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Early single photon emission computed tomography in Sturge-Weber syndrome
F Pinton1, C Chiron, O Enjolras
1Hospital Frédéric Joliot, Departmentt of Medical Research, Atomic Energy Committee, Orsay, France.
Insights
Sturge-Weber syndrome in infants shows altered cerebral blood flow (CBF) before seizures. The affected brain area is initially hyperperfused, shifting to hypoperfusion after one year, even without epilepsy.
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Malformations
Background:
- Sturge-Weber syndrome (SWS) is a rare neurological disorder.
- Previous studies show hypoperfusion in SWS with epilepsy.
- Limited data exists for infants and non-epileptic SWS patients.
Purpose of the Study:
- To investigate cerebral blood flow (CBF) patterns in infants with SWS.
- To examine perfusion changes before and after seizure onset.
- To include SWS patients without epilepsy.
Main Methods:
- SPECT imaging with xenon-133 in 22 infants with SWS (aged 8 days to 25 months).
- CBF measurement in affected hemispheres and vascular malformations.
- Comparison of CBF with age-matched controls; follow-up SPECT in some patients.
Main Results:
- Infants without seizures showed significantly increased CBF in affected areas (75%).
- Patients with prior seizures exhibited significantly decreased CBF.
- Follow-up SPECT revealed decreased CBF in all patients, including non-epileptic ones.
Conclusions:
- SPECT reveals age-dependent CBF asymmetry in infants with SWS.
- Hyperperfusion precedes seizures, followed by hypoperfusion after one year.
- Hypoperfusion occurs in SWS regardless of epilepsy status.
Objectives:
Functional cerebral imaging PET and SPECT have shown hypometabolism and hypoperfusion in the area of vascular malformation in children with epilepsy due to Sturge-Weber syndrome. However, data are scarce in infants and do not exist in patients with Sturge-Weber disease without epilepsy. The pattern of perfusion during the first two years of life was studied including patients before the onset of seizures.
Methods:
Twenty two infants with later confirmed Sturge-Weber disease underwent SPECT examination using TOMOMATIC 564 (Medimatic) and xenon-133 at ages ranging from 8 days to 25 months. Twelve had never had seizures before SPECT and seven underwent a second SPECT a mean seven months later. Cerebral blood flow (CBF) was measured in the whole hemisphere and in the part of the cortex involved in the vascular malformation on both sides as well as a "pathological to normal" index for the hemisphere and vascular malformation. These values were compared with normal age paired values.
Results:
Compared with controls, CBF and the indices in the hemisphere and vascular malformation were significantly decreased in patients who already had had seizures before SPECT, whereas they were significantly increased in 75% of the patients who had never had any seizures. On second SPECT, the indices were decreased in all patients, including the four who still remained non-epileptic.
Conclusions:
SPECT therefore detects CBF asymmetry in infants with Sturge-Weber disease, which tends to shift with age. The cortex involved in the vascular malformation is hyperperfused during the first year of life before first seizures. The classic hypoperfusion appears after one year of age, even in non-epileptic patients.