Progressive Supranuclear Palsy and Corticobasal Syndrome

Alexander Pantelyat, MD, FAAN Movement Disorders p. 1364-1378 October 2022, Vol.28, No.5 doi: 10.1212/CON.0000000000001158
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PURPOSE OF REVIEW The differential diagnosis of parkinsonism (tremor, rigidity, bradykinesia, and gait difficulty/postural instability) is broad and includes two neurodegenerative conditions that exist on a clinicopathologic spectrum: progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Early in their clinical course, PSP and CBS may be difficult to distinguish from Parkinson disease and several other illnesses, but it is crucial to do so because of implications for management and prognosis.

RECENT FINDINGS Early accurate diagnosis of PSP and CBS remains a challenge because of heterogeneity in presenting symptoms and high frequency of coexisting pathologies (especially Alzheimer disease and vascular disease). It is increasingly recognized that patients with PSP, CBS, and other parkinsonian disorders require multidisciplinary care for optimal outcomes. With the recent proliferation of biomarker studies and therapeutic trials for tauopathies, there is growing hope that better treatments for PSP and CBS are on the horizon.

SUMMARY Although PSP and CBS currently lack disease-modifying therapies, it is important to diagnose them as early as possible so that the patient can benefit from the many available symptomatic therapies, support groups, and a growing number of clinical trials.

Address correspondence to Dr Alexander Pantelyat, 600 N Wolfe St, Meyer 6-181C, Baltimore, MD 21287, [email protected].

RELATIONSHIP DISCLOSURE: Dr Pantelyat has received personal compensation in the range of $5000 to $9999 for serving as a scientific advisory board member for MedRhythms, Inc, and in the range of $10,000 to $49,999 for serving as an expert witness for Kelly & Ignoffo Law Group. The institution of Dr Pantelyat has received research support from the National Institutes of Health/National Institute on Aging.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Pantelyat discusses the unlabeled/investigational use of dextromethorphan/quinidine or selective serotonin reuptake inhibitors (SSRIs) for the treatment of pseudobulbar affect in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS); methylphenidate or modafinil for the treatment of apathy in PSP and CBS; miglustat for Niemann-Pick disease type C; onabotulinumtoxinA for the treatment of sialorrhea, cervical and limb dystonia/spasticity, muscle pain, and eyelid opening apraxia/blepharospasm in PSP and CBS; and SSRIs for the treatment of depression and anxiety in PSP and CBS.

© 2022 American Academy of Neurology.