J Korean Orthop Assoc.  1999 Aug;34(4):769-772.

Dopa-responsive Dystonia Misdiagnosed as Cerebral Plasy: A Case Report

Affiliations
  • 1Department of Orthopaedic Surgery, College of Medicine, Dong-A University, Pusan, Korea.
  • 2Department of Neurology, College of Medicine, Dong-A University, Pusan, Korea.

Abstract

Dopa-responsive dystonia (DRD) is a slowly progressive dystonia with childhood onset and is characterized by marked diurnal fluctuation of symptoms, dramatic response to levodopa treatment and concurrent signs of parkinsonism. We report a 16-year-old girl diagnosed as DRD. Around the age of 11, gait disturbance was developed with equinocavovarus deformity of both feet. The plantar fasciotomy, triple arthrodesis and posterior tibialis tendon transfer for left foot with the diagnosis of cerebral palsy were done. She complained of a persistent dystonia of all extremities after operation and was successfully treated with low-dose levodopa after a diagnosis of DRD. For the accurate diagnosis and prevention of unnecessary operation, trial of levodopa is warranted in patients suspected with cerebral palsy with similar symptoms of DRD.

Keyword

Dopa-responsive dystonia; Diurnal fluctuation; Levodopa
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