Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis

• Published in: American journal of neuroradiology 1994-11, Vol.15 (10), p.1921-1928
• Main Authors: Poe, LB, Dubowy, RL, Hochhauser, L, Collins, GH, Crosley, CJ, Kanzer, MD, Oliphant, M, Hodge, CJ, Jr
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Biopsy; Cerebellar Diseases - diagnosis; Cerebellar Nuclei - pathology; Cerebellum - pathology; Child; Child, Preschool; Demyelinating Diseases - diagnosis; Female; Gliosis - diagnosis; Hematologic and hematopoietic diseases; Histiocytes - pathology; Histiocytosis, Langerhans-Cell - diagnosis; Humans; Magnetic Resonance Imaging; Male; Medical sciences; Myelin Sheath - pathology; Neurologic Examination; Other diseases. Hematologic involvement in other diseases; Prospective Studies; Retrospective Studies; Review; Tomography, X-Ray Computed
• ISSN: 0195-6108; 1936-959X

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