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Keywords:

  • Dentatorubro pallidoluysian atrophy;
  • Neurodegenerative disease;
  • Ramsay Hunt syndrome

Abstract

Dentatorubro-pallidoluysian atrophy (DRPLA) has a variable clinical presentation but the pathology is routinely confined to cerebellifugal and pallidofugal systems. We present a case of DRPLA of the myoclonus epilepsy type in which prominent posterior column degeneration was added to the pathological picture. This case illustrates the concept that the neurodegenerative diseases can be a continuum of both clinical and pathological presentations.