gms | German Medical Science

Objective: Sarcoidosis is a multisystemic granulomatous disease of unknown cause with over 90% affecting the lung or bilateral hilar lymphadenopathy. Neurosarcoidosis is rare and accounts for approximately 5-15% of the cases. Involvement of all parts of the central and peripheral nervous system is possible with various clinical symptoms, for example seizures, hyprocephalus, optic/ facial nerve palsy or hearing loss. Therefore, neurosarcoidosis is important to consider as differential diagnosis in the presence of a variety of neurologic symptoms.

Method: We screened the neuropathological data bases and the medical records of two neurosurgical University Hospitals for cases of neurosarcoidosis. All these cases were verified through surgical biopsy. We retrospectively evaluated the patients records with special regard to the histopathology reports and specific clinical symptoms.

Results: We identified 9 cases of neurosarcoidosis betwenn 1994 and 2014 (3 female, 6 male patients). They were 29 to 54 years at time of diagnosis (mean 42,8 years). Various clinical symptoms like hydrocephalus (n=3), seizures (n=1), menigitis (n=1), optical nerve involvement with vision disorder (n=1), myelitis with paraplegia (n=1), mastoiditis with hearing loss (n=1), back pain syndrome (n=2) were present. 7 patients were treated with corticosteroids, 1 patient with cyclophosphamid and 1 with corticosteroids combined with methotrexate.

Conclusions: Neurosarcoidosis is a rare but potentially life-threating disease. It is difficult to distinguish sarcoidosis from other granulomatous disease, infectious diseases like tuberculosis, multiple sclerosis or neoplasma. It needs a neurosurgical biopsy with histological evidence of non-caseating epitheloid cell granulomas for a secure diagnosis (gold standard). Corticosteroids and other immuno-suppressants (e.g. Methotreaxate, Cyclophosphamide) are the most frequently applied drugs.