Status epilepticus and venous infarction in Sturge-Weber syndrome

Abstract

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.

Physiatric Findings in Individuals with Sturge-Weber Syndrome

“…Individuals with SWS are prone to significant functional setbacks, often associated with seizure activity, as noted previously by a number of authors. 1,3,6,15,16 One hypothesis for this phenomenon is that impaired cerebral autoregulation in individuals with SWS during seizure activity results in inadequate delivery of oxygen and glucose to meet the metabolic demand, thereby increasing the risk of ischemic injury. 1 The physiatrist must understand the importance of seizure control in optimizing overall function in this population.…”

Anaesthesia for caesarean section in a patient with Sturge–Weber syndrome following acute neurological deterioration

“…lar abnormalities typically in the leptomeninges. 1,11,12 Our selection of lumbar epidural anaesthesia was also influenced by the presence of cerebral oedema on neuroimaging. Diminished blood flow in angiomas predisposes to venous stasis, which in turn impairs arterial flow and may result in chronic tissue hypoxia.…”

A Spectrum of Unusual Neuroimaging Findings in Patients with Suspected Sturge-Weber Syndrome

“…24 It is proposed that repetitive or prolonged seizure activity produces permeability changes in the blood-brain barrier. 26 Indeed, several studies describe cases of SWS in which cerebral leptomeningeal enhancement transiently increased immediately following seizure activity, [27][28][29] sometimes with complete resolution on subsequent imaging. 29 It is possible that subtle pial angiomas only become evident on conventional contrast-enhanced imaging once the seizure activity or venous ischemia within the underlying cortex results in sufficient disturbance of the blood-brain barrier or venous stasis within the angioma to unmask its presence.…”