Paroxysmal choreoathetosis: an epileptic or non-epileptic disorder?

Ital J Neurol Sci. 1995 Jun;16(5):271-7. doi: 10.1007/BF02249102.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Athetosis / classification*
  • Athetosis / diagnosis
  • Brain / diagnostic imaging
  • Brain / physiopathology
  • Chorea / classification*
  • Chorea / diagnosis
  • Electroencephalography
  • Epilepsy / classification*
  • Epilepsy / physiopathology
  • Female
  • Humans
  • Monitoring, Physiologic
  • Television
  • Tomography, Emission-Computed, Single-Photon