Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas Journal Article


Authors: Yeh, E. A.; Greenberg, J.; Abla, O.; Longoni, G.; Diamond, E.; Hermiston, M.; Tran, B.; Rodriguez-Galindo, C.; Allen, C. E.; McClain, K. L.; on behalf of the North American Consortium for Histiocytosis
Article Title: Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas
Abstract: Central nervous system (CNS) involvement in Langerhans cell histiocytosis (LCH) can include mass lesions of the hypothalamic pituitary axis, choroid plexus, cerebrum, and cerebellum or magnetic resonance imaging (MRI) signal abnormalities of the cerebellum, pons, and basal ganglia. The term neurodegenerative (ND) CNS-LCH has been given to the MRI signal abnormalities and neurologic dysfunction, although initially patients may have no clinical symptoms. Standardized evaluations to better understand the natural history and response to therapy are needed. We propose guidelines for clinical, radiologic, and physiologic tests as a framework for developing the best methods of evaluation, which can then be tested in prospective treatment protocols. © 2017 Wiley Periodicals, Inc.
Keywords: review; nonhuman; neuroimaging; nuclear magnetic resonance imaging; staging; disease association; image analysis; practice guideline; quantitative analysis; neuropsychological test; radiography; neurologic examination; nerve degeneration; evoked response; central nervous system disease; disease surveillance; langerhans cell histiocytosis; brain damage; nervous system inflammation; brain mri; human; priority journal; neurologic dysfunction; psychological dysfunction; neurophysiological monitoring
Journal Title: Pediatric Blood and Cancer
Volume: 65
Issue: 1
ISSN: 1545-5009
Publisher: Wiley Periodicals, Inc  
Date Published: 2018-01-01
Start Page: e26784
Language: English
DOI: 10.1002/pbc.26784
PROVIDER: scopus
PUBMED: 28944988
DOI/URL:
Notes: Review -- Export Date: 2 January 2018 -- Source: Scopus
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  1. Eli Louis Diamond
    202 Diamond