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A Rare Form of Lewy Body Disease Examined

Neurology; ePub 2018 Jun 13; Kasanuki, et al

The neuropathology underlying corticobasal syndrome (CBS) is heterogeneous, including corticobasal degeneration, Alzheimer disease (AD), and progressive supranuclear palsy (PSP), according to a recent study. These results suggest that atypical variants of Lewy body disease with severe peri-Rolandic Lewy-related pathology can present clinically as CBS. In 523 autopsy-confirmed cases of diffuse Lewy body disease (DLBD), researchers identified 11 patients diagnosed with CBS. For comparison, they studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). They found:

  • DLBD patients with antemortem features of CBS were significantly younger at disease onset and less likely to have rapid eye movement (REM) sleep behavior disorder than DLBD cases who met clinical criteria for DLB during life.
  • Patients with DLBD manifesting as CBS had more Lewy bodies in the motor cortex than DLBD manifesting as clinically probable DLB.
  • 3 cases had concomitant PSP and 4 cases had concomitant AD as probable correlates of CBS.


Kasanuki K, Josephs KA, Ferman TJ, et al. Diffuse Lewy body disease manifesting as corticobasal syndrome. A rare form of Lewy body disease. [Published online ahead of print June 13, 2018]. Neurology. doi:10.1212/WNL.0000000000005828.

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