Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis
H. Proscha,b,
N. Groisb,
M. Wnorowskib,
M. Steinerb and
D. Prayerc
a Department of Radiology, Otto Wagner Hospital, Vienna, Austria
b Children's Cancer Research Institute, Vienna, Austria
c Medical University of Vienna, Division of Neuroradiology, Vienna, Austria
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Fig 1. Course of signal intensity alteration in the basal ganglia in patient 6.
A, Axial T1-weighted images show mild hyperintense signal intensity alterations limited to the pallidum.
B, Three years later, prominent hyperintense signal intensity alterations involve the pallidum and the putamen.
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Fig 2. Course of cerebellar atrophy in patient 4. Sagittal T1-weighted images show mild cerebellar atrophy at the time of the diagnosis of ND-LCH (A). Seven years later the atrophy is more pronounced (B).
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Fig 3. Course of cerebellar MR imaging findings in patient 8. Left column, T1-weighted images (T1WI); right column, T2-weighted images (T2WI). The first signal intensity abnormalities in the cerebellum were detected at the age of 4 years (1994) and were composed of subtle increased signal intensities on T2WI limited to the region of the dentate nucleus. In 1997, the signal intensity abnormalities on T1- and T2WI involved the dentate nucleus, surrounding white matter, middle cerebellar peduncle, and pons. In 2001, the patient developed an obstructive hydrocephalus, a thinning of the corpus callosum, and progressive cerebellar neurologic symptoms. At the last follow-up (2005), the dentate nucleus appeared atrophic.
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Fig 4. (A) Axial T2-weighted image obtained 2 months after a biopsy of the left cerebellar cortex (arrow) shows extensive hyperintensities in the cerebellar white matter extending to the middle cerebellar peduncle and the dorsal pons.
B and C, Cerebellar biopsy: the cerebellar cortex shows diminished thickness of the molecular layer. Immunocytochemistry for microtubule associated protein II (MAP II) reveals a massive loss of Purkinje cells and their dendrites. (B; x60), the adjacent sections, stained for glial fibrillary acidic protein, show massive astrocytic gliosis, reflected by thick radial glial cell processes in the molecular layer (C; x60). (Courtesy of Prof Hans Lassmann, Brain Research Institute, Medical University of Vienna, Vienna, Austria).
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