This chapter contrasts the prion-like behavior of alphasynuclein in Parkinson’s disease with other prion diseases.
In prion diseases, the infectious, amyloid form of the
protein induces the corresponding normally folded protein
to assume the toxic fold, spreading the disease and slowly
destroying the brains of infected patients. Lewy pathology
in Parkinson’s disease appears to spread in a manner
similar to that of other prion diseases, typically following
the Braak staging scheme, starting in the vagus nerve and
in the olfactory bulb and spreading to the basal ganglia,
slowly ravaging the substantia nigra. Ultimately, the
pathology spreads through the limbic system and into the
neocortex, eventually leading to dementia. While the
pathology in the majority of Parkinson’s disease patients
appears to follow the Braak staging scheme, the spread of
pathology in many familial cases and in dementia with
Lewy bodies can differ. Different “strains” with varying
alpha-synuclein amyloid structure might be related to
disease severity, as well as how the pathology spreads. CLICK TO REVIEW