Clinical Features, Prothrombotic Risk Factors, and Long-Term Follow-Up of Eight Pediatric Moyamoya Patients.
- Author:
Burak TATLI
1
;
Baris EKICI
;
Altay SENCER
;
Serra SENCER
;
Kubilay AYDIN
;
Nur AYDINLI
;
Mine CALISKAN
;
Meral OZMEN
;
Talat KIRIS
Author Information
- Publication Type:Original Article
- Keywords: Moyamoya disease; prothrombotic risk factors; surgical revascularization
- MeSH: Down Syndrome; Follow-Up Studies; Humans; Moyamoya Disease; Neurofibromatosis 1; Paresis; Recurrence; Risk Factors; Thrombosis
- From:Journal of Clinical Neurology 2012;8(2):100-103
- CountryRepublic of Korea
- Language:English
- Abstract: BACKGROUND AND PURPOSE: The aim of this study was to elucidate the clinical features, prothrombotic risk factors, and outcome of pediatric Moyamoya patients. METHODS: Patients diagnosed with Moyamoya disease at a tertiary center between January 2000 and December 2006 were enrolled in this study. The clinical presentations, underlying diseases, prothrombotic risk factors, family history of thrombosis, radiological findings, treatment, and outcome of the patients were reviewed retrospectively. RESULTS: Eight patients with angiographically proven Moyamoya disease were identified, one of whom had neurofibromatosis type I and one had Down syndrome. The age at diagnosis varied between 19 months and 11 years (73.4+/-41.8 months, mean+/-SD). The follow-up period after diagnosis was 52.5+/-14.8 months. In six patients, the initial clinical presentation was hemiparesis. None of the patients had any identifiable prothrombotic factors. Despite medical and surgical treatment, three patients had recurrences and one died. Only two patients recovered without sequelae. CONCLUSIONS: The value of prothrombotic risk factor evaluation appears to be limited in Moyamoya patients; the outcome for pediatric patients remains dismal.
