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.
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| http://dx.doi.org/10.1111/j.1651-2227.2010.02125.x | DOI Listing |
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