Progressive Supranuclear Palsy 

  • Author: Eric R Eggenberger, DO, MS, FAAN; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Jan 5, 2010
 

Background

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a neurodegenerative disease that affects cognition, eye movements, and posture.[1] PSP was first described as a clinicopathologic entity in 1964. Characteristics include supranuclear, primarily vertical, gaze dysfunction accompanied by extrapyramidal symptoms and cognitive dysfunction. The disease usually develops after the sixth decade of life, and the diagnosis is purely clinical. Currently, no therapy is proven to be effective.

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Pathophysiology

  • Pathologically, PSP is defined by the accumulation of neurofibrillary tangles in the brain.[2] Different rates and patterns of the accumulation of phosphorylated tau protein may account for the variation in clinical phenomena seen in patients with PSP.
  • Liao and colleagues suggest that abnormal otolith-mediated reflexes may be at least partly responsible for the frequent falls in patients with PSP. They found that during near viewing, the translational vestibulo-ocular reflex responses in patients with PSP were, on average, only 12% of those of control subjects (p = 0.001). The amplitude of vestibular-evoked myogenic potentials was also significantly reduced in PSP patients compared with normal controls.[3]
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Epidemiology

Frequency

United States

A population-based study in New Jersey by Golbe and colleagues revealed an overall prevalence of 1.39 cases per 100,000 population. The male prevalence was 1.53 cases, while the female prevalence was 1.23 cases (both figures are per 100,000 population); this finding was in accordance with a previously noted slight male preponderance. The adjusted prevalence ratio among patients older than 55 years was 7 cases per 100,000 population.[4]

Prevalence data derived from tertiary centers suggest that PSP affects 4-6% of patients with parkinsonism.[5]

International

Incidence has been assessed in Perth, Australia; crude incidence rates are 3-4 per million cases per year, approximately 5% of the incidence of Parkinson disease.[6]

Mortality/Morbidity

  • PSP is usually fatal within approximately 6 years of onset, with a range of 2-17 years, based on cohort patients dying under surveillance; life table analysis among Golbe's entire cohort revealed a median disease duration of 9.7 years. Conflicting reports exist regarding the influence of age at diagnosis on survival; Golbe found a tendency for younger patients to survive longer, although this is not a uniform finding among other studies.[4, 7]
  • The primary causes of death in patients with PSP are infections and pulmonary complications (eg, pneumonia) that are frequently related to immobility. Often, the primary morbidity relates to imbalance leading to immobility, although dementia, visual symptoms, and dysphagia are major concerns. Approximately 50% of patients with PSP require some aid to walk within 3 years of the initial symptoms. The usual interval from initial symptom occurrence to the need for a cane or a walker is 3.1 years, and the interval to confinement to a chair or bed is 8.2 years.[4]

Race

Most reported cases have been in whites. The affected cohort in Golbe's 1988 study was comprised entirely of white persons; however, the survey population included only 5.7% black individuals, thus preventing any meaningful analysis regarding race.[4]

Sex

PSP has a slight male predominance in most studies. According to Kristensen, the male-to-female ratio is 1.5:1.[8]

Age

The mean age at onset is approximately 63 years, with a range of 44-75 years.[4, 7] The median interval between onset and diagnosis is 3 years, with a range of 6 months to 9 years.

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Contributor Information and Disclosures
Author

Eric R Eggenberger, DO, MS, FAAN  Professor, Vice-Chairman, Department of Neurology and Ophthalmology, Colleges of Osteopathic Medicine and Human Medicine, Michigan State University; Director of Michigan State University Ocular Motility Laboratory; Director of National Multiple Sclerosis Society Clinic, Michigan State University

Eric R Eggenberger, DO, MS, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Zeba F Vanek, MD, MBBS, DCN  Associate Professor of Neurology, David Geffen School of Medicine at UCLA; Director, UCLA Spasticity Clinic

Zeba F Vanek, MD, MBBS, DCN is a member of the following medical societies: Movement Disorders Society

Disclosure: Nothing to disclose.

David Clark, DO  Chief Neurology Resident, Department of Neurology and Ophthalmology, Michigan State University College of Human Medicine

David Clark, DO is a member of the following medical societies: American Academy of Neurology and American Osteopathic Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Joseph Quinn, MD  Assistant Professor, Department of Neurology, Portland VA Medical Center, Oregon Health Sciences University

Joseph Quinn, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience, and Society for Pediatric Radiology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Nestor Galvez-Jimenez, MD, MSc, MHA  Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

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