A 29-year-old man was admitted due to focal aware epileptic seizures with transient facial paresis and aphasia. He reported a 1-year history of fatigue symptoms with lack of appetite and weight loss. Neurological examination showed no focal deficit and slight generalized EEG slowing. Cranial MRI revealed multiple contrast-enhancing small nodular structures along the leptomeninges of the cortical sulci and the choroid plexus of the lateral ventricles. The CSF cell count was normal but the blood–brain barrier function was severely impaired (total protein 2485 mg/l, normal range 200–450) with elevated CSF lysozyme (>1500 µg/l, normal value <62). Serum levels of ACE, sIL-2-receptor, and neopterin were elevated, radiography and HR-CT of the thorax showed bihilar lymphadenopathy and proliferation of reticulonodular streaking, and epithelioid cell granulomas were demonstrated in an endosonographically obtained biopsy sample. These findings confirmed the presence of a rare neurological primary manifestation of sarcoidosis. The patient was initially treated with steroids but developed a delusional psychosis. Only when he was given the TNF blocker infliximab did his condition stabilize.
Dr. med. Ralph Werner, Prof. Dr. med. Johannes C. Wöhrle, Abteilung für Neurologie/Stroke Unit, Katholisches Klinikum Koblenz-Montabaur, email@example.com
Dr. med. Wolfgang Neumeister, Klinik für Innere Medizin/Pneumologie, Schlaf- und Beatmungsmedizin, Katholisches Klinikum Koblenz-Montabaur
Conflict of interest statement: The authors declare that no conflict of interest exists.
Translated from the original German by David Roseveare
Cite this as: Werner R, Neumeister W, Wöhrle JC: Neurosarcoidosis. Dtsch Arztebl Int 2018; 115: 635. DOI: 10.3238/arztebl.2018.0635