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Neurology 2002;59:1447-1449
© 2002 American Academy of Neurology
Brief Communications

N-acetylcysteine and Unverricht–Lundborg disease Variable response and possible side effects

M.J.J. Edwards, MRCP, I.P. Hargreaves, PhD, S.J.R. Heales, PhD, S.J. Jones, PhD, V. Ramachandran, MD, K.P. Bhatia, FRCP and S. Sisodiya, PhD

From Sobell Department of Motor Neuroscience and Movement Disorders (M.J.J. Edwards and Drs. Ramachandran and Bhatia) and Department of Clinical Epilepsy (Dr. Sisodiya), Institute of Neurology, University College London, Queen Square; Neurometabolic Unit (Drs. Hargreaves and Heales), Department of Clinical Biochemistry, National Hospital For Neurology and Neurosurgery; and Department of Clinical Electrophysiology (Dr. Jones), National Hospital For Neurology, London, UK.

Address correspondence and reprint requests to Dr. K. Bhatia, Sobell Dept. of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London, UK WC1N 3BG; e-mail: k.bhatia{at}ion.ucl.ac.uk

Serum glutathione levels were assessed in a patient with genetically proven Unverricht–Lundborg disease (ULD) before and during treatment with the antioxidant N-acetylcysteine (NAC). Glutathione levels were low before treatment, and increased during treatment. This increase was mirrored by an improvement in seizures, but not in myoclonus or ataxia. Three other patients with clinically determined ULD showed a variable response and some notable side effects during treatment with NAC.




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