Reversible Cerebral Vasoconstriction Syndrome And Fibromuscular Dysplasia: An Epiphenomenon Or A Causal Relationship?

Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a nonatheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months. While most RCVS cases have identifiable triggers, a significant portion occurs without known causes. Recent studies have reported a high prevalence of neurovascular abnormalities in RCVS patients. We present a case of a thirty-year-old woman with a sudden-onset severe headache, diagnosed with RCVS associated with carotid web. The patient had no ischemic involvement and responded well to flunarizine treatment. Follow-up imaging showed no stenosis. This case highlights a potential association between carotid web and RCVS, suggesting that FMD may contribute to vascular hyperreactivity and presents as a risk factor for RCVS. Further investigations are needed to understand the underlying mechanisms connecting these two vascular disorders. Keywords: reversible vasoconstriction syndrome; fibromuscular dysplasia; carotid web; structural abnormalities; vascular hyperreactivity.

Neuropsychological aspects of reversible cerebral vasoconstriction syndrome / Aspectos neuropsicológicos da síndrome de vasoconstrição cerebral reversível

ABSTRACT. Despite the increasing knowledge in the past years, only minimal attention has been directed to the neuropsychological aspects and the prevalence of cognitive impairment associated with reversible cerebral vasoconstriction syndrome (RCVS). Objective: To describe the frequency and expand the understanding of cognitive dysfunction in RCVS. Methods: The neuropsychological evaluation was performed using a battery consisting of specific neuropsychological instruments that were administered to patients diagnosed with RCVS. A triage was conducted to exclude other potential causes of cognitive impairment. Performance on the tests was treated as a categorical variable, and a cutoff of −1.5 Z-score was adopted to indicate impaired performance. Results: Seven patients diagnosed with RCVS were evaluated, all of whom had a bachelor's degree and normal score in the Mini-Mental State Examination. The average time between diagnosis and neuropsychological evaluation was 1.8 years. Among the patients, 85.6% (n=6) exhibited performance below that of the normal population in at least two of the administered tests. Specifically, 71.4% (n=5) showed alterations in tests from the Psychological Battery for Attention Assessment, with impairment observed in concentrated (n=1), divided (n=3), or alternating (n=4) attention. Furthermore, 28.6% (n=2) demonstrated impairments in the Phonological Verbal Fluency Task, another 28.6% (n=2) exhibited difficulties copying elements of the Rey Complex Figure, and 14.3% (n=1) displayed lower performance in the Five-Digit test, all indicating executive dysfunction. Conclusion: This study provides evidence that cognitive impairment associated with RCVS is more prevalent than previously believed and has not received sufficient attention. Specifically, attention and executive functions are the cognitive domains most significantly impacted by RCVS.

RESUMO. Apesar do crescente conhecimento nos últimos anos, pouca atenção tem sido direcionada aos aspectos neuropsicológicos e à prevalência de declínio cognitivo associado à Síndrome de Vasoconstrição Cerebral Reversível (SVCR). Objetivo: Descrever a frequência e expandir o entendimento da disfunção cognitiva associada à SVCR. Métodos: A avaliação neuropsicológica foi realizada por meio de uma bateria composta de instrumentos neuropsicológicos específicos, que foram aplicados aos pacientes com SVCR. Uma triagem foi realizada para excluir outras potenciais causas de declínio cognitivo. O desempenho nos testes foi considerado como variável categórica, e o corte de −1,5 escore z foi adotado para indicar desempenho comprometido. Resultados: Sete pacientes com diagnóstico de SVCR foram avaliados, os quais tinham todos nível de ensino superior e pontuação normal no Miniexame do Estado Mental. A média de tempo entre o diagnóstico e a avaliação neuropsicológica foi de 1,8 ano. Dentre os pacientes, 85,6%(n=6) apresentaram desempenho comprometido em pelo menos dois dos testes aplicados. Especificamente, 71,4% (n=5) apresentaram alterações nos testes da Bateria Psicológica para Avaliação da Atenção, com comprometimento observado nas atenções concentrada (n=1), dividida (n=3), ou alternada (n=4). Além disso, 28,6% (n=2) demonstraram comprometimento na Fluência Verbal Fonológica, 28,6% (n=2) exibiram dificuldades na cópia da Figura Complexa de Rey e um paciente obteve desempenho alterado no Teste dos Cinco Dígitos, todos indicando disfunção executiva. Conclusão: Este estudo fornece evidência de que o declínio cognitivo associado à SVCR é mais prevalente do que se acreditava anteriormente. Especialmente, os domínios mais significativamente comprometidos foram a atenção e as funções executivas.