Neurosarcoidosis - Epidemiological, Clinical, Diagnostic, and Therapeutic Aspects: A Systematic Review.

introductionNeurosarcoidosis is a rare and severe manifestation of sarcoidosis, whose natural history still lacks a comprehensive and theoretical understanding. Therefore, we aimed to conduct a qualitative systematic review of the literature on the clinical-epidemiologic, diagnostic, and therapeutic assessment of patients with neurosarcoidosis.

methodsA qualitative systematic literature review was conducted in accordance with the PRISMA protocol. The search was conducted between January 1954 and December 2024 in the following databases: PubMed, Scopus, Web of Science, CAPES Journal Portal, and the Virtual Health Library (VHL). Articles were selected if they had at least one of the selected descriptors in the title or abstract, were written in English, Portuguese, or Spanish, and dealt with the clinical-epidemiologic, diagnostic, and therapeutic aspects of neurosarcoidosis. Review articles, experimental studies, and short communications were excluded from the analysis.

resultsSeventy-four articles with 551 cases of neurosarcoidosis were included. The majority of studies were case reports, followed by cohort studies conducted in European countries. The methodological quality of most studies was "good". DISCUSSION: Patients with neurosarcoidosis are primarily women Caucasian ethnicity with a mean age of 43.5 years. The disease most commonly involves the cranial nerves (especially cranial nerve II), followed by aseptic meningitis, panhypopituitarism, ocular changes, and diabetes insipidus. The most common laboratory findings were cerebrospinal fluid abnormalities, hypogonadotropic hypogonadism, hyperprolactinemia, and hypothyroidism. The most common neuroimaging changes were meningeal enhancement and pituitary destruction. The treatment of choice was glucocorticoids in conjunction with or without immunosuppressants, with methotrexate and azathioprine being the most commonly used.

conclusionThis review identified that manifestations such as cranial nerve alterations, aseptic meningitis, and pituitary infiltrations are frequent patterns in neurosarcoidosis, generally associated with laboratory and imaging findings that aid in the diagnosis. Systematizing these data provides a broad view of the disease and may contribute to early diagnosis and effective therapy. Nevertheless, longitudinal studies with larger samples are needed to better understand its natural history and clinical outcomes in the medium and long term.