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MR Imaging of Autopsy-Proved Paraneoplastic Limbic Encephalitis in Non-Hodgkin Lymphoma

Christina Thuerla, Klaus Müllerb, Jörg Laubenbergera, Benedikt Volkb and Mathias Langera

a Departments of Radiology, University of Freiburg, Freiburg, Germany
b Neuropathology, University of Freiburg, Freiburg, Germany



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FIG 1. Initial MR images obtained 1 day after the first generalized seizure occurred.

A, Axial fluid-attenuated inversion recovery image (9000/110 [TR/TE]; inversion time, 2261 ms) shows a slightly elevated signal intensity of both hippocampal formations (black arrows) and amygdala (white arrows).

B, Coronal conventional T2-weighted turbo spin-echo image (4462/120/3 [TR/TE/NEX]) shows no signal intensity abnormality.



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FIG 2. Follow-up MR images obtained 4 days after the first seizure occurred.

A, Fluid-attenuated inversion recovery image shows high signal intensity in both medial temporal lobes and hyperintensities of the right frontobasal cortex (arrowhead).

B, Fluid-attenuated inversion recovery image shows high signal intensity in both medial temporal lobes and hyperintensities of the right insular cortex (arrowhead). No mass effect was seen.

C, Diffusion-weighted echo-planar image (5400/103/1; b value of 1000 s/mm2) shows the corresponding alterations of the limbic (arrows) and the right frontobasal cortex (arrowhead).

D, T1-weighted image (548/12/2) shows low signal intensity of the right medial temporal lobe (arrow).

E, Contrast-enhanced T1-weighted image (525/17/2) shows no contrast enhancement.



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FIG 3. Reactive astrocytosis and lymphocytic infiltration with T cells were found.

A, Glial fibrillary acidic protein immunostaining. Neuronal loss in the hippocampus and replacement by astrocytes (arrows). Granular layer of CA1 of the hippocampus (arrowhead). Scale bar = 250 µm.

B, Immunostaining with anti-CD3 antibody shows interstitial inflammatory infiltrates of T cells in the mesial temporal lobe (arrows). Section counterstained with hemalaun. Scale bar = 50 µm.



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FIG 4. CD68 immunostaining reveals activated microglial cells, which cluster around damaged neurons (arrows). Scale bar = 70 µm.



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FIG 5. Immunoglobulin M immunostaining. Antineuronal antibodies of immunoglobulin M type are bound to the cytoplasm of hippocampal neuron (arrow). Scale bar = 50 µm.