Recognizing and treating atypical Parkinson disorders

Though less common than Parkinson's disease (PD), the atypical Parkinson disorders such as such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration are increasingly recognized and important to distinguish from PD. Atypical or "Parkinson-plus" disorders are multisystem disorders and generally progress more rapidly and respond poorly to current therapies compared to PD. Recent advances in our understanding of the pathophysiology of these disorders, however, have generated new interest in the development of novel diagnostics and disease-modifying therapeutics aimed at identifying and treating these disorders. In this review we discuss the clinical approach to the atypical Parkinson disorders and the recent developments in diagnostic and research criteria that take into account the phenotypic heterogeneity and advances in our understanding of the pathophysiology of these disorders.