title: Spectrum of imaging appearances in Australian children with central nervous system hemophagocytic lymphohistiocytosis.
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.
authors.affiliation: Department of Medical Imaging, Royal Children's Hospital Melbourne, Flemington Road, Parkville, VIC 3052, Australia. Electronic address: guandalini_ms@internode.on.net.
authors.forename: Michael
authors.initials: M
authors.lastname: Guandalini
authors.affiliation: Department of Medical Imaging, Royal Children's Hospital Brisbane, Herston, QLD, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Butler
authors.affiliation: Department of Medical Imaging, Royal Children's Hospital Melbourne, Flemington Road, Parkville, VIC 3052, Australia; Florey Neuroscience Institutes, Melbourne Brain Centre, Melbourne, VIC, Australia; Department of Radiology, University of Melbourne, Parkville, VIC, Australia.
authors.forename: Simone
authors.initials: S
authors.lastname: Mandelstam
mesh_terms: D000293:Adolescent; D001284:Atrophy / Q000209:etiology / Q000473:pathology; D001315:Australia; D001921:Brain / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000150:complications / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D005682:Gadolinium; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology*; D012189:Retrospective Studies; D013355:Subdural Space / Q000473:pathology*; D016896:Treatment Outcome
chemical_list: D005682:Gadolinium
keywords: Brain; Hemophagocytic lymphohistiocytosis; Magnetic resonance imaging