ABSTRACT Neurosarcoidosis is a rare disease. Diagnosis and treatment can be very challenging since clinical examination, laboratory and imaging findings are often non-specific. Neural tissue biopsy is frequently not feasible because of the location of suspicious lesions, and biopsy results can be misleading. We screened the local database at our University Hospital, offering an outpatient unit for patients with neurosarcoidosis, for patients with major obstacles and turnarounds during diagnostic work-up or treatment for suspected neurosarcoidosis. We identified 4 patients with a challenging work-up and unexpected final diagnosis. The first patient showed an intramedullary lesion suspicious for a malignant brain tumor. Diagnostic reevaluation showed mediastinal lymphadenopathy with non-caseating granulomas. Another patient turned out to have cerebral tuberculosis after two misleading biopsies and was being treated for neurosarcoidosis for almost one year. The third patient with only minor imaging findings showed severe problems with hydrocephalus after lumbar puncture despite having a ventriculoperitoneal shunt. The last patient turned out to have an intracranial germinoma after three biopsies and more than 6 months of treatment for suspected neurosarcoidosis. Neurosarcoidosis is known to mimic several neurological diseases and even with histopathology diagnosis can be challenging. The final diagnoses that were made in our patients had dramatic impact on treatment. Therefore, diagnosis and treatment should be preferentially done at tertiary hospitals in a wide interdisciplinary setting. In particular, the necessity of obtaining multiple biopsies in order to establish a reliable diagnosis emerges from the experience with the case series presented here.
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