title: Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
abstract: Clinical features, brain magnetic resonance imaging findings and EDSS scores of 11 patients with neurodegenerative central nervous system Langerhans cell histiocytosis were analyzed in Japan. All patients initially had multi-system-type Langerhans cell histiocytosis; 8 at 1-2 years of age and 3 at a later age. Neurodegenerative central nervous system Langerhans cell histiocytosis disease developed after a median time interval of 3.9 years from initial diagnosis. With a median follow-up of 4.5 years, 6 patients showed progression of disease with an EDSS score >3. This study demonstrates the importance of early detection of neurodegenerative central nervous system Langerhans cell histiocytosis by brain magnetic resonance imaging, particularly in the follow-up of patients who developed multi-system-type Langerhans cell histiocytosis in early infancy.
authors.affiliation: Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago, Hyogo Prefecture, Japan 676-0812. shinim95@mbox.kyoto-inet.or.jp
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
authors.forename: Yoko
authors.initials: Y
authors.lastname: Shioda
authors.forename: Ryoji
authors.initials: R
authors.lastname: Kobayashi
authors.forename: Gaku
authors.initials: G
authors.lastname: Hosoi
authors.forename: Hisanori
authors.initials: H
authors.lastname: Fujino
authors.forename: Shiro
authors.initials: S
authors.lastname: Seto
authors.forename: Hisashi
authors.initials: H
authors.lastname: Wakita
authors.forename: Akira
authors.initials: A
authors.lastname: Oka
authors.forename: Nagisa
authors.initials: N
authors.lastname: Okazaki
authors.forename: Naoto
authors.initials: N
authors.lastname: Fujita
authors.forename: Toshinori
authors.initials: T
authors.lastname: Minato
authors.forename: Kenichi
authors.initials: K
authors.lastname: Koike
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Tsunematsu
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001921:Brain / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology; D054022:Cerebrum / Q000473:pathology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D019636:Neurodegenerative Diseases / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D011149:Pons / Q000473:pathology; D012042:Registries / Q000706:statistics & numerical data; D012720:Severity of Illness Index