1. Moyamoya Disease Clinical Course and Severity in Childhood
- Author
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Halil Donmez, Ayşe Kaçar Bayram, Sefer Kumandaş, Selim Doganay, Mehmet Canpolat, Hakan Gümüş, and Hüseyin Per
- Subjects
Moyamoya disease ,Pediatrics ,medicine.medical_specialty ,Clinical course ,business.industry ,lcsh:R ,Medicine ,lcsh:Medicine ,business ,Cerebrovascular disease - Abstract
Aim: Moyamoya disease (MMD) is a rare, progressive and oclusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries (ICA) and its main branches. The purpose of this study is to evaluate the clinical course and severity of MMD in pediatric patients. Material and Method: We examined 5 consecutive pediatric patients with MMD, focusing on clinical and radiological features, the therapy and outcome over the 58-month follow-up period. Results: The study population consisted of 3 boys and 2 girls. The mean age at diagnosis of patients was 7.2 ± 3.4 years (age range: 3-10 years). The mean duration of follow-up was 30.4 ± 17.4 months (follow-up interval: 12-58 months). Neurological findings at presentation included: motor deficit in 4 patients (80.0%), epileptic seizures in 2 patients (40.0%), movement disorders in 3 patients (60.0%), and headache in 1 patients (20.0%). There was areas of infarction on brain MRI in all patients. Angiographic findings included: internal carotid artery stenosis in all patients, anterior cerebral artery stenosis in 3 patients, middle cerebral artery stenosis in 3 patients, posterior cerebral artery stenosis in 2 patients, and vertebral artery stenosis in 1 patient. Enoxaparine therapy was started to all patients. Subdural hematoma developed in 1 patient during follow-up. Cerebral infarctions recurred despite medical treatment in 4 patients. Discussion: Although this disease is rare, it is an important cause of pediatric stroke. MMD shows different clinical course and disease severity in childhood. Early diagnosis and appropriate treatment are crucial.
- Published
- 2016