MR Imaging Presentation of Intracranial Disease Associated with Langerhans Cell Histiocytosis
Daniela Prayera,
Nicole Groisb,
Helmut Proschb,
Helmut Gadnerb and
Anthony J. Barkovichc
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
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FIG 1. Mastoid involvement at the diagnosis of LCH in a 3-year-old patient.
A, Axial contrast-enhanced T1WI shows enhancing lesions in both mastoids.
B, Axial bone-window CT scan shows bilateral osseous destruction of the mastoids.
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FIG 2. Images in two patients with LCH.
A and B, Lesions in a 13-year-old male patient at the diagnosis of LCH. Axial contrast-enhanced T1WI in A shows an extra-axial, enhancing, space-occupying lesion originating from the meninges. Axial bone-window CT scan in B shows calcification of the extra-axial lesion on the right side. Note the opacification of the left maxillary sinus.
C, Choriod plexus lesion in a 6-year-old girl with a 4.5-year history of LCH. Axial T2WIs show bilateral, hypointense masses in the choroid plexus and hyperintense changes in the parieto-occipital white matter.
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FIG 3. Images in two patients with LCH.
A, Thickened, enhancing pituitary stalk in a 2-year-old girl with a 1-year history of LCH and new-onset diabetes insipidus
B, Coronal contrast-enhanced T1WI in a 6-year-old girl obtained 2 years after the onset of diabetes insipidus. Image shows a thickened pituitary stalk at the cranial portion, in the region of the median eminence (arrow).
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FIG 4. Axial images in patients with LCH.
A, T2WI in a 16-year-old male patient with LCH diagnosed 1 year before this study. CSF-intense VRSs are visible in the deep white matter of both hemispheres. The patients had additional hyperintense changes in the dentate nucleus (not shown) and midbrain atrophy (Fig 7).
B, Contrast-enhanced T1WI in a 15-year-old female patient with a 10-year history of LCH and progressive cerebellar symptoms since 18 months of age. Enhancing lesions show a vascular pattern; some even have a space-occupying effect (arrows). Additional T1WIs (not shown) depicted hyperintense changes in the dentate nucleus, cerebellar white matter, and basal ganglia.
C, T2WI in an 8-year-old patient with a 6-year history of LCH and severe neurologic disabilities. Hyperintense changes in the posterior limb of the internal capsule (white arrow) and periventricular region have a leukodystrophy-like pattern. Image also shows hypointensity of the pallidum with a hyperintense center (black arrow).
D, T2WI in the same patient as in C shows hyperintense changes in the central pons (white arrow), dentate nucleus (black arrow), and surrounding white matter (arrowhead).
E, Contrast-enhanced T1WI in a 28-year-old man with a 1-year history of LCH and moderate dysarthria and ataxia. Image shows an enhancing lesion in the center of the pons.
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FIG 5. Axial images in a 9-year-old asymptomatic boy with a 7-year history of LCH. Top row, T2WI show the hyperintense appearance of the dentate nucleus (white arrow) and its surrounding white matter (black arrow). Note the normal appearance of the lentiform nucleus. Bottom row, T1WI with magnetization transfer contrast show the hyperintense appearance of the dentate nucleus and the lentiform nucleus (white arrows).
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FIG 6. Coronal T1WI in a 12-year-old boy with a 10-year history of LCH, severe neurologic symptoms, and intellectual impairment. Image shows CSF-intense holes in the regions of the dentate nuclei.
Fig 7.
Axial T2WIs in the same patient as in Figure 4A.
A, Cerebellar atrophy with thinned cerebellar peduncles.
B, Midbrain atrophy with wide interpeduncular cistern and distant mammillary bodies, with hypointensity of the pars compacta of the substantia nigra (arrow)
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