What rare clinical presentations of primary central nervous system lymphoma (PCNSL) have been reported?

Updated: Jan 19, 2018
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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Levin et al report 100 cases of PCNS lymphoma over a period of 10 years, of which 5% were primary meningeal lymphoma. [10] These 5 patients presented with leptomeningeal involvement as the sole manifestation and 4 of them presented with neuronal lymphomatosis affecting cranial and peripheral nerves. These manifestations can mimic other neurologic conditions such as pseudotumor cerebri or vasculitis. Meningeal or nerve biopsies were required for conclusive diagnosis.

Lenarz et al report a case of primary CNS lymphoma involving both internal auditory canals that presented with sudden deafness and disequilibrium accompanied by facial and abducens nerve palsy. [11]

Khong et al reported a rare case of neurolymphomatosis manifesting as acute cauda equina syndrome in a 16-year-old patient. [12]

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