Parkinsonism Plus Syndromes

The ‘Parkinsonism-Plus’, or atypical Parkinsonian syndromes, are a group of movement disorders that present with significant Parkinsonism and may initially mimic idiopathic Parkinson’s Disease (IPD). They are however a diverse group of proteinopathies and their diagnosis is important, both in terms of management and prognosis. There are general “red flags” that may herald the diagnosis, including atypical rapid progression, poor response to domaminergic medications, and very early occurrence of features that do eventually arise in IPD. Examples of the latter include frequent falls with marked postural instability, autonomic failure or dementia/hallucinations. This chapter covers pathogenesis, diagnostic features and specific management issues of the four main Parkinsonism-Plus disorders: Multiple Systems Atrophy [MSA], Progressive Supranuclear Palsy [PSP], Corticobasal Degeneration [CBD] and Dementia with Lewy Bodies [DLB]. The diagnosis remains clinical and vigilant neurological monitoring along with a strong multidisciplinary team support are the cornerstone of management.