PP04.11 – 3037: Moyamoya in children

Abstract

Objective Moyamoya is progressive occlusive cerebral arteriopathy of unknown etiology, a common cause of cerebral ischemic stroke in children. It is characterized by progressive constriction (and finally, occlusion) of the terminal part of internal carotid artery and proximal parts of the middle and anterior cerebral arteries, resulting in collateral circulation in the thalamus and basal ganglia. Blood vessels, representing collateral flow in areas of the brain hypoperfusion distal to the stenosis, on the DSA look like “cigarette smoke”, which in Japanese is called “moyamoya”. The highest incidence of the disease was found in Japan. Methods In this paper, we will present nine patients, five boys and four girls, different age (2–10 years, peak 5–6 years) and with different clinical picture (transient hemiparesis in 5/9 children, convulsions in 4/9 children, headaches in 2/9 and dizziness in 1 child), admitted to our clinic. Results By processing (Transcranial color doppler, MRI/MRA and DSA) in all progressive occlusive cerebrovascular arteriopathy was proven (Moyamoya disease in 6/9 and Moyamoya syndrome in 3/9 children). Six of the nine patients underwent neurosurgical revascularization with variable outcome. One patient in young age had extremely progressive course of the disease, developing tetraparesis, epilepsy, blindness, motor aphasia and dysphagia, renal failure and death. Other patients had a better outcome: 3/6 operated have orderly development, and the two have mild cognitive deterioration. Conclusion Regardless differences between idiopathic and syndromal (associated) forms, angiographic characteristics, clinical features and outcome are the same. Unfavorable outcome have small children with bilateral ischemic infarcts and fixed neurologic deficit. The clinical presentation and neurological status at the moment of diagnosis are most important prognostic factors and predictors of long term outcome. It is important to make a diagnosis on time and prevent serious permanent neurological damage.