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From the Human Motor Control Section (Drs. Ibáñez, Sadato, Karp, Deiber, and Hallett), Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; the Neuroimaging Unit (Dr. Ibáñez), Division de Neuropsychiatrie, Belle-Idée, Genève, Switzerland; and the Institut National de la Santé et de la Recherche Médicale CERMEP (Dr. Deiber), Lyon, France.
Address correspondence and reprint requests to Dr. Mark Hallett, NINDS, NIH, Building 10, Room 5N226, 10 Center Drive MSC 1428, Bethesda, MD 20892-1428; e-mail: hallett{at}codon.nih.gov
OBJECTIVE: To study regional cerebral blood flow (rCBF) in patients with simple writer’s cramp using PET to identify regions that malfunction.
BACKGROUND: Several lines of evidence indicate impaired cortical function in patients with focal dystonia, but the precise pathophysiology is still unknown.
METHODS: Seven patients with writer’s cramp were compared with seven age- and sex-matched control subjects. Control subjects and patients were scanned during sustained contraction, tapping, and writing with the right hand. After realignment and stereotactic normalization of the scans, all tasks were compared with a rest condition. For each task, an intra- and intergroup comparison was performed using statistical parametric mapping. For each condition and within groups, rCBF correlation analysis was performed between some selected regions that were activated during movement.
RESULTS: In control subjects and patients, significant increases of rCBF were observed for each task in areas already known to be activated in motor paradigms. The intergroup comparison disclosed less activation in writer’s cramp patients for several areas for all three tasks. This decrease reached significance for the sensorimotor cortex during the sustained contraction task and for the premotor cortex during writing. rCBF correlation analysis showed different patterns between control subjects and patients. At rest and during writing, the correlations between the putamen and premotor cortical regions and between the premotor cortical regions themselves were stronger in control subjects.
CONCLUSIONS: Deficient activation of premotor cortex and decreased correlation between premotor cortical regions and putamen suggest a dysfunction of the premotor cortical network in patients with writer’s cramp possibly arising in the basal ganglia. The dysfunction is compatible with a loss of inhibition during the generation of motor commands, which in turn could be responsible for the dystonic movements.
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