Small Molecules, α-Synuclein Pathology, and the Search for Effective Treatments in Parkinson's Disease.

Parkinson's disease (PD) is a progressive age-related neurodegenerative disorder affecting millions of people worldwide. Essentially, it is characterised by selective degeneration of dopamine neurons of the nigro-striatal pathway and intraneuronal aggregation of misfolded α-synuclein with formation of Lewy bodies and Lewy neurites. Moreover, specific small molecules of intermediary metabolism may have a definite pathophysiological role in PD.

New Therapeutic Paradigms in Neonatal Hypoxic-Ischemic Encephalopathy.

In neonates with hypoxic-ischemic encephalopathy (HIE), neuroprotection mediated by therapeutic hypothermia (TH) is the standard of care in developed nations; however TH may be not beneficial or contraindicated in developing world. Moreover, in most of the clinical trials, hypothermic neuroprotection was incomplete because many infants still die or suffer significant neurological sequelae. Therefore, finding innovative, neuroprotective compounds for this encephalopathy represents an important and urgent need.

Beneficial Effect of Phenytoin and Carbamazepine on Gene Expression and Mutant GFAP Folding in a Cellular Model of Alexander's Disease.

Alexander's disease (AxD) is a rare, usually relentlessly progressive disorder of astroglial cells in the central nervous system related to mutations in the gene encoding the type III intermediate filament protein, glial fibrillary acidic protein (GFAP). The pathophysiology of AxD is only partially understood. Available data indicate that an excessive GFAP gene expression may play a role.

Geographic differences in the incidence of Huntington's disease in Sardinia, Italy.

Background: The frequency of Huntington's disease (HD) may vary considerably, with higher estimates in non-Asian populations. We have recently examined the prevalence of HD in the southern part of Sardinia, a large Italian Mediterranean island that is considered a genetic isolate. We observed regional microgeographic differences in the prevalence of HD across the study area similar to those recently reported in other studies conducted in European countries.

Prevalence of Huntington's disease in Southern Sardinia, Italy.

Background: The frequency of Huntington's disease (HD) may vary considerably, with higher estimates in non Asian populations. In Italy, two recent studies performed in Ferrara county and Molise provided different prevalence estimates, varying from 4.2 × 10 to 10.