Hemiparkinsonism and hemiatrophy syndrome: A rare observation

Hemiparkinsonism with hemiatrophy syndrome is defined by the occurrence of a body hemiatrophy with features of an early onset, slowly progressive, asymmetric parkinsonism more prominent on the side of the hemiatrophy. The underlying pathogenesis is not well understood but perinatal cerebral insults seem to play a crucial role. We describe the case of a 52-year-old woman who presented with a two year history of slowness and stiffness of left arm and leg with dystonic posturing of the ipsilateral hand. When examined, she had a left body hemiatrophy which was present since early childhood. Clinical and imaging findings supported the diagnosis of hemiparkinsonism with hemiatrophy syndrome with a good response to dopamine agonist therapy. This case report further characterizes this heterogeneous form of parkinsonism which deserves attention for clinical management and prognostic evaluation. (C) 2010 Elsevier B.V. All rights reserved.