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Keywords:

  • dentatorubral pallidoluysian atrophy, DRPLA, sporadic, intranuclear inclusion, polyglutamine repeat

Abstract

We present a patient with a 14-year course beginning at the age of 44 years with hemidystonia followed by generalized choreoathetosis, behavioral, and oculomotor disturbances. Family history and genetic testing were unrevealing. Neuropathological findings were identical to genetic dentatorubral pallidoluysian (DRPLA) except for the lack of intranuclear inclusions. © 2003 Movement Disorder Society