[The pathology of Joseph's disease in a Chinese family: a report of two autopsy cases].
This is the first pathology report on Joseph's disease in China, based on autopsies of 2 members of the Huang family. The main pathological changes were chiefly located in the central nervous system including globus pallidus and Luy's body, cerebellar dentate nucleus, cranial nerve motor nuclei of brain stem, substantia nigra and red nuclei, and also there were severe loss of neurons in the anterior horn and Clarke's nuclei; severe demyelinization of cerebellospinal tract and degeneration with demyelinization of the peripheral nerves accompanied by neurogenic skeletal muscular atrophy. It is concluded that the pathologic characteristics were similar to those found in Europe, United States and Japan.
Zhao
J B
JB
Department of pathology, China-Japan Friendship Hospital, Beijing.
Wang
T L
TL
Wang
G X
GX
D000328:Adult; D002529:Cerebellar Nuclei / Q000473:pathology; D005260:Female; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D017827:Machado-Joseph Disease / Q000235:genetics / Q000473:pathology*; D008297:Male; D010375:Pedigree