ORIGINAL
Introdução: A Síndrome de Moyamoya (SMM) é uma vasculopatia relacionada a autoimunidade que representa menos de 20% dos diagnósticos da Doença de Moyamoya. Sua patogênese ainda precisa ser melhor compreendida, e as opções de tratamento são limitadas. Objetivo: Avaliar a incidência de pacientes com Síndrome de Ehlers-Danlos Vascular Subjetivo (SEDv) e SMM, bem como suas características clínicas, manejo e resultados neurovasculares. Métodos: Um estudo observacional retrospectivo que analisa a base de dados de um serviço de Neurocirurgia do sul do Brasil (agosto de 2005 – dezembro de 2023). Resultados e Discussão: O estudo revela uma prevalência mais alta de casos de SEDv e SMM do que relatado na literatura. Estudos de imagem, como
Tomografia Computadorizada e Ressonância Magnética, são ferramentas valiosas para o diagnóstico precoce e estratificação de risco.
O tratamento se concentra na prevenção de acidentes vasculares cerebrais e no controle dos sintomas por meio de medicamentos e cirurgia endovascular em casos selecionados. Conclusão: Mais pesquisas são cruciais para desvendar os mecanismos subjacentes que ligam a SMM e a SEDv, a fim de melhorar a qualidade de vida e a expectativa de vida dos pacientes que enfrentam essas condições.
Introduction: Moyamoya Syndrome (MMS) is an autoimmune-related vasculopathy that accounts for less than 20% of Moyamoya Disease diagnoses. Its pathogenesis needs to be better understood, and treatment options are limited. Objective: Evaluate the incidence of patients with subjective vascular Ehlers-Danlos Syndrome (vEDS) and MMS and clinical characteristics, management, and neurovascular outcomes. Methods: A retrospective observational study analyzes a database from a Neurosurgery service in southern Brazil (August 2005 - December 2023). Results and Discussion: The study reveals a higher prevalence of vEDS and MMS cases than reported in the literature. Imaging studies such as Computed Tomography and Magnetic Resonance Imaging are valuable tools for early diagnosis and risk stratification. Treatment focuses on stroke prevention and symptom control through medication and endovascular surgery in selected cases. Conclusion: Further research is crucial to unravel the underlying mechanisms linking MMS and vEDS to improve the quality of life and life expectancy for patients facing these conditions.
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1Interventional Neuroradiology, Universidade Regional de Blumenau, Blumenau, SC, Brasil.
2Endovascular Neurosurgery, Hospital Santa Isabel, Blumenau, SC, Brasil.
3Universidade Regional de Blumenau, Blumenau, SC, Brasil.
4Neurosurgery Residency, Hospital Santa Isabel, Blumenau, SC, Brasil.
Received Oct 28, 2024
Corrected Jan 17, 2025
Accepted Jan 17, 2025
