PT  - JOURNAL ARTICLE
AU  - Bird, T D
AU  - Shaw, C M
TI  - Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.
AID  - 10.1136/jnnp.41.2.140
DP  - 1978 Feb 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 140--149
VI  - 41
IP  - 2
4099  - http://jnnp.bmj.com/content/41/2/140.short
4100  - http://jnnp.bmj.com/content/41/2/140.full
SO  - J Neurol Neurosurg Psychiatry1978 Feb 01; 41
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.