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Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis

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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.

Keywords: Central nervous system; Intravenous immunoglobulin; Langerhans cell histiocytosis; Therapy

Document Type: Research Article


Publication date: July 1, 2011


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