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Globoid cell leukodystrophy ( K rabbe's disease) in a J apanese domestic cat
Author(s) -
Ogawa Mizue,
Uchida Kazuyuki,
Isobe Kyoko,
Saito Miyoko,
Harada Tomoyuki,
Chambers James K.,
Nakayama Hiroyuki
Publication year - 2014
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12076
Subject(s) - pathology , gliosis , leukodystrophy , white matter , medicine , spinal cord , myelin , cerebellum , internal capsule , infiltration (hvac) , disease , central nervous system , magnetic resonance imaging , psychiatry , physics , thermodynamics , radiology
A male J apanese domestic cat developed progressive limb paralysis from 4 months of age. The cat showed visual disorder, trismus and cognitive impairment and died at 9 months of age. At necropsy, significant discoloration of the white matter was observed throughout the brain and spinal cord. Histologically, severe myelin loss and gliosis were observed, especially in the internal capsule and cerebellum. In the lesions, severe infiltration of macrophages with broad cytoplasm filled with PAS ‐positive and non‐metachromatic granules (globoid cells) was evident. On the basis of these findings, the case was diagnosed as feline globoid cell leukodystrophy ( K rabbe's disease). Immunohistochemical observation indicated the involvement of oxidative stress and small HSP in the disease.