Lafora-like inclusion bodies in the CNS of aged dogs (original) (raw)
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Topographic distribution pattern of Lafora-like bodies in the spinal cord of some animals
Acta Neuropathologica, 1980
The topographic distribution pattern and morphological features of Lafora-like bodies in the spinal cord of the dog, cat, fox, and baboon were examined by light and electron microscopy. The caudal lumbar and the coccygeal parts of the spinal cords were the predilection sites for the bodies in all animals and were very prominent in the ventral columns and intermediate substance. The bodies mainly composed of branching filaments were preferentially located in neuronal processes and rarely in astrocytes. The histochemical characteristics of the bodies were identical in all animals and consisted mainly of polyglucosan.
Lafora Disease: Report of a Rare Entity
Cureus
Lafora disease is a rare, genetic, glycogen metabolism disorder inherited as autosomal recessive characterized by the presence of inclusion bodies, known as Lafora bodies, within the cytoplasm of the cells in the heart, liver, muscle, and skin. Lafora disease presents as a neurodegenerative disorder that causes impairment in the development of cerebral cortical neurons. We present here a case of Lafora disease that presented with progressive myoclonus epilepsy (PME) and investigated at our center. She was diagnosed to have Lafora disease with typical histological findings on skin biopsy and was found to be positive for the pathogenic mutation on genetic testing.
Revista MVZ Córdoba, 2021
Objetive. To determine the casuistry of neurological diseases, as well as the age, race and affected neuroanatomical sites. Materials and methods. A retrospective study of the clinical records of canine patients attended was carried out at the Veterinary Hospital of the Faculty of Veterinary Medicine and Zootechnics of the University Universidad de San Carlos de Guatemala in 2017. The data were classified according to the VitaminD mnemonic rule. The variables sex, race, age, and neuroanatomical localization of lesions were included. The data were summarized using descriptive statistics through frequency distribution tables. Results. From 1127 case records, the prevalence of neurological diseases was 8.43%. The frequency was higher in males than in females (53.68% vs 46.32%). The most affected age group ranges from zero to seven years. Regarding breeds, a higher frequency was observed in canines without defined breed (25.53%) followed by French Poodle dogs (20.21%). The pathologies found were vascular (1.05%), inflammatory/infectious (25.26%), traumatic (13.68%), metabolic (8.42%), idiopathic (6.32%), neoplastic (1.05%) and degenerative (44.21%). The most common neurolocalization was at the CNS level (86.32%). Conclusions. According to the findings of this study, it was determined that degenerative diseases represented most of the neurological casuistry. The highest frequency of cases occurred in young canine patients, males of mixed breeds. The most frequent neuroanatomical site of lesions was in the spinal cord at the thoracolumbar level.
Veterinarski Arhiv, 2016
Marked lateral ventricular enlargement associated with atrophic cerebral cortex is described in three dogs. In case 1 (irish terrier) and case 2 (german shepherd), hydranencephaly was diagnosed and it was characterized by complete loss of cerebral hemispheres leaving only about 2 mm of brain tissue that encircled fluid filled sacs. Clinically, irish terrier showed only sleepines and retroflexion of the head during sleeping, while german shepherd showed no neurological or any other symptoms. In the third case (english setter), internal hydrocephalus probably secondary to the severe periventricular lesions characterized by neutrophilic/mononuclear cell infiltration was found. This lesion was diagnosed as hydrocephalus associated with periventricular encephalitis and this dog showed more severe neurological symptoms such as somnolence, opisthotonus, rigor of muscles due to seizures, poor eye-sight, stager, ataxia, and unresponsivness to vocalisation. In all cases, no infectious agent o...
Neuronal intranuclear inclusion disease in a horse
Acta Neuropathologica, 2005
Neuronal intranuclear inclusion disease (NIID) is reported in a 16-year-old Pure Spanish breed female horse suffering from progressive ataxia and motor deficiencies. The neuropathological study revealed NIIs throughout the central nervous system, although mainly in the brain stem and spinal cord. This distribution did not correlate with neuron loss, which was marked in the hippocampus and moderate in the neocortex, particularly in the occipital cortex. As in humans, NIIs in the horse were hyaline autofluorescent inclusions composed of non-membrane-bound aggregates of filaments and fine granules. NIIs were stained with anti-ubiquitin and anti-clusterin antibodies. In addition, NIIs were stained with antibodies raised against subunits of the 19S and PA28, but not of the 20S, components of the proteasome. These observations indicate similarities between NIID in humans and horses, and suggest that clusterin and abnormal ubiquitin-proteasomal expression participate in NII formation.
Primary central white matter degeneration in old dogs
Acta Neuropathologica, 1993
Degeneration of the central white matter is described in old dogs. The presence of ubiquitin-immunoreactive free granules and intracytoplasmic globules in glial cells and macrophages, together with galacerebroside-immunoreactive precipitates and lipofusion storage, point to the likelihood of a primary myelin degeneration with deposits of non-degraded ubiquitin-protein conjugates and complex galactolipids.
Acta Neuropathologica, 2005
Abnormal neuronal cytoplasmic inclusions (NCIs) containing aggregates of alpha-internexin and the neurofilament (NF) subunits, NF-H, NF-M, and NF-L, are the signature lesions of neuronal intermediate filament (IF) inclusion disease (NIFID). The disease has a clinically heterogeneous phenotype, including frontotemporal dementia, pyramidal and extrapyramidal signs presenting at a young age. NCIs are variably ubiquitinated and about half of cases also have neuronal intranuclear inclusions (NIIs), which are also ubiquitinated. NIIs have been described in polyglutamine-repeat expansion diseases, where they are strongly ubiquitin immunoreactive. The fine structure of NIIs of NIFID has not previously been described. Therefore, to determine the ultrastructure of NIIs, immunoelectron microscopy was undertaken on NIFID cases and normal aged control brains. Our results indicate that the NIIs of NIFID are strongly ubiquitin immunoreactive. However, unlike NCIs which contain ubiquitin, alpha-internexin and NF epitopes, NIIs contain neither epitopes of alpha-internexin nor NF subunits. Neither NIIs nor NCIs were recognised by antibodies to expanded polyglutamine repeats. The NII of NIFID lacks a limiting membrane and contains straight filaments of 20 nm mean width (range 11-35 nm), while NCIs contain filaments with a mean width of 10 nm (range 5-18 nm; t-test, P<0.001). Biochemistry revealed no differences in neuronal IF protein mobilities between NIFID and normal brain tissue. Therefore, NIIs of NIFID contain filaments morphologically and immunologically distinct from those of NCIs, and both types of inclusion lack expanded polyglutamine tracts of the triplet-repeat expansion diseases. These observations indicate that abnormal protein aggregation follows separate pathways in different neuronal compartments of NIFID.