Ramsay Hunt syndrome in dentatorubral-pallidoluysian atrophy.
We report a case of Ramsay Hunt syndrome which was clinically characterized by myoclonus epilepsy, cerebellar ataxia, convulsions, and dementia. Major necropsy findings were dentatorubral-pallidoluysian atrophy. Dentatorubral-pallidoluysian atrophy may be associated with a variety of clinical symptoms, amongst which Ramsay Hunt syndrome can be included.
D001284:Atrophy; D002524:Cerebellar Ataxia / Q000473:pathology*; D002529:Cerebellar Nuclei / Q000473:pathology*; D002648:Child; D005917:Globus Pallidus / Q000473:pathology*; D006801:Humans; D008297:Male; D002527:Myoclonic Cerebellar Dyssynergia / Q000473:pathology*; D009410:Nerve Degeneration; D012012:Red Nucleus / Q000473:pathology*; D013787:Thalamic Nuclei / Q000473:pathology*