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J Clin Neurosci. 2014 Feb;21(2):305-10. doi: 10.1016/j.jocn.2013.03.032. Epub 2013 Oct 9.

Spectrum of imaging appearances in Australian children with central nervous system hemophagocytic lymphohistiocytosis.

Author information

1
Department of Medical Imaging, Royal Children's Hospital Melbourne, Flemington Road, Parkville, VIC 3052, Australia. Electronic address: guandalini_ms@internode.on.net.
2
Department of Medical Imaging, Royal Children's Hospital Brisbane, Herston, QLD, Australia.
3
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.

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

PMID:
24119957
DOI:
10.1016/j.jocn.2013.03.032
[Indexed for MEDLINE]

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