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Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis
Author(s) -
Gavhed Désirée,
Laurencikas Evaldas,
Åkefeldt Selma O,
Henter JanInge
Publication year - 2011
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.2010.02125.x
Subject(s) - medicine , langerhans cell histiocytosis , central nervous system , histiocytosis , ataxia , dysphagia , dysarthria , intravenous immunoglobulin therapy , disease , pathology , immunology , antibody , surgery , psychiatry , radiology
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.