An unusual form of neurosarcoidosis with intracranial mass lesion, chronicmeningitis, hydrocephalus and hypothalamic dysfunction

A 30-year-old woman developed progressive left sided hemiparesis with intra cranial hypertension signs. CT scan and MRI showed a large temporo parietal cystic mass with marked surrounding edema. Surgical excision was performed and histological analysis revealed an inflammatory granuloma. No disease e lsewhere was found and all classical causes of granulomas such as tuberculo sis, toxoplasma, fungus infections, inflammatory diseases, lymphomas and ca ncers were excluded. No treatment was administred and she remained neurolog ically stable for two years. Afterwards, she developed chronic meningo-ence phalitis, hypothalamic-pituitary dysfunction and hydrocephalus requiring de compression. Sarcoidosis was suspected, a steroid therapy was initiated she gradually improved and a ventricular biopsy confirmed this diagnosis. Nerv ous system lesions complicate the course of sarcoidosis in 5 to 15 % of pat ients and most commonly involve the cranial and peripheral net-yes. CNS inv olvement is typically meningeal with a predilection for the hypothalamic re gion. Intracranial mass lesions are rare and their occurrence in the absenc e of disease elsewhere is still more unusual. Three presentations have been described: art isolated intra parenchymatous mass, multiples nodules, and subdural plaques, that can be mistaken for meningiomas, gliomas or metastas es. When systemic manifestations of sarcoidosis are absent, the diagnosis is di fficult, and Gd-enhanced MRI is now considered the diagnostic method of cho ice. However brain biopsy is sometimes necessary. Corticosteroids are the m ainstay of therapy. Immunosupressive agents are also used and brain irradia tion has been tried in some refractory cases. Surgical approach may be indi cated to establish tissue diagnosis, to perform decompression and To remove brain tumors.