Globoid Cell Leucodystrophy in Polled Dorset Sheep

1980 ◽  
Vol 17 (4) ◽  
pp. 399-405 ◽  
Author(s):  
D. H. Pritchard ◽  
D. V. Napthine ◽  
A. J. Sinclair
Keyword(s):  
White Matter ◽  
Hind Limb ◽  
Periodic Acid ◽  
Clinical Signs ◽  
Periodic Acid Schiff ◽  
Histologic Changes ◽  
The Brain ◽  
Globoid Cell Leucodystrophy ◽  
Globoid Cell ◽  
Globoid Cells

Globoid cell leucodystrophy (Krabbe's disease) was diagnosed in two Polled Dorset sheep from a stud farm. Clinical signs were hind limb incoordination progressing to tetraplegia. Histologic changes in white matter of the brain were myelin destruction, loss of oligodendroglia, astrogliosis and accumulation of distinctive periodic acid-Schiff (PAS)-positive globoid cells. The activities of galactocerebroside β-galactosidase, the lysosomal enzyme specifically deficient in globoid leucodystrophy, and of three other glycosidase enzymes were compared in brain tissue of one affected and six unaffected sheep. Activities of the three nonspecific glycosidases were similar in all seven brains. Galactocerebrosidase activity was similar in the six control sheep, but in the affected brain was less than 6% of the control mean.

Globoid Cell-like Leukodystrophy in a Domestic Longhaired Cat

2002 ◽  
Vol 39 (4) ◽  
pp. 494-496 ◽  
Author(s):  
C. J. Sigurdson ◽  
R. J. Basaraba ◽  
E. M. Mazzaferro ◽  
D. H. Gould
Keyword(s):  
Mononuclear Cells ◽  
Periodic Acid ◽  
Clinical Signs ◽  
Krabbe Disease ◽  
Periodic Acid Schiff ◽  
Nervous Systems ◽  
Central Nervous Systems ◽  
Mental Regression ◽  
Globoid Cell ◽  
Globoid Cells

Globoid cell leukodystrophy (GLD; Krabbe disease), is a rare heritable metabolic disorder in humans, dogs, mutant twitcher mice, and rhesus monkeys that is caused by a deficiency in the lysosomal enzyme galactocerebrosidase (GALC). GALC deficiency results in the accumulation of psychosine, which is toxic to oligodendrocytes and Schwann cells of the central and peripheral nervous systems. Clinical signs include hypotonia, mental regression, and death by 2 years of age in most human patients. Here we describe a domestic longhaired kitten with rapidly progressive neurologic disease and brain and spinal cord lesions characteristic of GLD. Pathologic hallmarks of the disease reflect the loss of oligodendrocytes and include myelin loss, gliosis, and the perivascular accumulation of large mononuclear cells with fine cytoplasmic vacuoles (globoid cells) in the peripheral and central nervous systems. Globoid cells were CD68 and ferritin positive, confirming their monocytic origin, and cytoplasmic contents were nonmetachromatic and periodic acid-Schiff positive.

scholarly journals Clinical and MRI findings in two unrelated cats with globoid cell leucodystrophy

2020 ◽  
Vol 8 (2) ◽  
pp. e001129
Author(s):  
Alessia Silvia Colverde ◽  
Teresa Gagliardo ◽  
Cristian Falzone ◽  
Gualtiero Gandini
Keyword(s):  
Periodic Acid ◽  
Mri Findings ◽  
Periodic Acid Schiff ◽  
Flexor Reflex ◽  
Neurological Signs ◽  
Brain And Spinal Cord ◽  
The Brain ◽  
Unrelated Male ◽  
Globoid Cell Leucodystrophy ◽  
Globoid Cell

Two unrelated male cats, both five months old, were referred for progressive neurological signs characterised by intentional tremors, paraplegia with absence of nociception in the pelvic limbs, weakness, dysmetria with reduced flexor reflex in the thoracic limbs, and bilaterally reduced menace response. MRI, performed by a 0.25 T Esaote Vet Grande, showed diffuse and irregular intramedullary T2 weighted hyperintensity between T3 and L3; these lesions were isointense on T1 weighted images without contrast uptake. In one patient MRI of the brain was obtained and mild cerebellar volume reduction was found. Histological examination of the brain and spinal cord demonstrated myelin loss and perivascular accumulation of periodic acid-Schiff-positive big macrophages. These findings were consistent with globoid cell leucodystrophy, as previously described in cats. Although not specific, in young cats with progressive spinal neurological signs, especially when associated with cerebellar signs, and irregular but diffuse T2 intramedullary hyperintensity and T1 isointensity without contrast uptake, globoid cell leucodystrophy should be suspected.

