PT  - JOURNAL ARTICLE
AU  - H. Prosch
AU  - N. Grois
AU  - M. Wnorowski
AU  - M. Steiner
AU  - D. Prayer
TI  - Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis
AID  - 10.3174/ajnr.A0509
DP  - 2007 Jun 01
TA  - American Journal of Neuroradiology
PG  - 1022--1028
VI  - 28
IP  - 6
4099  - http://www.ajnr.org/content/28/6/1022.short
4100  - http://www.ajnr.org/content/28/6/1022.full
SO  - Am. J. Neuroradiol.2007 Jun 01; 28
AB  - 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.