Abstract Aim: There is currently no well-accepted therapy for central nervous system Langerhans cell histiocytosis (CNS-LCH), a neuroinflammatory disease clinically characterized by often progressive, neurological symptoms including ataxia, dysarthria, dysphagia, hypertonicity,
intellectual impairment and behavioural abnormalities. We applied immunomodulative/anti-inflammatory treatment on a patient with progressive CNS-LCH disease. Method: Intravenous immunoglobulin (IVIG) was administered monthly for 15 years to a patient with severe, image-verified
neurodegenerative CNS-LCH. Results: During the IVIG treatment, the neurological deterioration initially appeared to be haltered, but over time there was still some deterioration. Conclusions: IVIG may be beneficial in partly haltering CNS-LCH neurodegeneration,
but further studies are needed.