Status epilepticus and venous infarction in Sturge-Weber syndrome.
Sturge-Weber syndrome (SWS) is a neurocutaneous disorder that is typically associated with progressive neurological deterioration. We describe a 12-year-old girl with SWS who suffered a permanent cerebral insult as the result of a period of protracted status epilepticus. The case illustrates the unique susceptibility of patients with SWS to uncontrolled venous hypertension and emphasises the need for optimal seizure control and preservation of venous outflow. We discuss the relevance of our observations to haemodynamic concepts of neurological decline in SWS.
Coley
S C
SC
Department of Neuroradiology, Atkinson Morley's Hospital, Wimbledon, London, UK.
Britton
J
J
Clarke
A
A
D000927:Anticonvulsants / Q000627:therapeutic use; D001921:Brain / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000473:pathology; D002533:Cerebral Angiography; D002648:Child; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007238:Infarction / Q000150:complications* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D013226:Status Epilepticus / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed; D014690:Venous Pressure / Q000502:physiology
D000927:Anticonvulsants