Multiple system atrophy (MSA) may be the most feared of diseases affecting the autonomic nervous system. Accordingly, concerted efforts are underway to elucidate further its pathophysiology with the objective of discovering effective neuroprotective interventions that can slow or halt its progression. Although the epidemiology of MSA is sporadic rather than familial, recently reported associations with COQ2 and C9orf72 suggest that in some cases genetic factors may influence the risk of developing the disease. Research has also identified prion-like characteristics of the expansion of pathologic α-synuclein in the central nervous system. A large clinical trial of rifampicin, which disaggregates and inhibits the formation of α-synuclein fibrils, however, was clearly negative. Other research has shown the importance of recognizing and treating vocal cord paralysis, which is associated with adverse prognosis.
- Multiple system atrophy
- Prion diseases
ASJC Scopus subject areas
- Clinical Neurology
- Cellular and Molecular Neuroscience
- Endocrine and Autonomic Systems