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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:880-891, May 2004
© 2004 American Society of Neuroradiology

PEDIATRIC

MR Imaging Presentation of Intracranial Disease Associated with Langerhans Cell Histiocytosis

Daniela Prayer^a, Nicole Grois^b, Helmut Prosch^b, Helmut Gadner^b and Anthony J. Barkovich^c

^a Department of Neuroradiology, University Clinic of Radiodiagnostics, Vienna, Austria
^b Children’s Cancer Research Institute, Vienna, Austria
^c Department of Neuroradiology, University of San Francisco, CA

Address reprint requests to Daniela Prayer, MD, University Clinic of Radiodiagnostics, Department of Neuroradiology, Waehringerguertel 18–20, Vienna 1090, Austria

BACKGROUND AND PURPOSE: Intracranial manifestations of Langerhans cell histiocytosis (LCH) are underestimated in frequency and diversity. We categorized the spectrum of MR imaging changes in LCH.

METHODS: We retrospectively reviewed 474 MR images in 163 patients with LCH and 55 control subjects. Lesions were characterized by anatomic region and signal intensity. Brain atrophy was assessed.

RESULTS: We noted osseous lesions in the craniofacial or skull bones in 56% of patients, meningeal lesions in 29%, and choroid-plexus involvement in 6%. In the hypothalamic-pituitary region, infundibular thickening occurred in 50%; pronounced hypothalamic mass lesions in 10%; and infundibular atrophy in 29%. The pineal gland had a cystic appearance in 28%, and pineal-gland enlargement (>10 mm) was noted in 14%. Nonspecific paranasal-sinus or mastoid opacifications were seen in 55% of patients versus 20% of controls, and accentuated Virchow-Robin spaces occurred in 70% of patients versus 27% of controls (P < .001). Intra-axial, white-matter parenchymal changes resulted in a leukoencephalopathy-like pattern in 36%. Enhancing lesions in a vascular distribution were noted in 5%. Gray-matter changes suggestive of neurodegeneration were identified in the cerebellar dentate nucleus in 40% and in the supratentorial basal ganglia in 26%. All patients with neurodegenerative lesions had lesions in the extra-axial spaces. Cerebral atrophy was found in 8%.

CONCLUSION: In LCH, cranial and intracranial changes at MR imaging include 1) lesions of the craniofacial bone and skull base with or without soft-tissue extension; 2) intracranial, extra-axial changes (hypothalamic-pituitary region, meninges, circumventricular organs); 3) intracranial, intra-axial changes (white matter and gray matter); and 4) cerebral atrophy.

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