What are the neuropathologic findings in Huntingtons disease?
The most important pathologic findings in Huntingtons disease are neuronal loss and gliosis in the cortex and striatum, particularly the caudate nucleus.
Chorea seems to be primarily related to loss of medium spiny striatal neurons projecting to the lateral pallidum.
This results in functional hypoactivity of the STN with consequent hyperactivity of the thalamic tier.
Cortical thinning in various parts such as sensorimotor, parietal, occipital, and inferior temporal lobes is now being recognized in Huntingtons disease and has been associated with earlier cognitive symptoms.