Regional cerebral blood flow by SPECT imaging in Sturge-Weber disease: an aid for diagnosis.

Abstract

SUMMARY Regional cerebral blood flow (rCBF) was studied using SPECT (single photon emission computed tomography) with 133-Xenon in 13 patients with confirmed Sturge-Weber disease, aged 9 months to 18 years. CT scan, performed at the same time, showed evident cerebral angioma in 10 but not in three. A marked hypoperfused area was found in all patients, ranging from -32% to -72% and of the same location as the CT signs. The hypoperfusion seems to result from post ictal phenomenon as well as from chronic ischaemia. SPECT imaging is therefore a sensitive method for visualising intracranial angioma in Sturge-Weber disease and it provides an aid for diagnosis when a CT scan is not reliable.Sturge-Weber (SW) angiomatosis is a non-inherited neurocutaneous syndrome characterised by a congenital trigeminal port-wine stain and a piamater angioma.' This intracranial angioma, venous and purely meningeal,23 is embryologically derived from the neural crest. Along with the angioma, cerebral lesions, which are ischaemic in nature, are seen in the cortex underlying the angioma.4

Sturge–Weber syndrome: cerebral haemodynamics during seizure activity

“…Previous studies have shown hypoperfusion (Riela et al 1985, Chiron et al 1989, and hypometabolism (Chugani et al 1989) in affected hemispheres, often over a wider area than that obviously affected by the angioma. Bilateral hypometabolism appears to be associated with learning disability even with a unilateral pial nevus (Chugani et al 1989).…”

The Natural History of Patients with the Sturge-Weber Syndrome

“…metabolic analysis with positron emission tomogra phy (PET) [8] and single photon emission computed tomography (SPECT) has been reported [7], All of these imaging techniques are methods used to help identify the extent of the angiomatosis and. therefore, the area which could be ablated surgically.…”

The Role of Radionuclide Imaging in Epilepsy, Part 2: Epilepsy Syndromes

“…Perfusion SPECT, using 133 Xe, shows hyperperfusion in the lesion even before seizure onset (67), analogous to the transient hypermetabolism seen on 18 F-FDG PET (60,61). After the age of 1 y, these areas typically show hypoperfusion, as revealed by another 133 Xe SPECT study (68). In a single case report on a patient with failed functional hemispherectomy, ictal ethylcysteinate dimer SPECT showed hyperperfusion in the residual lesion with falsely lateralized EEG; further surgery resulted in freedom from seizures (69).…”