Fixation-sensitive myoclonus in Lafora disease.
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.
Kumada
S
S
Department of Neuropediatrics, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan. skumada@tmnh.fuchu.tokyo.jp
Kubota
M
M
Hayashi
M
M
Uchiyama
A
A
Kurata
K
K
Kagamihara
Y
Y
D000293:Adolescent; D000927:Anticonvulsants / Q000008:administration & dosage / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000503:physiopathology*; D003704:Dementia / Q000209:etiology; D018450:Disease Progression; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D005073:Evoked Potentials, Somatosensory; D005074:Evoked Potentials, Visual; D005143:Eyelids / Q000502:physiology; D005403:Fixation, Ocular; D006801:Humans; D020192:Lafora Disease / Q000150:complications* / Q000188:drug therapy / Q000628:therapy; D015225:Magnetoencephalography; D008297:Male; D009044:Motor Cortex / Q000503:physiopathology; D012798:Sialorrhea / Q000209:etiology / Q000628:therapy; D013226:Status Epilepticus / Q000188:drug therapy / Q000209:etiology / Q000517:prevention & control; D014793:Visual Cortex / Q000503:physiopathology
D000927:Anticonvulsants