Progressive supranuclear palsy

Revision as of 09:35, 12 January 2009 by Zorkun (talk | contribs)
(diff) ← Older revision | Latest revision (diff) | Newer revision → (diff)
Jump to navigation Jump to search
Progressive supranuclear palsy
ICD-10 G23.1
ICD-9 333.0
OMIM 601104
DiseasesDB 10723
eMedicine neuro/328 
MeSH D013494

WikiDoc Resources for Progressive supranuclear palsy

Articles

Most recent articles on Progressive supranuclear palsy

Most cited articles on Progressive supranuclear palsy

Review articles on Progressive supranuclear palsy

Articles on Progressive supranuclear palsy in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Progressive supranuclear palsy

Images of Progressive supranuclear palsy

Photos of Progressive supranuclear palsy

Podcasts & MP3s on Progressive supranuclear palsy

Videos on Progressive supranuclear palsy

Evidence Based Medicine

Cochrane Collaboration on Progressive supranuclear palsy

Bandolier on Progressive supranuclear palsy

TRIP on Progressive supranuclear palsy

Clinical Trials

Ongoing Trials on Progressive supranuclear palsy at Clinical Trials.gov

Trial results on Progressive supranuclear palsy

Clinical Trials on Progressive supranuclear palsy at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Progressive supranuclear palsy

NICE Guidance on Progressive supranuclear palsy

NHS PRODIGY Guidance

FDA on Progressive supranuclear palsy

CDC on Progressive supranuclear palsy

Books

Books on Progressive supranuclear palsy

News

Progressive supranuclear palsy in the news

Be alerted to news on Progressive supranuclear palsy

News trends on Progressive supranuclear palsy

Commentary

Blogs on Progressive supranuclear palsy

Definitions

Definitions of Progressive supranuclear palsy

Patient Resources / Community

Patient resources on Progressive supranuclear palsy

Discussion groups on Progressive supranuclear palsy

Patient Handouts on Progressive supranuclear palsy

Directions to Hospitals Treating Progressive supranuclear palsy

Risk calculators and risk factors for Progressive supranuclear palsy

Healthcare Provider Resources

Symptoms of Progressive supranuclear palsy

Causes & Risk Factors for Progressive supranuclear palsy

Diagnostic studies for Progressive supranuclear palsy

Treatment of Progressive supranuclear palsy

Continuing Medical Education (CME)

CME Programs on Progressive supranuclear palsy

International

Progressive supranuclear palsy en Espanol

Progressive supranuclear palsy en Francais

Business

Progressive supranuclear palsy in the Marketplace

Patents on Progressive supranuclear palsy

Experimental / Informatics

List of terms related to Progressive supranuclear palsy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Please Take Over This Page and Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [2] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Overview

Progressive supranuclear palsy (PSP) (or the Steele-Richardson-Olszewski syndrome, after the Canadian physicians who described it in 1963[3] [4]) is a rare degenerative disorder involving the gradual deterioration and death of selected areas of the brain.

The sexes are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000 population have PSP.

Symptoms and signs

The initial symptom in two-thirds of cases is loss of balance and falls. Other common early symptoms are changes in personality or general slowing of movement.

Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, most specifically in the downward direction.

Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.

Prognosis

There is currently no effective treatment for PSP, although some of the symptoms can respond to nonspecific measures. The average age at symptoms onset is 63 and survival from that point averages 7 years with a wide variance.

Differential diagnosis

PSP is frequently misdiagnosed as Parkinson's disease because of the slowed movements and gait difficulty, or as Alzheimer's Disease because of the behavioral changes. It is one of a number of diseases collectively referred to as Parkinson plus syndromes.

Pathophysiology

The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles, which are clumps of tau protein, a normal part of brain cell's internal structural skeleton.

The principal areas of the brain affected are:

Genetics

Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17, has been linked to PSP. Nearly all people with PSP received a copy of that variant from each parent, but this is true of only about two-thirds of the general population. Therefore, the H1 haplotype appears to be necessary but not sufficient to cause PSP. Other genes and environmental toxins are being investigated as other contributors to the cause of PSP.

Notable cases

Actor Dudley Moore, who suffered from progressive supranuclear palsy, increased public awareness of this disease. He died on March 27, 2002 from its complications. Lee Philips, 1950s actor-turned-director of such shows as Peyton Place and The Ghost and Mrs. Muir also died after suffering from this disease.

Support groups

Several international organizations serve the needs of patients with PSP and their families and support research. The Society for PSP ("CurePSP") is based in the US and the PSP Association is based in the UK.

Reference

  1. ^ Richardson JC, Steele J, Olszewski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of "heterogeneous system degeneration". Trans Am Neurol Assoc 1963;88:25-9. PMID 14272249.
  2. ^ Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy: a heterogeneous degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-359. PMID 14107684.
  3. ^ Online Mendelian Inheritance in Man (OMIM) 601104

External links

Template:Diseases of the nervous system Template:SIB it:Paralisi sopranucleare progressiva fi:Progressiivinen supranukleaarinen halvaus Template:WH Template:WS