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Spectrum of imaging appearances in Australian children with central nervous system hemophagocytic lymphohistiocytosis
Institution:1. Department of Medical Imaging, Royal Children’s Hospital Melbourne, Flemington Road, Parkville, VIC 3052, Australia;2. Department of Medical Imaging, Royal Children’s Hospital Brisbane, Herston, QLD, Australia;3. Florey Neuroscience Institutes, Melbourne Brain Centre, Melbourne, VIC, Australia;4. Department of Radiology, University of Melbourne, Parkville, VIC, Australia;1. Department of Molecular Neurobiology and Neuropathology, National Institute of Neurology, La Rabta, Tunis, Tunisia;2. National Institute on Aging, Porter Neuroscience Research Center, National Institutes of Health, Building 35, Room 1A1010, Bethesda, MD 20892, USA;3. Laboratory of Biochemistry, Rabta Hospital, Tunis, Tunisia;1. Neurology Service, Cruces University Hospital, Department of Neurosciences, University of the Basque Country, Plaza de Cruces, s/n., Baracaldo, Vizcaya CP 48993, Spain;2. Neurophysiology Service, Cruces University Hospital, Vizcaya, Spain;1. Department of Neurology and Institute of Neurology, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai 200040, China;2. Department of Radiology, Huashan Hospital, Fudan University, Shanghai, China;3. State Key Laboratory of Medical Neurobiology, Fudan University, Shanghai, China;1. Department of Pediatric Neurology, Central Hospital, Aichi Human Service Center, Kasugai, Aichi, Japan;2. Department of Pediatrics, Okazaki City Hospital, Okazaki, Aichi, Japan;3. Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan;4. Department of Pediatrics, Anjo Kosei Hospital, Anjo, Aichi, Japan;5. Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan;6. Department of Pediatrics, Juntendo University Faculty of Medicine, Bunkyo-ku, Tokyo, Japan;7. Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan
Abstract:Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder characterised by the proliferation and infiltration of lymphocytes and histiocytes. Central nervous system (CNS) infiltration is particularly devastating. Neuroradiological findings have been reported predominantly as individual case reports due to the rarity of the condition. To our knowledge there have been no published studies of imaging in Australian patients. This study aimed to retrospectively describe and illustrate the MRI appearances of CNS involvement by HLH in a cohort of seven Australian children from two paediatric centres between 2000 and 2011. MRI appearances demonstrate intersubject and intrasubject variability over time, likely reflecting the severity of CNS infiltration and associated demyelination. Familiarity with MRI patterns is important for assessing and monitoring disease activity.
Keywords:Brain  Hemophagocytic lymphohistiocytosis  Magnetic resonance imaging
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