REVIEW
Este estudo visa mapear como fenótipos atípicos da hipertensão intracraniana idiopática, especialmente sexo masculino, índice de massa corporal normal e idade mais avançada, influenciam a apresentação clínica, os achados de neuroimagem, o risco visual e a necessidade de tratamento invasivo. Esta revisão de escopo foi conduzida de acordo com a metodologia do Joanna Briggs Institute e relatada conforme a diretriz PRISMA-ScR. Foi realizada busca abrangente em múltiplas bases de dados, sem restrição de idioma ou período. Quatorze estudos compuseram a síntese qualitativa final. O sexo masculino foi o subgrupo atípico mais consistentemente associado a maior gravidade visual na apresentação. Pacientes com índice de massa corporal normal e indivíduos mais velhos apresentaram perfil mais heterogêneo, incluindo papiledema grave, predomínio de manifestações visuais e atraso diagnóstico. A neuroimagem contribuiu para o suporte diagnóstico, mas nenhum padrão específico permitiu diferenciar de forma confiável esses subgrupos. A hipertensão intracraniana idiopática fora do perfil clássico representa uma manifestação legítima da doença e exige suspeição diagnóstica ativa. O reconhecimento precoce desses fenótipos é decisivo para reduzir atrasos terapêuticos e prevenir perda visual evitável.
This study aims to map how atypical phenotypes of idiopathic intracranial hypertension, particularly male sex, normal body mass index, and older age, influence clinical presentation, neuroimaging findings, visual risk, and the need for invasive treatment. This scoping review was conducted according to the Joanna Briggs Institute methodology and reported in accordance with PRISMA-ScR, based on a comprehensive search across multiple databases. Fourteen studies composed the final qualitative synthesis. Male sex was the atypical subgroup most consistently associated with greater visual severity at presentation, whereas patients with normal body mass index and older individuals showed a more heterogeneous profile, including severe papilledema, predominance of visual manifestations, and delayed diagnosis. Neuroimaging contributed to diagnostic support, but no specific pattern reliably distinguished these subgroups. Idiopathic intracranial hypertension outside the classical profile is a legitimate expression of the disease and requires active diagnostic suspicion, since early recognition is decisive to reduce therapeutic delay and prevent avoidable visual loss.
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1Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo – FMUSP, São Paulo, SP, Brazil.
2Fundação Oswaldo Cruz – FIOCRUZ, Rio de Janeiro, RJ, Brazil.
3Instituto de Neurologia de Curitiba – INC, Curitiba, PR, Brazil.
4Universidade Federal do Pará – UFPA, Belém, PA, Brazil.
Received Apr 13, 2026
Corrected May 7, 2026
Accepted May 9, 2026
