Paroxysmal choreoathetosis: an epileptic or non-epileptic disorder?
The pathophysiology of paroxysmal kinesigenic choreoathetosis (PKC) is controversial. Some classify it as a non-epileptic movement disorder, others consider PKC as a form of reflex-epilepsy but postulate that the epileptogenic source is within basal ganglia rather than in the cortex. An extensive invasive longterm monitoring in a girl with PKC demonstrated a consistent ictal discharge arising focally from the supplementary sensory-motor cortex (SMC), with a concomitant discharge recorded from the ipsilateral caudate nucleus, without significant spread to other neocortical areas. An hypothesis is presented to explain how a focal discharge within the supplementary motor cortex, demonstrated for the first time to occur in a patient with PKC, might cause phenomenologies distinct from the habitual SMC seizures and strongly suggesting a basal ganglia semeiology.
Department of Neurology, Children's Hospital, Boston, Massachusetts, USA.
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Lombroso
D000293:Adolescent; D001264:Athetosis / Q000145:classification* / Q000175:diagnosis; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D002819:Chorea / Q000145:classification* / Q000175:diagnosis; D004569:Electroencephalography; D004827:Epilepsy / Q000145:classification* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008991:Monitoring, Physiologic; D013690:Television; D015899:Tomography, Emission-Computed, Single-Photon