Lysia S. Forno

TL;DR: Neuropathological examination revealed moderate to severe depletion of pigmented nerve cells in the substantia nigra in each case, suggesting that a time‐limited insult to the nigrostriatal system can set in motion a self‐perpetuating process of neurodegeneration.

TL;DR: An independent family with hereditary early‐onset parkinsonism with dementia due to α‐synuclein triplication is presented, and studies of brain mRNA and soluble protein levels demonstrate a doubling of α‐ synuclein expression, consistent with molecular genetic data.

TL;DR: Brain membrane-associated, sodium dodecyl sulfate-soluble alpha-synuclein accumulations in Parkinson's disease and multiple system atrophy are regionally specific, suggesting that these sporadic alpha- Synucleinopathies, unlike familial Parkinsonism-dementia, are not associated with a simple global over-expression of the protein.

TL;DR: If the combination of ubiquitin-positive neuronal and tau 2-positive glial inclusions is found to be consistently present in FTD of motor neuron type, this feature will provide a firmer basis for this diagnosis than previously available.