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John G. Nutt, MD; Torbjoern G. Nygaard, MD

Arch Neurol. 2001;58:905-910.

Background  Dopa-responsive dystonia (DRD) is similar to Parkinson disease in that both disorders have impaired dopamine synthesis and respond to levodopa treatment. Dopa-responsive dystonia differs in that dopamine storage is intact in contrast to Parkinson disease in which it is markedly reduced.

Objective  To examine the short- and long-duration responses to levodopa dosing in subjects with DRD.

Methods  The response to brief infusions of levodopa was examined in 4 subjects with DRD and the effects of withdrawal of levodopa for 3 to 7 days studied in the 3 subjects receiving long-term levodopa therapy. Motor function was measured with tapping speed, Unified Parkinson's Disease Rating Scale motor score, and global dystonia score.

Results  The short-duration response to levodopa dosing seems to develop more slowly and persists longer in subjects with DRD than in subjects with Parkinson disease. Withdrawal of levodopa leads to a gradual decline in tapping speed and reemergence of dystonia over several days, similar to the rate of decay of motor function in Parkinson disease. The short- and long-duration responses were not clearly differentiated in DRD.

Conclusions  This pilot study suggests that retained dopamine storage in DRD may prolong the short-duration response and blur the distinction of the short- and long-duration responses. The decline in motor function in DRD on withdrawal of long-term levodopa therapy resembles that in Parkinson disease, suggesting that a long-duration response, if it exists in DRD, is unrelated to dopamine storage.

From the Departments of Neurology and Physiology and Pharmacology, Oregon Health Sciences University, Portland (Dr Nutt); Department of Neurology, East Orange Veterans Affairs Medical Center, East Orange, NJ (Dr Nygaard); and the Department of Neurosciences, University of Medicine and Dentistry–New Jersey Medical School, Newark, NJ (Dr Nygaard).

Corresponding author: John G. Nutt, MD, Department of Neurology, OP 32, Oregon Health Sciences University, 3181 SW Sam Jackson Park Rd, Portland, OR 97201-3098.

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