Abstract

Dystonia associated with neurodegenerative disease has minimal effective treatment options and can be devastating to a patient's ability to perform tasks of daily living. We present a case of a 55 year-old man who had progressive symptoms of an atypical asymmetric parkinsonian neurodegenerative disease. This patient presented with a dystonic left upper extremity that was refractory to treatment. In an attempt to treat worsening pain associated with the dystonia, he was given a five-day lidocaine infusion for associated pain and within 24 h had improvement in mobility of his dystonic extremity. Dystonia was measured by the Burke–Fahn–Marsden (BFM) dystonia rating and disability scales on hospital day five and at an eight week follow up visit. These scores were compared with scores derived from his previous pre-treatment neurologic examination. The BFM dystonia scale score was initially 16 and improved to 12 on both immediate post-treatment and eight-week follow-up. The BFM disability score improved from 16 to 6 post treatment and to 8 on follow-up appointment. Most importantly, the patient could feed and dress himself for the first time in several years. No adverse events of treatment were encountered. Treatment effect lasted three months with a slow return to baseline motor function. This case report raises interesting questions regarding the mechanism of dystonia in neurodegenerative disease and suggests the afferent sensory system as a potential target for therapeutics.

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