scholarly journals Neuroaxonal Dystrophy in a Group of Related Cats

1993 ◽  
Vol 5 (4) ◽  
pp. 585-590 ◽  
Author(s):  
K. Paige Carmichael ◽  
Elizabeth W. Howerth ◽  
John E. Oliver ◽  
Kurt Klappenbach
Keyword(s):  
Spinal Cord ◽  
Hind Limb ◽  
Coat Color ◽  
Amorphous Material ◽  
Periodic Acid ◽  
Clinical Signs ◽  
Neuroaxonal Dystrophy ◽  
Periodic Acid Schiff ◽  
Dystrophic Axons ◽  
Positive Core

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.

scholarly journals Brain lesions associated with acute toxic hepatopathy in cattle

2017 ◽  
Vol 29 (3) ◽  
pp. 287-292 ◽  
Author(s):  
Angelica T. B. Wouters ◽  
Flademir Wouters ◽  
Fabiana M. Boabaid ◽  
Tatiane T. N. Watanabe ◽  
Gabriela Fredo ◽  
...  
Keyword(s):  
Liver Disease ◽  
White Matter ◽  
Periodic Acid ◽  
S100 Protein ◽  
Clinical Signs ◽  
Periodic Acid Schiff ◽  
Hematoxylin And Eosin ◽  
Trema Micrantha ◽  
Vacuolated Cytoplasm ◽  
Acute Toxic

Samples of the liver, telencephalon, brainstem, and cerebellum were obtained from 22 bovids suffering from spontaneous or experimental acute toxic liver disease. Perreyia flavipes larvae, and leaves of Cestrum corymbosum, Cestrum intermedium, Dodonaea viscosa, Trema micrantha, and Xanthium cavanillesii were the causal agents in the disorders studied. Hematoxylin and eosin and periodic acid–Schiff staining, as well as anti-S100 protein (anti-S100), anti–glial fibrillary acidic protein (anti-GFAP), and anti-vimentin immunostaining were used to evaluate the brain sections. Astrocytic changes were observed in all samples and were characterized by swollen vesicular nuclei in gray (Alzheimer type II astrocytes) and white matter; and by abundant eosinophilic or vacuolated cytoplasm with pyknotic nuclei in the white matter. These changes were evidenced by anti-S100 and anti-GFAP immunostaining. Our study demonstrates major changes in astrocytes of cattle that died with neurologic clinical signs as the result of acute toxic liver disease.

scholarly journals Glutamine synthetase expression in the brain during experimental acute liver failure (immunohistochemical study)

2021 ◽  
Vol 11 (10) ◽  
pp. 342-356
Author(s):  
T. Shulyatnikova ◽  
V. Tumanskiy
Keyword(s):  
White Matter ◽  
Glutamine Synthetase ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Caudate Nucleus ◽  
Immunohistochemical Study ◽  
Clinical Signs ◽  
Brain Regions ◽  
Progressive Increase ◽  
The Brain

The aim of the study was to determine the immunohistochemical level of glutamine synthetase (GS) expression in different brain regions in the conditions of experimental acute liver failure in rats. Materials and methods. The study was conducted in Wistar rats: 5 sham (control) animals and 10 rats with acetaminophen induced liver failure model (AILF). The immunohistochemical study of GS expression in the sensorimotor cortex, white matter, hippocampus, thalamus, caudate nucleus/putamen was carried out in the period of 12-24 h after acetaminophen treatment. Results. Beginning from the 6th hour after acetaminophen treatment all AILF-animals showed the progressive increase in clinical signs of acute brain disfunction finished in 6 rats by comatose state up to 24 h - they constituted subgroup AILF-B, “non-survived”. 4 animals survived until the 24 h - subgroup AILF-A, “survived”. In the AILF-B group, starting from 16 to 24 hours after treatment, a significant (relative to control) regionally-specific dynamic increase in the level of GS expression was observed in the brain: in the cortex – by 307.33 %, in the thalamus – by 249.47%, in the hippocampus – by 245.53%, in the subcortical white matter – by 126.08%, from 12th hour – in the caudate nucleus/putamen, by 191.66 %; with the most substantive elevation of GS expression in the cortex: by 4.07 times. Conclusion. Starting from the 16th hours after the acetaminophen treatment (from the 12th h in the caudate nucleus/putamen region) and up to 24 h, it is observed reliable compared to control dynamic increase in GS protein expression in the cortex, white matter, hippocampus, thalamus, caudate nucleus/putamen of the rat brain with the most significant elevation in the cortex among other regions. The heterogeneity in the degree of GS expression rising in different brain regions potentially may indicate regions more permeable for ammonia and/or other systemic toxic factors as well as heterogeneous sensitivity of brain regions to deleterious agents in conditions of AILF. Subsequently, revealed diversity in the GS expression reflects the specificity of reactive response of local astroglia in the condition of AILF-encephalopathy during specific time-period. The dynamic increase in the GS expression associated with impairment of animal state, indicates involvement of increased GS levels in the mechanisms of experimental acute hepatic encephalopathy.

