HEREDITARY PARKINSONISM WITH MULTIPLE SYSTEM DEGENERATION - BENEFICIAL EFFECT OF ANTICHOLINERGICS, BUT NOT OF LEVODOPA

Two siblings who exhibited hereditary parkinsonism with pyramidal sign s and cerebellar ataxia are reported, Anticholinergics had a dramatic beneficial effect in both cases, but levodopa did not. This responsive ness, which is similar to that reported in patients with Joseph's dise ase, suggests dysfunction of an ''indirect pathway'' involving the glo bus pallidus and the subthalamic nucleus, in addition to that of the n igrostriatal system. We propose a new hereditary variant of early onse t Parkinson's disease, distinct from the levodopa sensitive forms of j uvenile Parkinson's disease.