Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas |
| |
| Authors: | E. Ann Yeh Jay Greenberg Oussama Abla Giulia Longoni Eli Diamond Michelle Hermiston Brandon Tran Carlos Rodriguez‐Galindo Carl E. Allen Kenneth L. McClain |
| |
| Affiliation: | 1. Department of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada;2. Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, District of Columbia;3. Division of Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada;4. Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, New York;5. Department of Pediatric Oncology, UCSF Medical Center‐Mission Bay, San Francisco, California;6. Department of Radiology, Baylor College of Medicine, Houston, Texas;7. Department of Pediatrics, St. Jude Children's Research Hospital, Memphis, Tennessee;8. Department of Pediatrics, Texas Children's Cancer and Hematology Centers and Baylor College of Medicine, Houston, Texas |
| |
| 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. |
| |
| Keywords: | brain MRI Langerhans cell histiocytosis neurologic dysfunction psychological dysfunction |
|
|