scholarly journals Thalamic Melanosis in Goats

2021 ◽  
Vol 49 ◽  
Author(s):  
Yanca Góes Dos Santos Soares ◽  
Draenne Micarla dos Santos Silva ◽  
Maria Jussara Rodrigues do Nascimento ◽  
Millena de Oliveira Firmino ◽  
Rodrigo Cruz Alves ◽  
...  
Keyword(s):  
Cross Sections ◽  
Color Change ◽  
Periodic Acid ◽  
Clinical Signs ◽  
Brown Pigment ◽  
Neoplastic Processes ◽  
Abundant Cytoplasm ◽  
Multiple Blood ◽  
The Brain ◽  
Masson Staining

Background: Melanosis is a blackened pigmentation resulting from the accumulation of melanocytes in tissues that are not normally pigmented. This change in the color of the organs occurs due to the agglomeration of melanocytes originating from abnormal migration during embryogenesis and does not cause dysfunction to the affected organ. Although melanosis frequently occurs in several species and affects several organs such as the brain and spinal cord leptomeninges, involvement in the thalamus region is unusual. The objective of this work was to report two cases of thalamic melanosis in goats, determining the pathological and histochemical aspects that assist in the diagnosis of this condition.Cases: Two cases of thalamic melanosis in goats were diagnosed. In both cases, the animals had no nervous history disease and clinical signs. The cause of death in cases 1 and 2 was established based on anatomopathological findings and clinical signs being diagnosed with mycoplasmosis and asphyxia, respectively. After fixing and making cross-sections of the brain, a focal lengthy blackened area was observed on the thalamus surface in both cases. Microscopically, lesions in the brain were similar in both cases and exclusively affected the thalamus. These cells had abundant cytoplasm, well delimited with brownish granular pigment. The nuclei were difficult to visualize and in some cells, it was rounded, well-defined, morphologically compatible with melanocytes. Melanocytes were mainly distributed around neurons and often distended the perivascular space of multiple blood vessels. In Fontana Masson staining, the granules in the cytoplasm of these cells stained strongly black. The Prussian Blue, Periodic Acid- Schiff's, Von Kossa, and Giemsa stains were negative, and the pigment remained brown. In the unstained slides, assembled after the deparaffinization and clarification process, it was observed the permanence of cells with blackish-brown pigment in the cytoplasm. In immunohistochemistry, strong immunostaining of pigmented cells with the Anti-MelanA antibodies was observed in both cases.Discussion: The diagnosis of thalamic melanosis in goats was carried out based on the characteristic pathological findings, in which melanin pigments were demonstrated and identified through HE, Fontana-Masson staining, and unstained slides and confirmed by the IHC. The use of complementary histochemical techniques was fundamental for the classification of the pigment as melanin, demonstrating to be an accessible and reliable tool for the diagnosis of pathological processes that lead to the accumulation of pigments and or material in the tissues. The occurrence of melanin in the thalamus may be associated with a failure in the migration of melanoblasts, which would go to the optical pathways or to the thalamus. This erratic migration of melanoblasts can be explained by the fact that the forebrain is the embryogenic origin of the optic and diencephalon pathways. Macroscopically, thalamic melanosis must be differentiated mainly from neoplastic processes such as melanoma and hemangiosarcoma, pigmented fungus infections, Phalaris angusta poisoning, listeriosis, neurocutaneous melanosis, and neuromelanin. It was concluded that thalamic melanosis is an uncommon alteration in goats and although it has been diagnosed as an incidental necropsy finding, should be included in the differential diagnosis of diseases that affect the central nervous system, especially those that have a color change associated with the deposition of pigments in the tissues. Keywords: melanin, necropsy findings, pigment, thalamus.Descritores: melanina, achados de necropsia, pigmento, tálamo.Título: Melanose talâmica em caprinos. 

