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.

Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil.

BACKGROUND: Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by progressive deposition of ß-amyloid peptides in the walls of small and medium-sized cortical and leptomeningeal vessels. Until today, the prevalence of CAA is unknown in our region. OBJECTIVE: This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. METHODS: A cross-sectional, retrospective study with the enrollment of patients aged 65 or older followed in the neurological outpatient service of the Universidade Federal do Piauí, Brazil, who underwent brain magnetic resonance imaging (MRI) from July 2016 to June 2018. RESULTS: One hundred and seventy-four patients were enrolled, of whom 100 were women (57.4%) and 74, men (42.6%), aged from 65 to 91 years old (median age 73.27). Nine patients were excluded from the study due to unavailability of MRI sequences needed for an appropriate analysis. Out of the 165 remaining patients, 12 (7.2%) had established the diagnosis of CAA, according to the modified Boston criteria. CONCLUSION: The prevalence of CAA in our study was like those of medical literature, with a progressive age-related increase.

Cerebral amyloid angiopathy: a cross-sectional study in a single center in Northeastern Brazil / Angiopatia amiloide cerebral: um estudo transversal em um único centro no Nordeste brasileiro

ABSTRACT Background: Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by progressive deposition of β-amyloid peptides in the walls of small and medium-sized cortical and leptomeningeal vessels. Until today, the prevalence of CAA is unknown in our region. Objective: This study aims to analyze the prevalence of this entity in a specific elderly population in a tertiary hospital in Northeastern Brazil. Methods: A cross-sectional, retrospective study with the enrollment of patients aged 65 or older followed in the neurological outpatient service of the Universidade Federal do Piauí, Brazil, who underwent brain magnetic resonance imaging (MRI) from July 2016 to June 2018. Results: One hundred and seventy-four patients were enrolled, of whom 100 were women (57.4%) and 74, men (42.6%), aged from 65 to 91 years old (median age 73.27). Nine patients were excluded from the study due to unavailability of MRI sequences needed for an appropriate analysis. Out of the 165 remaining patients, 12 (7.2%) had established the diagnosis of CAA, according to the modified Boston criteria. Conclusion: The prevalence of CAA in our study was like those of medical literature, with a progressive age-related increase.

RESUMO Introdução: A angiopatia amiloide cerebral (AAC) é uma desordem vascular causada pela deposição progressiva de peptídeos β-amiloides nas paredes de pequenos e médios vasos corticais e leptomeníngeos. Até a presente data, a epidemiologia da AAC é desconhecida em nossa região. Objetivos: Avaliar a prevalência da AAC em uma população específica de pacientes idosos de um hospital terciário no nordeste brasileiro. Métodos: Estudo transversal, retrospectivo, com seleção de pacientes com idade igual ou superior a 65 anos, acompanhados no serviço de Neurologia do Hospital Universitário da Universidade Federal do Piauí, Brasil, e que foram submetidos a exame de ressonância nuclear magnética entre julho de 2016 e junho de 2018. Resultados: Foram recrutados 174 pacientes, dos quais 100 eram mulheres (57,4%) e 74 homens (42,6%), com idades entre 65 e 91 anos (média de 73,27). Nove pacientes foram excluídos devido à indisponibilidade de sequências de ressonância magnética necessárias para uma análise apropriada. Dos 165 pacientes restantes, 12 (7,2%) foram diagnosticados com AAC de acordo com os critérios de Boston modificados. Conclusão: A prevalência da AAC em nosso estudo foi semelhante ao resultado encontrado na literatura médica, com um aumento progressivo relacionado à idade.