Paper Alert: Association of CSF alpha-synuclein-SAA seed amplification results with clinical features of possible and probable dementia with Lewy bodies
Dr. Andrea Bozoki, Neurology Division Chief, contributed to a recent paper accepted for publication in Neurology.
The clinical diagnosis of dementia with Lewy bodies (DLB) is based on the presence of significant cognitive decline with accompanying features of parkinsonism, hallucinations, cognitive fluctuations, and REM sleep behavior disorder. Supportive features include hyposmia (loss of sense of smell). Recently α-technique for detecting the main abnormal protein in DLB from cerebrospinal fluid has been developed, the synuclein seeding amplification assay (α-Syn-SAA). In this paper, the authors analyzed α-Syn-SAA results from a large sample of clinically diagnosed DLB patients from the Dementia with Lewy Bodies Consortium study (DLBC) and the associations of different clinical features with α-Syn-SAA positivity. Surprisingly, a significant proportion of clinically diagnosed DLB subjects had negative α-Syn-SAA tests. We suspect that this is due to challenges in diagnosing DLB on purely clinical grounds. Hyposmia was the strongest clinical predictor of α-Syn-SAA positivity. Our conclusion is that testing for hyposmia and α-Syn-SAA should both be considered as part of the diagnostic process, to improve the clinical diagnosis of individuals with potential DLB.
Coughlin DG, MacLeod KR, Middleton JS, Bozoki AC, Galvin JE, Irwin DJ, Lippa CF, Litvan I, Lopez OL, Berman S, Tsuang DW, Zabetian CP, Honig LS, Marder KS, Fleisher JE, Sabbagh M, Wint D, Taylor AS, Bekris L, Leverenz JB, Galasko D. Neurology. 2024 Aug 13;103(3):e209656. doi: 10.1212/WNL.0000000000209656. Epub 2024 Jul 16.PMID: 39013126