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A non‐familial Huntington's disease patient with grurnose degeneration in the dentate nucleus
Author(s) -
Ishikawa A.,
Oyanagi K.,
Tanaka K.,
Igarashi S.,
Sato T.,
Tsuji S.
Publication year - 1999
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1999.tb00684.x
Subject(s) - dentate nucleus , degeneration (medical) , pathology , cerebellum , huntington's disease , neuronal degeneration , cerebrospinal fluid , granule cell , nucleus , degenerative disease , medicine , disease , neuroscience , biology , central nervous system , dentate gyrus
We report a non‐familial Huntington's disease (HD) patient presenting with increased levels of protein and IgG in his cerebrospinal fluid (CSF), anti‐neuronal antibody in his serum and CSF, Purkinje cell and granule cell degeneration in the cerebellum, and grumose degeneration in the dentate nucleus, in addition to typical HD findings. This patient showed an expanded CAG repeat in the HD gene, and provides new information on the clinical and neuropathologic varieties of HD.