Paroxysmal kinesigenic choreoathetosis |
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Authors: | Shaul Harel U. Yurgenson M. Kutai |
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Affiliation: | (1) Pediatric Neurology Unit, Division of Pediatrics, Tel Aviv Medical Center, Sackler Medical School, University of Tel Aviv, Tel Aviv, Israel;(2) Child Development Center, 14 Balfour Street, 652211 Tel Aviv, Israel |
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Abstract: | A healthy intelligent 13.5-year-old boy is reported who presented with paroxysmal kinesigenic choreoathetosis. He had had unilateral tonic spasms for 2 months. In a stressful situation, especially after a period of immobility, a sudden voluntary movement provoked a dystonic-choreoathetoic posturing of the left arm and leg. Attacks were brought on, for instance, by standing up quickly from a sitting position. They lasted only 5–10 s, without loss of consciousness and occurred up to ten times daily. Between the attacks there were no abnormalities. Family history was non-contributory. Neurological examination and laboratory findings, including repeated EEGs, were all negative. The child responded very well to phenytoin treatment and has now been completely free of dystonic attacks for a period of 9 months. The importance of early recognition of this syndrome and the differential diagnosis with reflex epilepsy and other forms of dystonic choreoathetosis is discussed. |
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Keywords: | Paroxysmal disorder Choreoathetosis Dystonia Movement disorder |
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