title: Strategies for the Prevention of Central Nervous System Complications in Patients with Langerhans Cell Histiocytosis: The Problem of Neurodegenerative Syndrome.
abstract: Diseases of the central nervous system (CNS) are common in patients with Langerhans cell histiocytosis (LCH). Besides active LCH lesions, neurodegenerative (ND) lesions of the cerebellum and/or basal ganglia may occur as late sequelae of LCH. While the etiology of this ND disease remains unclear, biomarkers in cerebrospinal fluid (CSF) may reflect the activity of CNS disease in these patients. However, no well-planned CSF studies have yet been performed in patients at high risk for ND-CNS-LCH. Potential parallels with other neuroinflammatory/neurodegenerative disease suggest the utility of examining these other disorders in establishing strategies for the prevention and/or treatment of ND-CNS-LCH.
authors.affiliation: Division of Hematology, Takasago-Seibu Hospital, 1-10-41 Nakasuji, Takasago 676-0812, Japan. Electronic address: shinim95@mbox.kyoto-inet.or.jp.
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
authors.affiliation: Children's Center for Cancer and Blood Disorders, Department of Child Health, Phoenix Children's Hospital, University of Arizona College of Medicine, 445 North 5th Street, Suite 322, Phoenix, AZ 85004, USA.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Arceci
mesh_terms: D015415:Biomarkers; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control* / Q000628:therapy; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000209:etiology / Q000378:metabolism; D006801:Humans; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000209:etiology / Q000517:prevention & control / Q000628:therapy
chemical_list: D015415:Biomarkers
keywords: Central nervous system; Diabetes insipidus; Langerhans cell histiocytosis; Neurodegenerative disease; Neuroinflammatory disease