title: [Adult onset Langerhans cell histiocytosis with progressive cerebellar ataxia and spastic paraparesis].
abstract: A 44-year-old woman with progressive cerebellar ataxia and spastic paraparesis was referred to our hospital. Brain MRI showed bilateral high signals in superior, middle, and inferior cerebellar peduncles on the T2 weighted images. After 3 years, her symptoms progressively worsened in spite of various therapies including whole brain irradiation and high dose oral prednisone. No evidence of diabetes insipidus was noted. In MRI, brainstem lesions expanded to both hemispheres of the cerebellum without enhancement by contrast medium. We confirmed diagnosis of LCH by skin biopsy of intractable truncal rash which emerged after neurological symptoms.
authors.affiliation: Department of Neurology, Kobe University Hospital.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kobessho
authors.forename: Atsushi
authors.initials: A
authors.lastname: Fukunaga
authors.forename: Shigeru
authors.initials: S
authors.lastname: Mitsui
authors.forename: Kenji
authors.initials: K
authors.lastname: Sekiguchi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Oishi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ishihara
authors.forename: Fumio
authors.initials: F
authors.lastname: Kanda
mesh_terms: D000328:Adult; D001706:Biopsy; D001921:Brain / Q000473:pathology*; D002524:Cerebellar Ataxia / Q000209:etiology*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D020336:Paraparesis, Spastic / Q000209:etiology*; D012867:Skin / Q000473:pathology*