scholarly journals Intramural Vascular Edema in the Brain of Goats With Clostridium perfringens Type D Enterotoxemia

2019 ◽  
Vol 56 (3) ◽  
pp. 452-459 ◽  
Author(s):  
Joaquín Ortega ◽  
José Manuel Verdes ◽  
Eleonora L. Morrell ◽  
John W. Finnie ◽  
Jim Manavis ◽  
...  
Keyword(s):  
Clostridium Perfringens ◽  
Periodic Acid ◽  
Brain Lesions ◽  
Significant Finding ◽  
Periodic Acid Schiff ◽  
Worldwide Distribution ◽  
Type D ◽  
Cerebellar Peduncles ◽  
Cerebral Microangiopathy ◽  
The Brain

Enterotoxemia caused by Clostridium perfringens type D is an important disease of sheep and goats with a worldwide distribution. Cerebral microangiopathy is considered pathognomonic for ovine enterotoxemia and is seen in most cases of the disorder in sheep. However, these lesions are poorly described in goats. In this article, we describe the vasculocentric brain lesions in 44 cases of caprine spontaneous C. perfringens type D enterotoxemia. Only 1 goat had gross changes in the brain, which consisted of mild cerebellar coning. However, 8 of 44 (18%) cases showed microscopic brain lesions, characterized by intramural vascular proteinaceous edema, a novel and diagnostically significant finding. The precise location of the edema was better observed with periodic acid–Schiff, Gomori’s, and albumin stains. Glial fibrillary acidic protein and aquaporin 4 immunostaining revealed strong immunolabeling of astrocyte foot processes surrounding microvessels. The areas of the brain most frequently affected were the cerebral cortex, corpus striatum (basal ganglia), and cerebellar peduncles, and both arterioles and venules were involved. Most of the goats of this study showed lesions in the intestine (enteritis, colitis, and typhlitis), although pulmonary congestion and edema, hydrothorax, hydropericardium, and ascites were also described. Although the intramural edema described, for the first time, in these caprine cases is useful for the diagnosis of enterotoxemia when observed, its absence cannot exclude the disease.

scholarly journals A Single Amino Acid Change in nsP1 Attenuates Neurovirulence of the Sindbis-Group Alphavirus S.A.AR86

2000 ◽  
Vol 74 (9) ◽  
pp. 4207-4213 ◽  
Author(s):  
Mark T. Heise ◽  
Dennis A. Simpson ◽  
Robert E. Johnston
Keyword(s):  
Weight Loss ◽  
Hind Limb ◽  
Nonstructural Protein ◽  
Infectious Clone ◽  
Severe Disease ◽  
Clinical Signs ◽  
Single Amino Acid ◽  
Wild Type ◽  
Single Amino Acid Change ◽  
The Brain

ABSTRACT S.A.AR86, a member of the Sindbis group of alphaviruses, is neurovirulent in adult mice and has a unique threonine at position 538 of nsP1; nonneurovirulent members of this group of alphaviruses encode isoleucine. Isoleucine was introduced at position 538 in the wild-type S.A.AR86 infectious clone, ps55, and virus derived from this mutant clone, ps51, was significantly attenuated for neurovirulence compared to that derived from ps55. Intracranial (i.c.) s55 infection resulted in severe disease, including hind limb paresis, conjunctivitis, weight loss, and death in 89% of animals. In contrast, s51 caused fewer clinical signs and no mortality. Nevertheless, comparison of the virus derived from the mutant (ps51) and wild-type (ps55) S.A.AR86 molecular clones demonstrated that s51 grew as well as or better than the wild-type s55 virus in tissue culture and that viral titers in the brain following i.c. infection with s51 were equivalent to those of wild-type s55 virus. Analysis of viral replication within the brain by in situ hybridization revealed that both viruses established infection in similar regions of the brain at early times postinfection (12 to 72 h). However, at late times postinfection, the wild-type s55 virus had spread throughout large areas of the brain, while the s51 mutant exhibited a restricted pattern of replication. This suggests that s51 is either defective in spreading throughout the brain at late times postinfection or is cleared more rapidly than s55. Further evidence for the contribution of nsP1 Thr 538 to S.A.AR86 neurovirulence was provided by experiments in which a threonine residue was introduced at nsP1 position 538 of Sindbis virus strain TR339, which is nonneurovirulent in weanling mice. The resulting virus, 39ns1, demonstrated significantly increased neurovirulence and morbidity, including weight loss and hind limb paresis. These results demonstrate a role for alphavirus nonstructural protein genes in adult mouse neurovirulence.

