerebellar abiotrophy in a camelid and the first documentation of IHC findings associated with this condition.
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PMID:Cerebellar abiotrophy in an alpaca (Lama pacos). 1960 11

A pair of rabbits gave birth to a set of littermates (F1) with symptoms of early-onset ataxia. Microscopic examination revealed cerebellar degenerative disease in 5 of 6 littermates. Light microscopy was used to compare the thickness of each cerebellar layer in affected animals in contrast to a normal control. Affected animals showed narrowing of the molecular layer of the vermis, reduced density of Purkinje cell dendrites and irregular thickness in their branchlets, and reduced density of granular cells and scattered pyknotic cells in the granular layer. Pyknotic cells were apoptotic granular cells, confirmed by positive staining using the TUNEL method. Electron microscopy confirmed the thinning of the molecular layer seen by light microscopy and also showed a reduced number of parallel fibers, which indicate granular cells axons, and a reduced number of synaptic junctions between Purkinje and granular cells. Purkinje cells had electron-dense, irregularly shaped cytoplasm with irregularly shaped nuclei, and some of these cells had a central chromatolysis-like region. These findings support a diagnosis of cerebellar cortical abiotrophy, a hereditary condition that causes nerve function impairment leading to early-onset progressive degeneration of the cerebellar cortex.
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PMID:Hereditary cerebellar degenerative disease (cerebellar cortical abiotrophy) in rabbits. 2164 43

Cerebellar abiotrophies, also known as cerebellar ataxias, are characterized by premature post-natal degeneration of cerebellar neurons. This report describes the clinical, magnetic resonance imaging (MRI), gross, histopathological and immunohistochemical features of a novel inherited cerebellar abiotrophy in a cohort of three closely related mixed-breed goats (Capra aegagrus hircus) in the southeastern USA. The animals all presented with early juvenile-onset ataxia, hypermetria, wide-based stance, head tremors and nystagmus. On MRI and at gross examination, there was moderate thinning of the cerebellar vermis and sharpening of the folia. Histologically, the vermis, paravermis and flocculonodular lobe had moderate to severe segmental loss of Purkinje cells with sparing of the hemispheres and secondary loss of granule cells and astrogliosis. Heritable cerebellar ataxias have been reported in many domestic animal species, but not, to the authors' knowledge, as a heritable condition in goats.
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PMID:A Novel Inherited Cerebellar Abiotrophy in a Cohort of Related Goats. 2618 6