Late adult onset of Langerhans cell histiocytosis mimicking glioblastoma multiforme.
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.
Department of Neurology, University Hospital and Medical Faculty of Geneva, Geneva, Switzerland.
F
F
Perren
L
L
Fankhauser
B
B
Thiévent
J-C
JC
Pache
J
J
Delavelle
T
T
Rochat
T
T
Landis
C
C
Chizzolini
D017668:Age of Onset; D055964:Alien Limb Phenomenon / Q000209:etiology; D001706:Biopsy; D001842:Bone and Bones / Q000473:pathology; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D001932:Brain Neoplasms / Q000175:diagnosis; D002524:Cerebellar Ataxia / Q000209:etiology; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005909:Glioblastoma / Q000175:diagnosis; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D008168:Lung / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000175:diagnosis; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000627:therapeutic use; D010291:Paresis / Q000209:etiology
D003907:Dexamethasone; D009173:Mycophenolic Acid