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Moyamoya disease. Report of three cases in Brazilian patients
C M Franco1, M M Fukujima, R de M de Oliveira
1Department of Neurology and Neurosurgery, Universidade Federal de São Paulo-Escola Paulista de Medicina (UNIFESP-EPM), Brazil. clelia@unisys.com.br
Abstract:
Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease of unknown etiology reported mainly in the Japanese. Most cases occur in children. The disease is rare in non-Oriental adults manifesting itself mostly as intracerebral hemorrhages. We describe MMD in 2 non-Oriental young adults and one adolescent that developed cerebral infarctions. The adults were medicated with aspirin and no medication was given to the adolescent. All patients did not deteriorate in a follow-up period from 1 to 4 years. Although rare, MMD is an important cause of stroke in young individuals and may well be underreported: only 18 patients have been reported till 1997 in Brazil. Neurologists should include MMD in differential diagnosis of ischemic and hemorrhagic strokes in young adults.
Insights
Moyamoya disease (MMD), a rare cerebrovascular condition, can affect young adults and adolescents, presenting with ischemic strokes. Early diagnosis and management are crucial for preventing further neurological deterioration.
Area of Science:
- Neurology
- Vascular Neurology
- Cerebrovascular Diseases
Background:
- Moyamoya disease (MMD) is a rare, chronic occlusive cerebrovascular disorder primarily reported in Japanese populations, predominantly affecting children.
- In non-Oriental adults, MMD is uncommon and typically manifests as intracerebral hemorrhages.
Observation:
- This report details three cases of MMD in non-Oriental individuals: two young adults and one adolescent.
- All three patients presented with cerebral infarctions, a less common manifestation in this demographic.
- The adult patients received aspirin treatment, while the adolescent received no medication.
Findings:
- Follow-up over 1 to 4 years revealed no deterioration in any of the patients.
- This suggests that MMD, even in non-Oriental young individuals presenting with ischemic events, may have a stable or manageable clinical course with appropriate monitoring or treatment.
Implications:
- Moyamoya disease is an underrecognized cause of stroke in young individuals, potentially underreported globally, including in regions like Brazil.
- Neurologists should consider MMD in the differential diagnosis for both ischemic and hemorrhagic strokes occurring in young adults.
- Further research is warranted to understand the epidemiology and optimal management strategies for MMD in diverse populations.