期刊
PEDIATRIC BLOOD & CANCER
卷 65, 期 1, 页码 -出版社
WILEY
DOI: 10.1002/pbc.26784
关键词
brain MRI; Langerhans cell histiocytosis; neurologic dysfunction; psychological dysfunction
资金
- St. Baldrick's Foundation Consortium [318937]
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.
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