Neuropediatrics 2006; 37 - TP66
DOI: 10.1055/s-2006-945659

CLINICAL AND RADIOLOGICAL FINDINGS OF MOYA MOYA DISEASE AND SYNDROME IN SAUDI PATIENTS

A Mokeem 1, E Al-Shail 1, S Al-Yamani 1
  • 1Division of Neurosurgery, Department of Neurosciences, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia

Objectives: To study clinical feature, radiological findings Magnetic Resonance Imaging (MRI) of brain, Magnetic Resonance Angiography (MRA), Angiogram results and surgical outcome of 13 Saudi patients with MoyaMoya disease and syndrome.

Methods: Thirteen charts were reviewed for 13 patients with MoyaMoya disease and syndrome and the following information was obtained: age, gender, seizure, stroke, transient ischemic attach (TIA), underlying etiology, MRI, MRA, Angiogram findings, as well as treatment that patients received either medical or surgical, type of surgery and the outcome results.

Results: Out of 13 patients, 8 were male and 5 were female. The age of onset varied between 18 months and 20 years. The mean age of onset was 12.5 years.

The most common presentation was stroke which was reported among all 13 patients, while seizure was reported in 9 out of 13. Bleeding was seen in one patient. All 13 patients had neurological deficit. The etiology was identified in 7 out of 13. The most common cause was hemitalogical disorder. All 13 patients had findings of ischemic infarct in the MRI brain. Only one patient had ischemic infarct plus haemorrhage at the same time.

Nine of the 13 patients had MRA. All of the 9 patients had Internal Carotid Artery (ICA) stenosis, 7 out of 9 in addition had Middle Cerebral Artery (MCA) stenosis, and only 2 patients of the 9 had in addition Posteral Cerebral Artery (PCA) stenosis.

Eight of the patients had Angiogram; all of them showed collateral.

Seven of the 13 patients had medical treatment. Five patients received aspirin, 1 received Plavix, 1 received Flunarizine.

Eleven out of the 13 patients had surgery: 9 of them had bilateral encephalo-duro-arterio-synangiosis (EDAS), 2 of them had in addition multiple burr-hole, one of the 11 patients has right-sided EDAS.

Eight of the 11 patients who had seizures became seizure-free, 5 of them had no stroke recurrence, one of the 8 patients developed TIA, and for two patients there was no data.

One out of the 11 patients had more than 50% seizure reduction, one patient had seizure reduction between 30–50%.

Conclusion: MoyaMoya disease or syndrome is not a rare disease in Saudi children. The most common underlying etiology is blood discresis. The most common presentation was ichemic stroke and seizure. The result of surgical treatment in our patients is encouraging as it prevented further stroke in most of the cases and had a positive effect on seizure by seizure remmission or reduction.