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NEUROLOGY 2005;64:1391-1396
© 2005 American Academy of Neurology

Disorganized somatotopy in the putamen of patients with focal hand dystonia

C. Delmaire, MD, A. Krainik, MD, S. Tézenas du Montcel, MD, PhD, E. Gerardin, MD, S. Meunier, MD, PhD, J. -F. Mangin, PhD, S. Sangla, MD, L. Garnero, PhD, M. Vidailhet, MD and S. Lehéricy, MD, PhD

From the Department of Neuroradiology (Drs. Delmaire and Lehéricy), INSERM U610 (Drs. Delmaire, Krainik, Gérardin and Lehéricy), Biostatistics and Medical Informatics Department (Dr. Tézenas du Montcel), INSERM EO349 (Dr. Meunier), CNRS UPR 640 (Dr. Garnero), and INSERM U289 (Dr. Vidhailet), Hôpital de la Pitié-Salpétrière, Paris; Department of Neuroradiology (Dr. Gérardin), Hôpital C. Nicolle, Rouen; SHFJ-CEA and IFR49 (Drs. Mangin and Lehéricy), Orsay; and Department of Neurology (Drs. Sangla and Vidhailet), Hôpital Saint Antoine, Paris, France.

Address correspondence and reprint requests to Dr. Stéphane Lehéricy, Service de Neuroradiologie, Bâtiment Babinski, Hôpital de la Salpêtrière, 47/83 Bd de l’Hôpital, 75651 Paris CEDEX, France; e-mail: stephane.lehericy{at}psl.ap-hop-paris.fr

Objective: To explore the selectivity of neuronal somatotopic representation in the striatum of patients with unilateral task-specific dystonia of the right arm.

Materials and Methods: The authors used fMRI in 14 right-handed dystonic subjects to examine putaminal organization. Subjects performed flexion/extension of the right and left fingers and toes, and contraction of the lips.

Results: Compared to healthy volunteer subjects, dystonic subjects had altered somatotopic organization in the left putamen, contralateral to the affected hand. Disease severity correlated with underactivation and decreased distance between right hand and lip representations. In the right putamen, ipsilateral to the affected hand, the somatotopic organization was not altered but disease severity also correlated with reduced distances between limbs.

Conclusion: In dystonia there may be a dedifferentiation of the normally segregated cortico-subcortical sensorimotor maps in the putamen, which may contribute to the loss of functional selectivity of muscle activity observed in these dystonic subjects.


Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the April 26 issue to find the title link for this article.

Supported by grants from INSERM (RBM 99011), the Clinical Investigation Center of the Salpêtrière Hospital, Paris, the IFR 49, and by the Action Concertée Incitative 2001-CR UFR 65/6503H.

Received June 11, 2004. Accepted in final form January 4, 2005.




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