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Neurology 2001;56:1236-1239
© 2001 American Academy of Neurology
Brief Communications

A favorable response to antiparkinsonian treatment in juvenile neuronal ceroid lipofuscinosis

Laura E. Åberg, MD;, Juha O. Rinne, MD, PhD;, Irmeli Rajantie, PT; and Pirkko Santavuori, MD, PhD

From the Departments of Pediatric Neurology (Drs. Åberg and Santavuori) and Physical Therapy (Dr. Rajantie), Hospital for Children and Adolescents, University of Helsinki; and Department of Neurology and Turku PET Centre (Dr. Rinne), University of Turku, Finland.

Address correspondence and reprint requests to Dr. L. Åberg, Pediatric Neurology, Hospital for Children and Adolescents, PL 280, 00029 HYKS, Helsinki, Finland; e-mail: laura.aberg{at}helsinki.fi

To study the effect of dopaminergic drugs on the parkinsonism in juvenile neuronal ceroid lipofuscinosis, the authors conducted an open study of 21 patients. According to the motor Unified PD Rating Scale (UPDRS) score, treatment was initiated with either levodopa (n = 10) or selegiline (n = 6). Five patients served as a control group. The UPDRS score after 1 year was compared with the score at onset. Both in the control group and in the selegiline group, the mean UPDRS score increased, whereas in the levodopa group, the mean UPDRS score decreased. The difference between the levodopa group and the control group was significant.




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