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.
D000328:Adult; D001284:Atrophy; D001933:Brain Stem / Q000473:pathology; D002529:Cerebellar Nuclei / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology*; D004827:Epilepsy / Q000235:genetics / Q000473:pathology*; D005260:Female; D006624:Hippocampus / Q000473:pathology; D006801:Humans; D008636:Mesencephalon / Q000473:pathology; D002527:Myoclonic Cerebellar Dyssynergia / Q000473:pathology*; D009207:Myoclonus / Q000235:genetics / Q000473:pathology*; D009336:Necrosis; D009410:Nerve Degeneration; D012012:Red Nucleus / Q000473:pathology*; D012021:Reflex, Abnormal; D013116:Spinal Cord / Q000473:pathology; D013577:Syndrome