Update on Multiple System Atrophy

In Nature Reviews Neurology (Multiple system atrophy: insights into a rare and debilitating movement disorder, 13;232-243; 2017), Florian Krismer and Gregor Wenning from Innsbruck University Hospital provide a comprehensive overview of Multiple System Atrophy (MSA) linking the current state of knowledge from molecular-biological level and pathophysiology of MSA to disease dynamics and the impressive spectrum of therapeutic approaches. The thorough list of substances which failed to modify the course of disease brings to mind the challenging task of translative research in MSA. Although we have learned about the seeding ability of alpha synuclein from in-vitro and in-vivo experiments key events in the pathophysiological cascade of MSA are currently unknown. This pathophysiological cascade starts years before the onset of movement disorders.
In the recent years several studies have highlighted the importance of a careful neurovegetative anamnesis including questions focusing on symptoms of dysautonomia and REM sleep behavioral disorder (RBD). Patients concerned with dysautonomia and/or RBD should be offered a careful follow up in order to detect MSA early. Futile prostatic or bladder neck surgery is carried out in more than 40% of MSA patients
The systematic retrospective evaluation of the clinical course of MSA in 685 patients showed a median time course between onset and death of 7.51 years. Coon EA, Sletten DM, Suarez MD et al. showed that falls and incontinence within 3 years of disease onset or orthostatic hypotension within the first year are predictors of an unfavorable outcome. Clinical features and autonomic testing predict survival in multiple system atrophy. Brain. 2015; 138(Pt 12):3623-31
A cross-sectional study of patients with probable MSA from six academic centers in the US and Europe revealed that combined RBD screening and polysomnography that REM-Sleep-Behavior Disorder (RBD) can be found in the premotor phase of the majority of patients with MSA. Palma JA, Fernandez-Cordon C, Coon EA et al. Prevalence of REM sleep behavior disorder in multiple system atrophy: a multicenter study and meta-analysis. Clin Auton Res. 2015; 25(1): 69–75.
A prospective study in 175 patients, undertaken by Prof. Low and colleagues confirmed the relevance of a severe autonomic failure for an unfavorable outcome of MSA. Low PA, Reich SG, Jankovic J et al. Natural history of multiple system atrophy in the USA: a prospective cohort study. Lancet Neurol. 2015 Jul;14(7):710-9.