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A Rare Presentation of Hypertrophic Olivary Degeneration Secondary to Primary Central Nervous System Lymphoma

David Cachia, MD, MRCP1; Saef Izzy, MD1; Thomas Smith, MD1; Carolina Ionete, MD, PhD1
[+] Author Affiliations
1University of Massachusetts Medical School, Worcester
JAMA Neurol. 2013;70(9):1192-1193. doi:10.1001/2013.jamaneurol.218.
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A 71-year-old man presented with a 2-week history of nausea, vomiting, unsteady gait, and diplopia. Magnetic resonance imaging of the brain revealed a gadolinium-enhanced lesion involving the superior vermis and the right middle cerebellar peduncle (Figure 1A and B). A biopsy was consistent with a high-grade B-cell lymphoma (Figure 2). Three months later, the patient was readmitted with difficulty walking, diplopia, loss of coordination, and slurring of speech. On examination, he had scanning dysarthric speech and palatal myoclonus (Video). An eye examination revealed right-sided gaze-evoked nystagmus and bilateral vertical pendular nystagmus in the primary position. Significant truncal ataxia was present. There was evidence of appendicular ataxia with dysmetria more noticeable on the right. Tone was more increased on the right than on the left, with spasticity mostly affecting the lower extremities.

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Figure 1.
Central Nervous System Lymphoma Causing Contralateral Hypertrophic Olivary Degeneration

The fluid-attenuated inversion recovery (FLAIR) sequence (A) shows a hyperintense lesion in the medial aspect of the right cerebellum, along the right lateral margin of the fourth ventricle that is enhanced with gadolinium (B). The T2-weighted axial sequence (C) and the FLAIR sequence (D) show a hyperintense lesion in the left anterior medulla in the region of the olive.

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Figure 2.
High-Grade B-Cell Lymphoma

A, The overall cellularity of the tumor is high, and extensive necrosis is apparent (hematoxylin-eosin, original magnification ×40). B, The tumor cells react diffusely to CD20, confirming the B-cell phenotype of the tumor (hematoxylin-eosin, original magnification ×40).

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