Epilepsia Partialis Continua

Excerpt

Epilepsia partialis continua (EPC) is a rare brain disorder where a patient experiences recurrent and, sometimes, unrelenting focal onset seizures with retained awareness occurring over hours, days, or even years. The pathophysiology of the disease is understood to be complicated. The disease can manifest in a variety of forms such as myoclonic epilepsy, localized myoclonus, Jacksonian epilepsy, and sensorimotor clonic seizures to name a few. Both cortical and subcortical mechanisms have been identified as an etiology. The localized nature of the seizures along with preserved consciousness and postictal confusion and weakness (Todd paralysis) are understood to be characteristic of the disorder but may not be considered pathognomonic due to recorded exceptions. Treatment is centered on a combination of anti-epileptic medications coupled with the identification and resolution of the underlying cause.

The disorder was first described in 1894 by the legendary Russian neurologist Aleksei Kozhevnikov (1836-1902). The eponym Kozhevnikov epilepsy was coined as a tribute to his work. He postulated that the seizures in EPC were due to a form of focal cortical encephalitis and the subsequent inflammation of cerebral motor fibers. Several years later, in 1958, Rasmussen syndrome (chronic focal encephalitis) was formally described in which patients with the syndrome presented with seizures like that of EPC. Over the years, several neuroscientists have offered different explanations and interpretations for the possible pathophysiological mechanisms.