Clinical presentation

Abstract

MSA is clinically characterised by two major motor features, namely, parkinsonism and cerebellar ataxia. These motor features serve as the basis for classifying patients with predominant parkinsonism as MSA-P, and with cerebellar ataxia as MSA-C. Poorly l-dopa-responsive akinetic-rigid parkinsonism is the most common presenting feature of MSA, present in 60–82 % of patients already from disease onset, while cerebellar features occur in 10–30 % of cases at symptom onset. Interestingly, MSA-C outnumbered MSA-P in a Japanese cohort, whereas in Europe, America and Africa, MSA-P is the most common phenotype. Beyond parkinsonian and cerebellar features, MSA tipically presents with a variegate spectrum of autonomic as well as pyramidal, cognitive and behavioural symptoms, highlighting the peculiar multisystemic nature of this neurodegenerative disease. In the present chapter, motor as well non motor features and clinical red flags of MSA will be sistematically treated.