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

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.

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Globoid Cell Leucodystrophy in Polled Dorset Sheep

Veterinary Pathology ◽

10.1177/030098588001700402 ◽

1980 ◽

Vol 17 (4) ◽

pp. 399-405 ◽

Cited By ~ 33

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.

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

Veterinary Pathology ◽

10.1177/0300985818817071 ◽

2019 ◽

Vol 56 (3) ◽

pp. 452-459 ◽

Cited By ~ 2

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.

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Globoid Cell-like Leukodystrophy in a Domestic Longhaired Cat

Veterinary Pathology ◽

10.1354/vp.39-4-494 ◽

2002 ◽

Vol 39 (4) ◽

pp. 494-496 ◽

Cited By ~ 21

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.

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Histochemical characteristics and distribution of lipofuscin and polyglucosan bodies in the brain of dogs more than 10 years old

Veterinarski glasnik ◽

10.2298/vetgl201002001n ◽

2021 ◽

pp. 1-1

Author(s):

Sladjan Nesic ◽

Ivana Vucicevic ◽

Darko Marinkovic ◽

Vladimir Kukolj ◽

Milan Anicic ◽

...

Keyword(s):

Brain Tissue ◽

Medulla Oblongata ◽

Periodic Acid ◽

Ageing Process ◽

Specific Staining ◽

Periodic Acid Schiff ◽

Polyglucosan Bodies ◽

Age Related ◽

Lipofuscin Pigment ◽

The Brain

The ageing process is accompanied by numerous changes in the brain of dogs, such as accumulation of amyloid, fibrosis of blood vessel walls and meninges, accumulation of lipofuscin, and the presence of polyglucosan bodies (PGBs), satellitosis and neuronophagia. In this study, the presence of lipofuscin and PGBs in various parts of the brain in dogs of different sexes and ages was examined. For this purpose, brain samples were stained using haematoxylin eosin, modified Ziehl Neelsen and Periodic acid Schiff (PAS) methods. Lipofuscin was visualised by Ziehl Neelsen and PAS methods of specific staining on the same brain tissue segments. Lipofuscin had accumulated in 93% of old (more than 10 years old) dog brains, mostly in neurons of the medulla oblongata. The percentage of age-related lipofuscin pigment in other examined brain tissue segments was lower than in the medulla oblongata. There was a small difference in the percentage of lipofuscin-positive individuals between the two staining methods. The presence of PGBs was established by the PAS method for the vast majority (about 93%) of the old dogs (more than 10 years old), while PGBs were not detected in the group of young dogs (up to 5 years old). However, PGBs occurred in all examined segments of the dog?s brain tissues (for each of the tissue types, from 90% to 93% of dogs were positive for PGBs). The results obtained the oldest dogs (15 years old) harboured PGBs both extracellularly and intracellularly, while in other dogs, only extracellular PGBs were seen. Lipofuscin was accumulated mostly in large neurons of olivary nuclei of the medulla oblongata. PGBs were confirmed in all examined segments of the brain tissue of dogs more than 10 years old. This is one of the numerous indications that old dogs could be a very good animal model for studying the normal ageing process or neurodegenerative diseases.

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Cerebrospinal nematodiasis outbreak in an urban outdoor aviary of cockatiels (Nymphicus hollandicus) in southern California

Journal of Veterinary Diagnostic Investigation ◽

10.1177/1040638712455797 ◽

2012 ◽

Vol 24 (5) ◽

pp. 994-999 ◽

Cited By ~ 5

Author(s):

Santiago S. Diab ◽

Francisco A. Uzal ◽

Federico Giannitti ◽

H.L. Shivaprasad

Keyword(s):

Southern California ◽

Granulomatous Inflammation ◽

Lesion Site ◽

Axonal Swelling ◽

Neurological Signs ◽

Nematode Larvae ◽

Nymphicus Hollandicus ◽

The Central Nervous System ◽

Brain And Spinal Cord ◽

The Brain

An outbreak of cerebrospinal nematodiasis due to Baylisascaris sp. was documented in an urban outdoor aviary in southern California. Thirty-four out of 35 cockatiels ( Nymphicus hollandicus, 97%) showed a variety of neurological signs including ataxia, lateral recumbency, opisthotonus, and torticollis. Thirteen carcasses were submitted for necropsy; the histological lesions were restricted to the central nervous system (brain and spinal cord) and were predominantly degenerative, characterized by multifocal white matter vacuolation, gliosis, axonal swelling, gitter cell infiltration, and mild hemorrhage, rarely accompanied by mild granulomatous inflammation and mild lymphocytic perivascular cuffs. Nematode larvae morphologically compatible with Baylisascaris sp. were observed in the brain of 5 birds, away from the lesion site.

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Periodic acid-Schiff granules in the brain of aged mice: From amyloid aggregates to degenerative structures containing neo-epitopes

Ageing Research Reviews ◽

10.1016/j.arr.2016.03.001 ◽

2016 ◽

Vol 27 ◽

pp. 42-55 ◽

Cited By ~ 11

Author(s):

Gemma Manich ◽

Itsaso Cabezón ◽

Elisabet Augé ◽

Carme Pelegrí ◽

Jordi Vilaplana

Keyword(s):

Periodic Acid ◽

Periodic Acid Schiff ◽

Aged Mice ◽

Amyloid Aggregates ◽

The Brain

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A Case of Periarteritis Nodosa of the Central Nervous System

Journal of Mental Science ◽

10.1192/bjp.96.403.470 ◽

1950 ◽

Vol 96 (403) ◽

pp. 470-475 ◽

Cited By ~ 11

Author(s):

Mavis E. Mackay ◽

Turner McLardy ◽

Cyril Harris

Keyword(s):

Unusual Case ◽

Signs And Symptoms ◽

Periarteritis Nodosa ◽

Neurological Signs ◽

Mental Symptoms ◽

The Central Nervous System ◽

Pathological Report ◽

Brain And Spinal Cord ◽

The Brain ◽

Exacerbation Of Symptoms

This is a clinical and pathological report on an unusual case of periarteritis nodosa in which the disease was confined chiefly to the brain and spinal cord. The diffuseness, variability and intermittency of the neurological signs and symptoms over a period of two years, in the absence of many of the more usual somatic manifestations of periarteritis nodosa, was responsible for the nature of the disorder not being diagnosed before death. Mental symptoms were prominent throughout the last year of illness. T.A.B. pyrexial therapy coincided with a marked remission in physical and mental symptoms, whilst arsenical therapy appeared to precipitate the terminal exacerbation of symptoms.

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