Clinical syndrome of dystonia-parkinsonism

J Korean Neurol Assoc > Volume 15(2); 1997 > Article

Journal of the Korean Neurological Association 1997;15(2): 286-299.

근긴장이상-파킨슨증후군

김용덕, 이명식, 박재현, 장대일

연세대학교 의대 영동세브란스병원 신경과. 연세 뇌 연구소. 연세대학교 의대

Clinical syndrome of dystonia-parkinsonism

Yong Duk Kim, M.D., Myung Sik Lee, M.D., Jae Hyeon Park, M.D., Dae Il Chang, M.D.

Department of Neurology, Yongdong Severance Hospital, Yonsei Brain Research Center, Yonsei University. Department of Neurology, College of Medicine, Inje University, Sanggye Paik Hospital. Department of Neurology, Kyung Hee University

Abstract

We report 11 patients who developed dystonia and parkinsonian symptoms and signs. All of them developed the onset of parkinsonian symptoms 1 month to about 50 years(median: 2 yrs) after the onset of dystonia. Six of 11 patients developed dystonia affecting a hand; two affecting a foot; one affecting the neck; one affecting bilateral hands and a foot; one affecting a foot, trunk and neck Their parkinsonian symptoms had progressed very slowly and responded well to the levodopa treatment. Beside, none of them had clinical features suggestive of parkinsonian-plus syndrome. These findings suggest that they may have pathological changes confined to the nigrostriatal dopaminergic system as those with idiopathic Parkinson's disease. However, no change or worsening of dystonia after levodopa treatment suggest that they may have additional lesions out-side of the nigrostriatal dopaminergic system. More pathological studies and functional brain imaging studies are needed to define the exact pathogenesis of clinical syndrome of dystonia-parkinsonism.