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AJNR Am J Neuroradiol. 2004 May;25(5):880-91.

MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis.

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  • 1Department of Neuroradiology, University Clinic of Radiodiagnostics, Vienna, Austria.

Abstract

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.

PMID:
15140741
[PubMed - indexed for MEDLINE]
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