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• Vol.48 No.11
• CLINICA NEUROL, 48: 844−847, 2008
• Vol.48 No.11 contents

The 49th Annual Meeting of the Japanese Society of Neurology

Diagnosis and treatment of dystonia

Ryuji Kaji, M.D, Ph.D., Kenta Sato, M.D., Wataru Sako, M.D. and Satoshi Goto, M.D, Ph.D.

Department of Neurology, Tokushima University

Diagnosis of dystonia is not difficult by recognizing the pattern of clinical presentation. Dopa-responsive dystonia (DRD) and Wilson disease are important in differential diagnosis because of their specific treatment. The most common are the focal dystonias, including blepharospasm and spasmodic torticollis. Dystonia comprises mobile involuntary movements and abnormal postures, the latter is better described as hypokinetic disorder. The pathogenesis of dystonia is now being clarified, and includes abnormal neuroplasticity caused by the relative excess of dopamine in the matrix compartment of the striatum, the possible primary lesion being the striosome. In a dopa-responsive dystonia model, dopaminergic projection is more deficient to the striosome than to the matrix, which could produce imbalance between the direct versus. indirect pathway activities. The treatment options include trihexyphenidyl, minor tranquilizers, botulinum toxin injection, and deep brain stimulation.
Full Text of this Article in Japanese PDF (348K)

(CLINICA NEUROL, 48: 844−847, 2008)
key words: dystonia, striosome, matrix, DYT3, DRD

(Received: 16-May-08)