I was not really active in the last few months on my forum, wiki, homepage nor delivered a lot of news version of my Joomla! plugins. This is due to my mother (67 years old) slowly dying in hospital…I’ve spent the last 5 days more than 30 hours in hospital taking care of her. She was found in 2006 to have a PSP (Progressive Supranuclear Palsy), and was not able to walk since. Her disease has reach another level on 19th November 2009, when she was no more able to swallow any liquids, got a light brain attack.

Swallowing is affected, and drastic measures such as feeding tubes will be utilized to ensure that she is able to get much needed nutrients. Improper swallowing otherwise may lead to lung infections or pneumonia.

Her lifetime expectancy is now counted in days, weeks or months. I won’t be active in the next few weeks, as I can’t simply do too much things at this time efficiently.

Progressive Supranuclear Palsy (PSP)

Also named the Steele-Richardson-Olszewski syndrome), after the Canadian physicians who described it in 1963) is a rare degenerative disease involving the gradual deterioration and death of selected areas of the brain. Both males and females 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, general slowing of movement, and visual symptoms.

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, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down.

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.

The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the ophthalmoplegia experienced by these patients mainly concerns voluntary eye movement. Involuntary eye movement, as elicited by Bell's phenomenon, for instance, may be closer to normal. On close inspection, eye movements called "square wave jerks" may be visible when the patient fixes at distance. These are fine movements, similar to nystagmus, except that they are not rhythmic in nature. Difficulties with convergence, where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus on things at near, the patient may complain of double vision when reading.

Cardinal Manifestations:

  • Supranuclear ophthalmoplegia
  • Neck dystonia
  • Parkinsonism
  • Pseudobulbar palsy
  • Behavioral and Cognitive impairment
  • Imbalance and Difficulties walking
  • Frequent Falls

Prognosis

There is currently no effective treatment or cure for PSP, although some of the symptoms can respond to nonspecific measures. The average age at symptoms onset is 63 and survival from onset 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 a brain cell's internal structural skeleton. These tangles are often different from those seen in Alzheimer's disease, but may be similar when they occur in the cerebral cortex.Lewy bodies are seen in some cases, but it is not clear whether this is a variant or an independent co-existing process.

The principal areas of the brain affected are:

  • the basal ganglia, particularly the subthalamic nucleus, substantia nigra and globus pallidus;
  • the brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides;
  • the cerebral cortex, particularly that of the frontal lobes;
  • the dentate nucleus of the cerebellum;
  • and the spinal cord, particularly the area where some control of the bladder and bowel resides.

Some consider PSP, corticobasal degeneration, and frontotemporal dementia to be variations of the same disease.[6] Others consider them separate diseases.[7]

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 about two-thirds of the general population. Therefore, the H1 haplotype appears to be necessary but not sufficient to cause PSP. Other genes, as well as environmental toxins are being investigated as other possible contributors to the cause of PSP.

Notable cases

  • Teel Bivins (1947-2009), a former U.S. Ambassador to Sweden and a former member of the Texas State Senate from Amarillo, Texas, died at the age of sixty-one of progressive supranuclear palsy, first diagnosed in 2006, while he was in Stockholm, Sweden.[9]
  • Actor Dudley Moore, who suffered from progressive supranuclear palsy, increased public awareness of this disease. He died on March 27, 2002 at the age of sixty-six from its complications.
  • Lee Philips, the 1950s actor-turned-director of such shows as Peyton Place and The Ghost & Mrs. Muir, also died after suffering from this disease.
  • American singer Teresa Brewer ("Music! Music! Music!"), 76, died of this disease on October 17, 2007.
  • Film Director and Playwright Joshua Logan
  • Abe Pollin, D.C. sports mogul, longest tenured owner of an NBA franchise (since 1964) Washington Wizards, 85, died of this disease on November 24, 2009.
  • Dr. Anne Turner, who was the basis of the BBC drama A Short Stay in Switzerland about assisted suicide.[10]

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 (PSP-Europe Association) is based in the UK.

References

  1. ^ Richardson JC, Steele J, Olszewski J (1963). "Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of 'heterogeneous system degeneration'". Transactions of the American Neurological Association 88: 25–9. PMID 14272249.
  2. ^ Steele JC, Richardson JC, Olszewski J (April 1964). "Progressive supranuclear palsy: a heterogeneous degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia". Archives of Neurology 10: 333–59. PMID 14107684. http://archneur.ama-assn.org/cgi/pmidlookup?view=long&pmid=14107684.
  3. ^ Amano N, Iwabuchi K, Yokoi S (January 1989). "[The reappraisal study of the ultrastructure of Alzheimer's neurofibrillary tangles in three cases of progressive supranuclear palsy]" (in Japanese). Nō to Shinkei 41 (1): 35–44. PMID 2655673.
  4. ^ Uchikado H, DelleDonne A, Ahmed Z, Dickson DW (April 2006). "Lewy bodies in progressive supranuclear palsy represent an independent disease process". Journal of Neuropathology and Experimental Neurology 65 (4): 387–95. doi:10.1097/01.jnen.0000218449.17073.43. PMID 16691119.
  5. ^ Keith-Rokosh J, Ang LC (November 2008). "Progressive supranuclear palsy: a review of co-existing neurodegeneration". The Canadian Journal of Neurological Sciences 35 (5): 602–8. PMID 19235444. http://cjns.metapress.com/openurl.asp?genre=article&issn=0317-1671&volume=35&issue=5&spage=602.
  6. ^ Kertesz A, Munoz D (2004). "Relationship between frontotemporal dementia and corticobasal degeneration/progressive supranuclear palsy". Dementia and Geriatric Cognitive Disorders 17 (4): 282–6. doi:10.1159/000077155. PMID 15178937.
  7. ^ Hattori M, Hashizume Y, Yoshida M (August 2003). "Distribution of astrocytic plaques in the corticobasal degeneration brain and comparison with tuft-shaped astrocytes in the progressive supranuclear palsy brain". Acta Neuropathologica 106 (2): 143–9. doi:10.1007/s00401-003-0711-4. PMID 12732936.
  8. ^ Online 'Mendelian Inheritance in Man' (OMIM) 601104
  9. ^ ""Teel Bivins Services Held Today in Amarillo, October 29, 2009". Texas Insider. http://texasinsider.org. Retrieved October 30, 2009.
  10. ^ "Dr Anne Turner". Dignity in Dying. http://www.dignityindying.org.uk/personal-stories/uk/south-west/bath/dr-anne-turner-story-9.html. Retrieved 2009-01-25.

External links

© Copyright Authors - Source : http://en.wikipedia.org/wiki/Progressive_supranuclear_palsy – Article under CC-BY-SA

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