Document Detail

Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis.
MedLine Citation:
PMID:  17569949     Owner:  NLM     Status:  MEDLINE    
BACKGROUND AND PURPOSE: MR imaging signal intensity abnormalities in the cerebellum, the pons, and the basal ganglia, compatible with a neurodegenerative process (ND) were reported in up to 10% of patients with Langerhans cell histiocytosis (LCH). Although the imaging features of ND-LCH have been extensively described, the temporal course of ND-LCH has not been assessed as of yet. The purpose of this study was to describe the long-term course of MR imaging signal intensity abnormalities in ND-LCH on T1- and T2-weighted images. MATERIALS AND METHODS: In this retrospective study, 9 patients with ND-LCH with an observation time of at least 5 years were included. Three or more MR imaging studies per patient, performed in 3-year intervals (+/-11 months), were reviewed. Signal intensity abnormalities on T1- and T2-weighted images in the cerebellum, the pons, and basal ganglia were scored for their signal intensity quality and their extension. In addition, the severity of cerebellar atrophy was scored. RESULTS: The signal intensity alterations were not resolved in any of the patients. Instead, a progression of the signal intensity alterations either in the cerebellum or basal ganglia was observed in all of the patients but did not correlate with a clinical deterioration. Overt and severe neurologic symptoms were reported in only 2 patients in whom some form of atrophy was noted. CONCLUSIONS: ND-LCH appears to be a slowly progressive process. The increase of signal intensity abnormalities in the cerebellum and basal ganglia does not correlate with neurologic deterioration. MR imaging appears to be a sensitive technique to detect and monitor radiologic ND-LCH.
H Prosch; N Grois; M Wnorowski; M Steiner; D Prayer
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  AJNR. American journal of neuroradiology     Volume:  28     ISSN:  0195-6108     ISO Abbreviation:  AJNR Am J Neuroradiol     Publication Date:    2007 Jun-Jul
Date Detail:
Created Date:  2007-06-15     Completed Date:  2007-08-08     Revised Date:  2008-06-24    
Medline Journal Info:
Nlm Unique ID:  8003708     Medline TA:  AJNR Am J Neuroradiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1022-8     Citation Subset:  IM    
Department of Radiology, Otto Wagner Hospital, Vienna, Austria.
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MeSH Terms
Basal Ganglia / pathology*
Brain Diseases / diagnosis*
Cerebellum / pathology*
Child, Preschool
Histiocytosis, Langerhans-Cell / diagnosis*
Longitudinal Studies
Magnetic Resonance Imaging*
Neurodegenerative Diseases / diagnosis*
Comment In:
AJNR Am J Neuroradiol. 2008 Apr;29(4):e14; author reply e15   [PMID:  18296544 ]

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