Aseptic meningitis with recurrent headache episodes, vomiting, and central fever as first manifestation of isolated neurosarcoidosis: a case report

• Published in: BMC neurology 2024-08, Vol.24 (1), p.299-5, Article 299
• Main Authors: Aloizou, Athina-Maria, Gabriel, Theresa Anne, Lukas, Carsten, Gold, Ralf, Motte, Jeremias
• Format: Article
• Language: English
• Subjects: Adult; Angiography; Antibodies; Aseptic meningitis; Autoantibodies; Autoimmune diseases; Biopsy; Case Report; Case reports; Central Nervous System Diseases - complications; Central Nervous System Diseases - diagnosis; Central Nervous System Diseases - drug therapy; Cerebrospinal fluid; Corticosteroids; Dengue fever; Diagnostic tests; Diseases; Emergency medical care; Enzymes; Fever - diagnosis; Fever - drug therapy; Fever - etiology; Food consumption; Gadolinium; Granulomas; Headache; Headache - diagnosis; Headache - drug therapy; Headache - etiology; Headaches; Health aspects; Humans; Interleukin 2 receptors; Lungs; Meningitis; Meningitis, Aseptic - diagnosis; Meningitis, Aseptic - drug therapy; Meningitis, Aseptic - etiology; Neopterin; Nervous system; Nervous system diseases; Neuroimmunology; Neurological diseases; Neurosarcoidosis; Pathology; Patients; Pleocytosis; Relapse; Sarcoidosis; Sarcoidosis - complications; Sarcoidosis - diagnosis; Sarcoidosis - drug therapy; Skull; Spine (cervical); Spine (lumbar); Steroids; Stroke (Disease); Subarachnoid hemorrhage; Tropical diseases; Vasculitis; Vomiting; Vomiting - etiology; West Nile virus; β2 Microglobulin
• ISSN: 1471-2377; 1471-2377
• DOI: 10.1186/s12883-024-03794-x

Neurosarcoidosis is a rare entity, usually within the context of systematic sarcoidosis. Isolated neurosarcoidosis and especially a manifestation with pachymeningitis is a notable rarity. A 26-year-old patient presented to the emergency department with acute onset, recurrent episodes of occipital headaches spreading over the whole cranium and vomiting without food consumption, for three days. The clinical examination did not reveal any neurological deficits. The laboratory exams showed no pathological findings. A CT examination with angiography did not detect any acute intracranial or vessel pathology. A lumbar puncture was performed to rule out subarachnoid hemorrhage. The results showed a lymphocytic pleocytosis of 400/µL, elevated protein levels of 1077 mg/dL and reduced glucose levels (CSF: 55 mg/dL, Serum: 118 mg/dL). Extensive infectiological examinations did not reveal any signs of infection, including Borrelia spp. and M. tuberculosis. No positive auto-antibodies or vasculitis-related auto-antibodies were detected. The CSF analysis showed negative oligoclonal bands but an isolated increase in β2-microglobulin, neopterin, and IL-2R levels. The MRI examination revealed a dural gadolinium-enhancement, pronounced in the basal cerebral structures and the upper segment of the cervical spine, consistent with neurosarcoidosis. Corticosteroid treatment rapidly led to a significant improvement of the symptoms. No systemic manifestations of sarcoidosis were found. This case report aims to highlight aseptic meningitis with atypical, acute onset headache attacks as a possible manifestation of isolated neurosarcoidosis. Neurosarcoidosis is a clinical entity that requires prompt treatment to avoid permanent neurological deficits.