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NEUROLOGY 2006;66:1574-1576
© 2006 American Academy of Neurology
Brief Communications

Fixation-sensitive myoclonus in Lafora disease

S. Kumada, MD, M. Kubota, MD, M. Hayashi, MD, A. Uchiyama, MD, K. Kurata, MD and Y. Kagamihara, MD

From the Departments of Neuropediatrics (S.K.) and Neurology (Y.K.), Tokyo Metropolitan Neurological Hospital; Department of Pediatrics (S.K., A.U., K.K.), Metropolitan Fuchu Medical Center for Severe Motor and Intellectual Disabilities; Department of Pediatrics (M.K.), the University of Tokyo; and Department of Clinical Neuropathology (M.H.), Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan.

Address correspondence and reprint requests to Dr. S. Kumada, Department of Neuropediatrics, Tokyo Metropolitan Neurological Hospital, 2-6-1, Musashidai, Fuchu-shi, Tokyo, 183-0042, Japan; e-mail: skumada{at}tmnh.fuchu.tokyo.jp

The authors report a patient with Lafora disease, whose myoclonus was suppressed by passive eye closure. Neurophysiologic studies disclosed that fixation was the most important enhancer of myoclonus. Magnetoencephalographic studies of visual evoked fields revealed abnormal activation of the visual corticocortical pathway via the insular cortex not seen in controls. The authors hypothesize that abnormal activation of the insular cortex may be involved in triggering the mechanism of fixation-sensitive myoclonus.

Disclosure: The authors report no conflicts of interest.

Received June 27, 2005. Accepted in final form February 8, 2006.

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 May 23 issue to find the title link for this article.






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