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[Moyamoya and Down syndrome: study conducted by meta-analysis]
Paulo Alves Junqueira1, Maria Valeriana L de Moura-Ribeiro
1Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, SP, Brasil. ppaj@terra.com.br
Arquivos De Neuro-Psiquiatria
|June 18, 2002
Summary
Down syndrome (DS) patients experiencing hemiparesis should be evaluated for moyamoya syndrome (MMS). This meta-analysis highlights MMS as a probable diagnosis in DS patients with acute neurological events.
Area of Science:
- Neurology
- Genetics
- Epidemiology
Background:
- Down syndrome (DS) is a genetic disorder associated with various health complications.
- Moyamoya syndrome (MMS) is a rare cerebrovascular disease characterized by progressive stenosis of intracranial arteries.
- The co-morbidity of DS and MMS requires further investigation due to potential overlapping clinical manifestations.
Observation:
- A meta-analysis of 42 patients (1977-2000) identified publications predominantly from Japan, the United States, Brazil, and Italy.
- Cerebrovascular disease prevalence was noted in infants and preschool children with DS.
- The primary symptoms observed were hemiparesis (78.6%) and speech disorders (26.2%).
Findings:
- Ischemic infarction occurred in 76.2% of cases.
- Recurring ischemic episodes were reported in 62% of patients.
- Bilateral impairment was a significant finding in 83.3% of individuals.
Implications:
- Moyamoya syndrome should be considered in the differential diagnosis for Down syndrome patients presenting with acute hemiparesis.
- Early recognition and diagnosis of MMS in DS patients can lead to timely intervention.
- This study underscores the importance of a comprehensive neurological evaluation in individuals with Down syndrome.