RT Journal Article
SR Electronic
T1 Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 140
OP 149
DO 10.1136/jnnp.41.2.140
VO 41
IS 2
A1 Bird, T D
A1 Shaw, C M
YR 1978
UL http://jnnp.bmj.com/content/41/2/140.abstract
AB Ramsay Hunt's progressive myoclonus and epilepsy associated with dentatorubral degeneration is a rare disorder. We report a 19 year old woman with this clinical syndrome who also has a more mildly affected brother. Neuropathological in addition to dentatorubal involvement. The evidence suggests that this is a distinctive hereditary disorder producing neuromal degeneration at several levels in the central nervous system.