Rapid Eye Movement Sleep Behavior Disorder with Clinical Dementia with Lewy Bodies Phenotype but Lacking Postmortem Central Nervous System Synuclein Pathology: Alternate Etiologies - PubMed
4 days ago
- #synuclein pathology
- #dementia with Lewy bodies
- #REM sleep behavior disorder
- A case study of an 80-year-old man with idiopathic REM sleep behavior disorder (IRBD) and clinical dementia with Lewy bodies phenotype, but lacking postmortem central nervous system synuclein pathology.
- The patient had a negative cerebrospinal fluid (CSF) α-synuclein seeding amplification assay (SAA) and was followed for 16 years until his death at age 96.
- Neuropathology revealed aging-related tau astrogliopathy, limbic-predominant age-related TDP-43 encephalopathy, primary age-related tauopathy, and diffuse small vessel disease, with nearly absent Lewy pathology.
- The study suggests that RBD can arise independently of α-synuclein pathology, possibly due to dysregulation of brainstem REM sleep-related structures from age-related proteinopathies or venlafaxine use.
- CSF α-synuclein SAA may be useful in neuroprotective trials to exclude IRBD patients without synuclein pathology.