Multiple system atrophy

Abstract

This is a practical guide to diagnosing and managing multiple system atrophy (MSA). We explain the newly published Movement Disorders Society Consensus Diagnostic Criteria, which include new 'Clinically Established MSA' and 'Possible Prodromal MSA' categories, hopefully reducing time to diagnosis. We then highlight the key clinical features of MSA to aid diagnosis. We include a list of MSA mimics with suggested methods of differentiation from MSA. Lastly, we discuss practical symptom management in people living with MSA, including balancing side effects, with the ultimate aim of improving quality of life.

Keywords: CEREBELLAR DEGENERATION; CLINICAL NEUROLOGY; MOVEMENT DISORDERS; MULTISYSTEM ATROPHY.

Figure 1

Pathological hallmarks of multiple system atrophy. (A) shows the most frequent α-synuclein positive inclusions are glial cytoplasmic inclusions. There are also glial intranuclear inclusions (as seen in B) to a much lesser extent. However, there are also neuronal cytoplasmic and intranuclear inclusions as seen in C, D. The glial cytoplasmic inclusion density in the grey matter regions, correlates with the extent of neuronal atrophy and gliosis.

Figure 2

2022 MDS criteria for clinically diagnosed MSA. * †‡See tables 1–3 for details. MSA, multiple system atrophy; PVR,post-voiding residual volume.