Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis.
OBJECTIVE
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.


CONCLUSIONS
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.
Poe
L B
LB
Department of Radiology, State University of New York Health Science Center at Syracuse.
Dubowy
R L
RL
Hochhauser
L
L
Collins
G H
GH
Crosley
C J
CJ
Kanzer
M D
MD
Oliphant
M
M
Hodge
C J
CJ
D001706:Biopsy; D002526:Cerebellar Diseases / Q000175:diagnosis*; D002529:Cerebellar Nuclei / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003711:Demyelinating Diseases / Q000175:diagnosis*; D005260:Female; D005911:Gliosis / Q000175:diagnosis; D006644:Histiocytes / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009186:Myelin Sheath / Q000473:pathology; D009460:Neurologic Examination; D011446:Prospective Studies; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed