Clinical features and outcomes of paediatric moyamoya vasculopathy in Malaysia
Document Type
Article
Publication Date
9-1-2022
Abstract
Moyamoya vasculopathy (MMV) is a chronic progressive occlusive cerebrovascular disease causing recurrent strokes in children. We describe the clinical features, treatment and outcomes of childhood MMV in Malaysia; and compared their neurological outcome with the published literature. Methods: A retrospective study between 2005-2020 of Malaysian children with MMV seen at 4 tertiary Malaysian hospitals. Their post-stroke outcomes were assessed using the modified Rankin scale (mRS) and Pediatric Stroke Outcome Measure (PSOM). Results: Twenty one cases were included with a median age of presentation at 5.8 years and a median follow-up period of 4.6 years. The female-to-male-ratio was 2:1. All patients had acute ischaemic cerebrovascular accidents. The anterior cerebral circulation was affected in all cases with additional three having also posterior circulation involvement. Among 17 (81%) patients neuroimaging showed bilateral moyamoya. Eight (30%) had preceding febrile illness and the commonest presenting symptom was hemiparesis seen in 8 (38%). Seven (35%) had surgical revascularisation whilst the remainder opted for conservative management. Nineteen (90%) achieved a good stroke outcome but 2 had a poor PSOM outcome and 1 also had a poor mRS outcome. Both these patients declined surgical revascularisation.Conclusion: Our cohort demonstrated the following MMV features: young age at the first presentation, female and Chinese preponderance; the main type of MMV was moyamoya disease; and ischaemic stroke as the commonest presentation. The overall neurological and functional outcomes were good despite the parental preference on medical therapy over surgical revascularization.
Keywords
Moyamoya, Children, Stroke, Outcome
Divisions
fac_med
Publication Title
Neurology Asia
Volume
27
Issue
3
Publisher
ASEAN Neurological Association
Publisher Location
UNIV MALAYA MEDICAL CENTRE, NEUROLOGY LABORATORY, KUALA LUMPUR, 59100, MALAYSIA