Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme

• Published in: Journal of the neurological sciences 2011-02, Vol.301 (1), p.96-99
• Main Authors: Perren, F, Fankhauser, L, Thiévent, B, Pache, J.-C, Delavelle, J, Rochat, T, Landis, T, Chizzolini, C
• Format: Article
• Language: English
• Subjects: Age of Onset; Alien hand; Alien Hand Syndrome - etiology; Biological and medical sciences; Biopsy; Bone and Bones - pathology; Brain - pathology; Brain Diseases - complications; Brain Diseases - diagnosis; Brain Diseases - drug therapy; Brain Neoplasms - diagnosis; Cerebellar Ataxia - etiology; CNS involvement; Development. Senescence. Regeneration. Transplantation; Dexamethasone - administration & dosage; Dexamethasone - therapeutic use; Diagnosis, Differential; Drug Therapy, Combination; Female; Fundamental and applied biological sciences. Psychology; Glioblastoma - diagnosis; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - drug therapy; Histiocytosis, Langerhans-Cell - epidemiology; Humans; Langerhans cell histiocytosis; Lung - pathology; Magnetic Resonance Imaging; Medical sciences; Middle Aged; Multiple Sclerosis - diagnosis; Mycophenolate mofetil; Mycophenolic Acid - administration & dosage; Mycophenolic Acid - analogs & derivatives; Mycophenolic Acid - therapeutic use; Neurology; Paresis - etiology; Vertebrates: nervous system and sense organs
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2010.11.006

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.