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Images in Neurology |

Lymphomatosis Cerebri A Diagnostic Challenge

Kimmo J. Hatanpaa, MD, PhD1; Franklin Fuda, DO1; Prasad Koduru, PhD1; Ken Young, MD2; Bradley Lega, MD3; Weina Chen, MD, PhD1
[+] Author Affiliations
1Department of Pathology, University of Texas Southwestern Medical Center, Dallas
2Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston
3Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas
JAMA Neurol. 2015;72(9):1066-1067. doi:10.1001/jamaneurol.2015.1149.
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An immunocompetent woman in her mid-60s initially presented with episodes of spasms and tremors in her right hand and forearm. Lymphoma cells were present in cerebrospinal fluid but not in bone marrow. Findings are diagnostic of the lymphomatosis cerebri variant of diffuse, large B-cell lymphoma.

An immunocompetent woman in her mid-60s initially presented with episodes of spasms and tremors in her right hand and forearm. She was suspected of having carpal tunnel syndrome and underwent carpal tunnel release surgery. However, the tremors recurred. She was referred to a neurologist about 7 months later. Magnetic resonance imaging (MRI) of her brain revealed either a prior stroke or demyelination, and she was treated with levetiracetam. Over the subsequent 3 months, she developed progressive right-sided weakness, dysarthria, hand tremors, slurred speech, and gait instability. She was referred to our institution for a brain biopsy. T2-weighted fluid-attenuated inversion recovery MRI revealed hyperintensities throughout the bilateral cerebral hemispheric white matter, corpus callosum, basal ganglia, thalami, midbrain, upper pons, and middle cerebellar peduncles (Figure, A). T1-weighted fluid-attenuated inversion recovery MRI with contrast showed the absence of contrast enhancement (Figure, B). Given the multifocal distribution of the lesions and the lack of contrast enhancement, the main differential considerations were demyelinating disease, infection, and encephalitis.

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Extensive Nonenhancing Lesions in the Bilateral Cerebral Hemispheres Indicating Lymphomatosis Cerebri

A, Diffuse T2-weighted fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) reveals hyperintensities in the white matter and corpus callosum. The lesions extended to the basal ganglia, thalami, midbrain, pons, and middle cerebellar peduncles. B, T1-weighted FLAIR MRI with contrast reveals the absence of contrast enhancement. C, The brain parenchyma is diffusely infiltrated by large lymphoma cells (hematoxylin-eosin, original magnification ×200). D, The lymphoma cells express CD20 (immunohistochemistry, original magnification ×100).

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