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Review
. 1994 Nov;15(10):1921-8.

Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis

L B Poe  1 , R L Dubowy, L Hochhauser, G H Collins, C J Crosley, M D Kanzer, M Oliphant, C J Hodge Jr
Affiliations
Review

Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis

L B Poe et al. AJNR Am J Neuroradiol. 1994 Nov.

Abstract

Purpose: To describe the involvement of the cerebellum by a gliotic and demyelinating process in Langerhans cell histiocytosis.

Methods: A retrospective analysis of all (N = 30) cases of Langerhans cell histiocytosis followed at our institution since 1975 yielded four patients with CT and/or MR evidence of cerebellar abnormalities.

Results: Four patients manifested strikingly similar findings of symmetric nonenhancing hypodensities in the dentate nuclei region of the cerebellum, which were hypointense on short-repetition-time/short-echo-time MR and hyperintense on long-repetition-time/long-echo-time MR. Biopsy in one patient yielded areas of demyelination, cell loss, and gliosis without histiocytic infiltration.

Conclusion: Langerhans cell histiocytosis involves the cerebellum in a specific and poorly understood manner. Lesions on imaging may precede clinical findings by years. Lesions may occur in patients who have never experienced radiation therapy and may act as a marker for eventual central nervous system deterioration.

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