Table 1 Core neuropathological and clinical features of α-synucleinopathies
α-synucleinopathy | Neuropathology | Clinical features | Cognitive features |
|---|---|---|---|
PD, PD-D | Mild-to-moderate dopaminergic neuronal loss in the ventrolateral part of the substantia nigra; limbic and neocortical α-syn pathology1 | Bradykinesia, rigidity, resting tremor, non-motor features (e.g., olfactory loss, RBD, depression, cognitive impairment) | Impairment in executive domain in PD; deficits in multiple domains in PD-D |
DLB | Moderate-to-severe dopaminergic neuronal loss in the ventrolateral part of the substantia nigra; limbic and neocortical α-syn pathology | Dementia, fluctuating cognition, visual hallucinations, RBD, parkinsonism | Significant impairment in executive, language, visuo-spatial domains |
MSA | Glial cytoplasmatic inclusions in the basal ganglia, substantia nigra, pontine nuclei, medulla, cerebellum | Dysautonomia, parkinsonism, cerebellar syndrome, cognitive impairment | Significant deficit in executive (i.e., shifting) domain |