Abstract
Objective Characterization of clinical symptoms and disease course in children with Moyamoya angiopathy. Methods Between 2011 and 2014 34 children and adolescents were treated in the Moyamoya Center in Zurich. Evaluation comprised a detailed history, neurologic and developmental status including pediatric stroke outcome measure (PSOM). Cerebral MRI and MR angiography (MRA), conventional cerebral angiography and H215O-PET with acetazolamid challenge were performed followed by individualized treatment. Results Median age at symptom onset was 4 1/2 years (range 6 months–14 years). We found a female preponderance (female/male ratio 1.8/1). Diagnosis was Moyamoya disease in 18 and Moyamoya syndrome in 16 children. 5/16 Moyamoya syndromic children had Neurofibromatosis Type 1. Most but not all children presented with a transitory ischemic attack (TIA) or stroke. In 11 children headaches and migraine were the main complaint. Besides weakness and focal sensory deficits headache was often accompanied by peculiar and unspecific symptoms like short episodes of altered consciousness, involuntary movements, general weakness and falls. On presentation MRI revealed ischemic lesions in 21 cases. Cortical lesions were observed in 11 patients (mean age 2 years), subcortical in 10 patients (mean age 6 1/2 years). In 6 children under 3 years we found a rapid progressive angiopathy. Posterior circulation was involved in 10 patients, all of them were younger than 6 years. PSOM score tended to be higher in young children. Conclusion Early diagnosis and surgical revascularisation are mandatory for stroke prevention in Moyamoya angiopathy, especially in young toddlers. In school aged children with migraines or chronic headaches accompanied by weakness, focal sensory deficits, altered consciousness or involuntary movements it is worthwhile to consider Moyamoya angiopathy as an important differential in the diagnostic assessment.
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