Multiple system atrophy: a disorder targeting the brainstem control of survival

Excerpt

Multiple system atrophy (MSA) is a neurodegenerative synucleinopathy characterized by severe autonomic failure, manifested primarily by orthostatic hypotension (OH), neurogenic bladder and sexual dysfunction associated with parkinsonism, ataxia, or both. One salient feature of MSA is the coexistence of respiratory dysfunction (including sleep apnea and laryngeal stridor) and rapid eye movement (REM) sleep behavior disorder. This indicates that MSA neuropathology selectively targets brainstem areas participating in the integrative control of autonomic, respiratory, and arousal/sleep functions. Recently, rodent studies using selective activation or inactivation of specific brainstem neurons groups have provided further insight into the mechanisms controlling these vital functions and support the neuropathological evidence of extensive brainstem involvement in MSA. …