Ictal Priapism as an Autonomic Manifestation of Panayiotopoulos Syndrome

Abstract

Panayiotopoulos syndrome is an idiopathic epilepsy syndrome presenting with a large variety of autonomic symptoms. The mechanism of autonomic symptoms is still not well understood. A neurologically normal 13-month-old boy presented on 2 occasions with complex partial status epilepticus that included significant ictal priapism. Inpatient brain magnetic resonance imaging (MRI) scan showed restricted diffusion-weighted imaging within his left temporal lobe and electroencephalograms (EEGs) left temporal slowing only. An outpatient EEG 6 months later showed abundant multifocal, predominantly posterior, as well as irregular generalized spike-and-slow-wave discharges on normal background consistent with the diagnosis of Panayiotopoulos syndrome. Ictal priapism is a previously undescribed phenomenon that is consistent with parasympathetic manifestations noted in this young boy. Acute postictal MRI and EEG findings suggest that this undeniably fascinating seizure semiological sign may be localized to the left temporal region.