J Neurol Neurosurg Psychiatry 41:140-149 doi:10.1136/jnnp.41.2.140
  • Research Article

Progressive myoclonus and epilepsy with dentatorubral degeneration: a clinicopathological study of the Ramsay Hunt syndrome.


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.