Epilepsia partialis continua: what lies beneath?

Background: Epilepsia partialis continua (EPC) is a variant of simple focal motor status epilepticus, in which frequent repetitive muscle jerks continue over prolonged time periods. There are several aetiologies, being Rasmussen’s encephalitis (RE) the most common cause of EPC in children. RE is a rare and severe progressive disorder of unilateral brain dysfunction, focal seizures and inflammatory histopathology, with an uncertain, but probably immune-mediated etiopathology.

Case report: An 8-year-old girl, with unremarkable past medical and family history, was referred to the Neuropaediatrics clinic because of right hemifacial abnormal movements without loss of consciousness, for the last 3 months. Her physical exam showed repetitive clonic movements affecting the right hemiface, including tongue and soft palate on the right, and orbicularis oculi muscle. Blood tests, lumbar puncture and the initial brain-MRI were normal. The first video-EEG revealed abundant focal paroxysmal activity in the left frontocentral region frequently associated to clonic movements in the right hemiface. She was started on antiepileptic drugs, but there was a progressive clinical and electrophysiolgical worsening, with almost continual paroxysmal activity, despite the several drugs used. Immunosupression was initiated with systemic corticotherapy and intravenous immunoglobulin infusions, with reasonable seizure control. Serial brain-MRI evaluation revealed signal changes in left cortical and sub-cortical areas in the precentral gyrus, as well as cortical atrophy. Clinical, neurophysiologic and brain imaging data suggest the diagnosis of RE.

Conclusion: For the general pediatrician, the early recognition of an EPC is crucial, since it generally has a complex etiological diagnosis, whose functional prognosis can be very difficult. Even so, the early initiation of a targeted therapeutic intervention may help to address the potentially disabling effects of EPC.