Dados do Trabalho

Título

Profile Assessment of MoyaMoya Syndrome in patients from the Northeast: Case Series

Resumo

Introduction:
MoyaMoya syndrome is an uncommon cerebral vasculopathy worldwide and rare in South America, in which reduced blood flow in the internal carotid artery promotes compensatory angiogenesis. Known risk factors associated are systemic arterial hypertension, type 2 diabetes and Down Syndrome.
Objectives:
Describe clinical-epidemiologic profile of 7 patients with MoyaMoya Syndrome
Methods:
Report of seven cases of MoyaMoya Syndrome in a tertiary neurology department
Results:
In our series, all patients were female, in an age range of 18-49 years. All of them had sudden onset of focal neurological symptoms, which prompted the investigation.
Three patients had ischemic stroke, with varied Bamford Classification of anterior circulation syndrome. Four patients had hemorrhagic events, one with association of Subarachnoid Acute Hemorrhage (SAH) and Subdural Hemorrhage, one withTetraventricular Hemorrhage and two Frontal Lobar Intraparenchymal Hemorrhage. The angiographic study of the patients showed Suzuki-Takaku stages of 3 and 4. Two patients had neurosurgery indication, but one refused and another died in anesthesical procedure. Two patients had brain death because of hemorrhage. The other patients remain in neurologic department clinic follow-up.
Conclusion:
Moya Moya Syndrome is rare in our population, with high potential to provoke cerebrovascular events, with disabilities and death. There is low evidence of pharmacologic treatment, being surgery the main strategy of approach in these cases. Therefore, we recommend furthering studies of patogenesis, prognosis and treatment of this disease, to minimize complications.

Palavras Chave

MOYA-MOYA SYNDROME