scholarly journals Spontaneous Lung Lesions in Aging Laboratory Rabbits (Oryctolagus cuniculus)

2016 ◽  
Vol 54 (1) ◽  
pp. 178-187 ◽  
Author(s):  
T. K. Cooper ◽  
J. W. Griffith ◽  
Z. C. Chroneos ◽  
J. M. Izer ◽  
L. B. Willing ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Smooth Muscle Actin ◽  
Periodic Acid ◽  
Genetic Defect ◽  
Clinical Signs ◽  
Heterotopic Bone ◽  
Alpha Smooth Muscle Actin ◽  
Periodic Acid Schiff ◽  
Lung Lesions ◽  
Age Related

Spontaneous age-related lesions of laboratory rabbits are not well documented in the contemporary scientific literature. A retrospective study of diagnostic necropsies of 36 rabbits >2 years of age found a number of common lung lesions. Fibromuscular intimal hyperplasia affected medium and to a lesser extent large pulmonary arteries and was present to a variable extent in all 36 rabbits >2 years of age. The lesions were characterized by fragmentation and/or reduplication of the internal elastic lamina (IEL), proliferation of smoothelin+/alpha-smooth muscle actin (α-SMA)+/vimentin− smooth muscle cells and fewer smoothelin−/α-SMA+/vimentin+ myofibroblasts, and intimal deposition of collagen without thrombosis, embolism, or evidence of pulmonary hypertension. Pulmonary emphysema, present in 30/36 rabbits, was characterized by the loss of alveolar septa; most affected rabbits did not have clinical signs of respiratory disease. In 8/13 rabbits of the inbred EIII/JC audiogenic strain, we identified a unique syndrome of granulomatous pneumonia containing hyaline brown to gray, globular to ring-like acellular material that was Alcian blue and periodic acid-Schiff positive. The material was immunoreactive for surfactant protein-A and had the ultrastructural appearance of multilamellar vesicles, suggesting a genetic defect in surfactant metabolism. Additionally, we found small benign primary lung tumors (fibropapillomas, 5 rabbits) not previously described. Other findings included heterotopic bone (5 rabbits), subacute to chronic suppurative bronchopneumonia, pyogranulomatous pneumonia with plant material, and pulmonary artifacts from barbiturate euthanasia solution.

scholarly journals Halicephalobus gingivalis (H.deletrix) in the brain of a horse

2007 ◽  
Vol 37 (4) ◽  
pp. 1185-1187 ◽  
Author(s):  
Rosemeri de Oliveira Vasconcelos ◽  
Karen Regina Lemos ◽  
Julieta Rodini Engracia de Moraes ◽  
Vívian Palmeira Borges
Keyword(s):  
White Matter ◽  
Histological Examination ◽  
Inflammatory Infiltrate ◽  
Clinical Signs ◽  
Nematode Infection ◽  
Etiological Agent ◽  
Axonal Spheroids ◽  
The Brain ◽  
Halicephalobus Gingivalis

A 10-year-old Mangalarga gelding with rhabditiform nematode infection in the brain is described. Clinical signs were limited to circling and right side paralysis. Histological examination of the brain revealed marked gliosis and discreet edema. The perivascular mononuclear inflammatory infiltrate was composed of few layers of lymphocytes, plasmocytes and macrophages and rare eosinophils. The presence of rhabditiform nematodes was associated with the infiltrate. Areas of malacia associated with the parasites and parasite tracks with axonal spheroids were also seen close to the vessels and to the etiological agent and were more evident in the white matter. In the meninges there was moderate inflammatory infiltrate associated with perivascular parasites. The identification of the nematode was based on the histological examination of the cerebral fragments.

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