Detailed Clinical and Electrophysiological Illustration of a Patient with Sturge–Weber Syndrome Presenting with Prolonged Transient Neurological Symptoms

Abstract

ABSTRACTAlthough transient neurological deficits in Sturge–Weber syndrome (SWS) have been acknowledged for many years, the underlying pathogenesis still constitutes a major topic of discussion. Here, we present the case of a 10-year-old boy with SWS presenting with a progressive clinically mimicking right middle cerebral artery syndrome. Cranial magnetic resonance imaging showed unremarkable findings except extensive right hemisphere leptomeningeal angiomas. Antiepileptic drug (AED) treatments provided recovery of the patient, and concurrently recorded electroencephalography (EEG) revealed delta slowing of the lesional hemisphere in the acute period. However, slowing of the background activity extended to the contralateral hemisphere in subacute period, while ipsilateral background activity started to recover. In the 3rd week of follow-up on intensive AED treatments, the patient totally recovered as well as nearly the normalization of EEG was achieved. In this report, we focus on the dramatic revolution of EEG activity in both hemispheres during the recovery period. Based on this rare illustration and limited literature data, the author suggests some hypotheses regarding the pathophysiology of this mysterious manifestation (transient neurological deficit) in SWS. Future studies of large case series, including detailed paraclinical parameters and remarkably electrophysiological data, are warranted to clarify these arguments.