Sturge–Weber syndrome: cerebral haemodynamics during seizure activity

Abstract

The aim of this study was to examine the haemodynamic response to seizures in three infants with Sturge–Weber syndrome by measuring regional cerebral blood flow using transcranial Doppler sonography and 99mTc HMPAO SPECT. Time‐locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (–18 and –43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

“…Some of the literature in this area suggests that active seizures promote tissue damage in the affected and non-affected hemisphere that further contribute to cognitive decline and maintenance of seizures. [6][7][8] However the present case shows that despite early and aggressive treatment, cognitive decline was still observed and that seizures can appear de novo in an apparently unaffected hemisphere.…”

“…However, it should be noted that these progressive changes were seen in patients that did not have their leptomeningeal angiomas removed, either through lobectomy or hemispherectomy. In the cases described above, it is possible that the seizures lead to changes in blood flow and metabolism which may have contributed to progression of the structural lesions, as previously suggested 6,7 .…”

“…Proponents of early surgery argue that unilateral seizures may induce bilateral blood flow and metabolic changes that cause progressive damage in SWS. 6,7 It is postulated that these changes may lead to neuronal damage ultimately resulting in cognitive deterioration. 8 This is corroborated by the fact that seizure severity, 9 early onset of seizures and multiple seizure types may predict poor cognitive outcome.…”

Transient exacerbation of hemiplegia following minor head trauma in Sturge–Weber syndrome

“…This compromises an already ischaemic cortex, leading to increased focal deficit and deteriorating cognition. 11 An improvement in seizure control could be followed by improvement in focal deficit, or at least, prevent further events, and so aggressive control of seizures in SWS is advocated.…”

Ictal SPECT in Sturge-Weber syndrome

“…Right orbito-frontal resection and disconnection from corpus callosum resulted in seizure freedom. © 2007 Elsevier B.V. All rights reserved.Sturge-Weber syndrome (SWS) is a sporadically occurring phakomatosis (Juhasz et al, 2007), which frequently leads to epilepsy, hemiparesis, and learning disability in association with a pial angioma (Aylett et al, 1999). Epilepsy affects most of these patients who may be good candidates for hemispherectomy if anti-epileptic medication does not control the seizures (Kossoff et al, 2002).…”