A non-familial Huntington's disease patient with grumose degeneration in the dentate nucleus

Authors
Ishikawa, A Oyanagi, K Tanaka, K Igarashi, S Sato, T Tsuji, S
Citation
A. Ishikawa et al., A non-familial Huntington's disease patient with grumose degeneration in the dentate nucleus, ACT NEUR SC, 99(5), 1999, pp. 322-326
Citations number
12
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
ACTA NEUROLOGICA SCANDINAVICA
ISSN journal
00016314 → ACNP
Volume
99
Issue
5
Year of publication
1999
Pages
322 - 326
Database
ISI
SICI code
0001-6314(199905)99:5<322:ANHDPW>2.0.ZU;2-H
Abstract
We report a non-familial Huntington's disease (HD) patient presenting with increased levels of protein and IgG in his cerebrospinal fluid (CSF), antin euronal antibody in his serum and CSF, Purkinje cell and granule cell degen eration in the cerebellum, and grumose degeneration in the dentate nucleus, in addition to typical HD findings. This patient showed an expanded CAG re peat in the HD gene, and provides new information on the clinical and neuro pathologic varieties of HD.