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Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme

Thiévent, B
Published in Journal of the Neurological Sciences. 2011, vol. 301, no. 1-2, p. 96-9
Abstract Langerhans cell histiocytosis (LCH) with multiple organ involvement is a rare disorder in adults. Extrapituitary involvement of the central nervous system (CNS) is uncommon. We report the unusual case of a 55-year-old woman presenting with a left-sided hemiataxia-hemiparesis, left hemisensory loss and short-lasting episodes of an alien left hand due to lesions of the internal capsule and the right thalamus, extending into the mesencephalon associated with extensive surrounding edema, without pituitary involvement. The neuroradiological image suggested glioblastoma multiforme. Brain biopsy revealed inflammatory tissue and "pseudotumoral" multiple sclerosis was suspected. Biopsy of concomitant lung and bone lesions disclosed Langerhans cell histiocytosis. The treatment with pulsed steroids in association with mycophenolate mofetil led to a sustained, clinical neurological remission.
Keywords Age of OnsetAlien Hand Syndrome/etiologyBiopsyBone and Bones/pathologyBrain/pathologyBrain Diseases/complications/diagnosis/drug therapyBrain Neoplasms/diagnosisCerebellar Ataxia/etiologyDexamethasone/administration & dosage/therapeutic useDiagnosis, DifferentialDrug Therapy, CombinationFemaleGlioblastoma/diagnosisHistiocytosis, Langerhans-Cell/complications/diagnosis/drug therapy/epidemiologyHumansLung/pathologyMagnetic Resonance ImagingMiddle AgedMultiple Sclerosis/diagnosisMycophenolic Acid/administration & dosage/analogs & derivatives/therapeutic useParesis/etiology
PMID: 21131007
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Research groups Groupe Pache Jean-Claude (pathologie clinique) (658)
La matrice extracellulaire (651)
L'épithélium respiratoire (852)
(ISO format)
PERREN, Fabienne et al. Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme. In: Journal of the Neurological Sciences, 2011, vol. 301, n° 1-2, p. 96-9. doi: 10.1016/j.jns.2010.11.006 https://archive-ouverte.unige.ch/unige:24761

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Deposited on : 2012-12-19

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