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.
Sommario
La fisiopatologia della coreoatetosi parossistica kinesigenica (PKC) è controversa. Alcuni la classificano come un disturbo del movimento non epilettico, altri considerano la PKC come una forma di epilessia riflessa, ma ne postulano l'origine nei nuclei della base piuttosto che nella corteccia. Il monitoraggio prolungato con elettrodi intracranici in una ragazza con PKC ha dimostrato che la scarica critica inizia costantemente dall'area sensori-motoria supplementare, ma che una scarica concomitante è registrabile dal nucleo caudato ipsilaterale, senza significativa diffusione alle altre aree neocorticali. Viene presentata una ipotesi interpretativa di come una scarica focale nella corteccia motoria supplementare, dimostrata per la prima volta in un paziente con PKC, possa causare fenomenologie diverse da quelle delle tipiche crisi della SMC e che richiamano invece fortemente la semeiologia delle affezioni dei nuclei della base.
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Lombroso Cesare, T. Paroxysmal choreoathetosis: An epileptic or non-epileptic disorder?. Ital J Neuro Sci 16, 271–277 (1995). https://doi.org/10.1007/BF02249102
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DOI: https://doi.org/10.1007/BF02249102