3.8 Article

Lateralized ante mortem and post mortem pathology in a case of Lewy body disease with corticobasal syndrome

Publisher

WILEY
DOI: 10.1002/trc2.12294

Keywords

alpha-synuclein; Alzheimer's disease; cerebrospinal fluid; corticobasal syndrome; flortaucipir; Lewy body dementia; neuropathology

Funding

  1. AAN [CRTS 2059]
  2. National Institutes of Health [P30AG072979, AG043503, AG062429, AG062418, NS053488, NS109260]

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This study reported a case with overlapping clinical features of synucleinopathy and tauopathy, and through biomarker characterization and post-mortem sampling, showed lateralized tau and alpha-synuclein pathology suggesting possible synergistic relationships.
Introduction Lewy body diseases are pathologically characterized by alpha-synuclein pathology. Alzheimer's disease (AD) co-pathology can influence phenotypes. In vivo AD biomarkers can suggest the presence of this co-pathology in unusual cases, but pathological validation remains essential. Methods This patient originally presented with corticobasal syndrome and later developed visual hallucinations and parkinsonism consistent with a synucleinopathy. The patient underwent CSF sampling, 18F-flortaucipir PET scanning, and brain donation with bilateral regions available for digital histological analysis. Results CSF A beta 42 and t-tau were in the AD range. 18F-flortaucipir scanning showed right-lateralized retention in all lobes (t = 4.3-10.0, P < .006). Neocortical stage Lewy body pathology and high levels of AD neuropathological changes were present at autopsy. There was right lateralization of alpha-synuclein and tau pathology (T value = 3.1, P value = .007 and T value = 3.3, P value = .004 respectively). Discussion This case with overlapping tauopathy and synucleinopathy clinical features had in-depth biomarker characterization and rare bilateral post-mortem sampling showing lateralized tau and alpha-synuclein pathology suggesting possible synergistic relationships.

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