Behavioral Manifestations of Sturge-Weber Syndrome: A Case Report

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Sturge-Weber syndrome is a neurocutaneous syndrome that manifests with vascular malformations involving the brain, eye, and skin. We report the case of an elderly patient suffering from Sturge-Weber syndrome who presented with episodic "angry" slapping symptoms to a psychiatric facility. A detailed history, physical and mental-state examination, and elaborate neuropsychological, neuroimaging, and laboratory assessment were undertaken. Clinically, the "angry" slapping episodes were diagnosed as complex partial seizures that improved in frequency with an increase in the dose of the antiepileptic medication. We also have attempted to identify the pathophysiology of such behavioral episodes in Sturge-Weber syndrome. This case report underlines the need for detailed laboratory and neuroimaging work-up in the elderly presenting with atypical symptoms. It also emphasizes the need for identification of and differentiation between similar atypical presentations and appropriate management of resources by the medical staff.

Prim Care Companion J Clin Psychiatry 2006;8(4):198-200