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Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis
L B Poe1, R L Dubowy, L Hochhauser
1Department of Radiology, State University of New York Health Science Center at Syracuse.
Insights
Langerhans cell histiocytosis can affect the cerebellum with gliotic and demyelinating lesions, visible on imaging years before symptoms. These cerebellar changes may signal future central nervous system decline.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder.
- Cerebellar involvement in LCH is not well-characterized.
Purpose of the Study:
- To describe cerebellar involvement in LCH.
- To characterize the imaging and pathological findings of cerebellar lesions in LCH.
Main Methods:
- Retrospective analysis of 30 LCH cases.
- Review of CT and MRI scans for cerebellar abnormalities.
- Histopathological examination of one patient's cerebellar biopsy.
Main Results:
- Four patients showed symmetric hypodensities in the dentate nuclei region on CT/MRI.
- MRI revealed specific signal characteristics (hypointense on STIR/SE, hyperintense on TR/TE).
- Biopsy confirmed demyelination, cell loss, and gliosis without histiocytic infiltration.
Conclusions:
- LCH involves the cerebellum through a specific gliotic and demyelinating process.
- Cerebellar lesions may precede clinical symptoms by years.
- These lesions can occur without prior radiation and may predict CNS deterioration.
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.