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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 28:1022-1028, June-July 2007
DOI 10.3174/ajnr.A0509
© 2007 American Society of Neuroradiology

PEDIATRICS

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

H. Prosch^a,b, N. Grois^b, M. Wnorowski^b, M. Steiner^b and D. Prayer^c

^a Department of Radiology, Otto Wagner Hospital, Vienna, Austria
^b Children's Cancer Research Institute, Vienna, Austria
^c Medical University of Vienna, Division of Neuroradiology, Vienna, Austria

Address correspondence to Daniela Prayer, Division of Neuroradiology, Medical University of Vienna, Waehringerguertel 18–20, 1090 Vienna, Austria; e-mail: daniela.prayer{at}meduniwien.ac.at

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.

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				H. Prosch, N. Grois, and D. Prayer Reply: AJNR Am. J. Neuroradiol., April 1, 2008; 29(4): e15 - e15. [Full Text] [PDF]