Progressive Supranuclear Palsy, Corticobasal Degeneration, and Multiple System Atrophy

Paul Greene, MD Movement Disorders p. 919-935 August 2019, Vol.25, No.4 doi: 10.1212/CON.0000000000000751
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PURPOSE OF REVIEW Patients who have parkinsonian features, especially without tremor, that are not responsive to levodopa, usually have one of these three major neurodegenerative disorders rather than Parkinson disease: progressive supranuclear palsy (PSP), multiple system atrophy (MSA), or corticobasal degeneration (CBD). Each of these disorders eventually develops signs and symptoms that distinguish it from idiopathic Parkinson disease, but these may not be present at disease onset. Although these conditions are not generally treatable, it is still important to correctly diagnose the condition as soon as possible.

RECENT FINDINGS In recent years, it has been increasingly recognized that the symptoms of these diseases do not accurately predict the pathology, and the pathology does not accurately predict the clinical syndrome. Despite this, interest has grown in treating these diseases by targeting misfolded tau (in the case of PSP and CBD) and misfolded α-synuclein (in the case of MSA).

SUMMARY Knowledge of the characteristic signs and symptoms of PSP, MSA, and CBD are essential in diagnosing and managing patients who have atypical parkinsonian syndromes.

Address correspondence to Dr Paul Greene, Mt. Sinai School of Medicine, 5 E 98th St, New York, NY 10029, [email protected].

RELATIONSHIP DISCLOSURE: Dr Greene reports no disclosure.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Greene discusses the unlabeled/investigational use of medications for the treatment of atypical parkinsonism, none of which have been approved by the US Food and Drug Administration.

© 2019 American Academy of Neurology.