Progressive supranuclear palsy
Progressive supranuclear palsy (PSP) (or the Steele-Richardson-Olszewski syndrome, after the Canadian physicians who described it in 1963) is a degenerative disease
Degenerative disease
A degenerative disease, also called neurodegenerative disease, is a disease in which the function or structure of the affected tissues or organs will progressively deteriorate over time, whether due to normal bodily wear or lifestyle choices such as exercise or eating habits...

 involving the gradual deterioration and death of specific areas of the brain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...


Male refers to the biological sex of an organism, or part of an organism, which produces small mobile gametes, called spermatozoa. Each spermatozoon can fuse with a larger female gamete or ovum, in the process of fertilization...

s and female
Female is the sex of an organism, or a part of an organism, which produces non-mobile ova .- Defining characteristics :The ova are defined as the larger gametes in a heterogamous reproduction system, while the smaller, usually motile gamete, the spermatozoon, is produced by the male...

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

It has been described as a tauopathy
Tauopathies are a class of neurodegenerative diseases associated with the pathological aggregation of tau protein in the human brain.The best known of these illnesses is Alzheimer's disease , where tau protein is deposited within neurons in the form of neurofibrillary tangles...


Symptoms and signs

The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.

Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms.

Later symptoms and signs are dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...

 (typically including loss of inhibition and ability to organize information), slurring of speech
Relaxed pronunciation
Relaxed pronunciation is a phenomenon that happens when the syllables of common words are slurred together...

, 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
An eyelid is a thin fold of skin that covers and protects an eye. With the exception of the prepuce and the labia minora, it has the thinnest skin of the whole body. The levator palpebrae superioris muscle retracts the eyelid to "open" the eye. This can be either voluntarily or involuntarily...

 function, contracture
A muscle contracture is a permanent shortening of a muscle or joint.. It is usually in response to prolonged hypertonic spasticity in a concentrated muscle area, such as is seen in the tightest muscles of people with conditions like spastic cerebral palsy....

 of the facial muscles
Facial muscles
The facial muscles are a group of striated muscles innervated by the facial nerve that, among other things, control facial expression. These muscles are also called mimetic muscles.-Structure:...

, a backward tilt of the head with stiffening of the neck muscles
Table of muscles of the human body: Neck

, sleep disruption
Sleep disorder
A sleep disorder, or somnipathy, is a medical disorder of the sleep patterns of a person or animal. Some sleep disorders are serious enough to interfere with normal physical, mental and emotional functioning...

, urinary incontinence
Urinary incontinence
Urinary incontinence is any involuntary leakage of urine. It is a common and distressing problem, which may have a profound impact on quality of life. Urinary incontinence almost always results from an underlying treatable medical condition but is under-reported to medical practitioners...

 and constipation
Constipation refers to bowel movements that are infrequent or hard to pass. Constipation is a common cause of painful defecation...


The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the ophthalmoparesis
Ophthalmoparesis or ophthalmoplegia refers to paralysis of one or more extraocular muscles which are responsible for eye movements. It is a physical finding in certain neurologic illnesses.-Classification:...

 experienced by these patients mainly concerns voluntary eye movement. Patients tend to have difficulty looking down (a downgaze palsy
In medicine, palsy is the paralysis of a body part, often accompanied by loss of sensation and by uncontrolled body movements, such as shaking. Medical conditions involving palsy include cerebral palsy , brachial palsy , and Bell's palsy ....

) followed by the addition of an upgaze palsy. Involuntary eye movement, as elicited by Bell's phenomenon
Bell's phenomenon
Bell's phenomenon is a medical sign that allows observers to notice an upward and outward movement of the eye, when an attempt is made to close the eyes. The upward movement of the eye is present in the majority of the population, and is a defensive mechanism...

, 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
Nystagmus is a condition of involuntary eye movement, acquired in infancy or later in life, that may result in reduced or limited vision.There are two key forms of Nystagmus: pathological and physiological, with variations within each type. Nystagmus may be caused by congenital disorders,...

, except that they are not rhythmic in nature. Difficulties with convergence
Convergence (eye)
In ophthalmology, convergence is the simultaneous inward movement of both eyes toward each other, usually in an effort to maintain single binocular vision when viewing an object. This action is mediated by the medial rectus muscle, which is innervated by Cranial nerve III...

 (convergence insufficiency), 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 at short distances, the patient may complain of diplopia
Diplopia, commonly known as double vision, is the simultaneous perception of two images of a single object that may be displaced horizontally, vertically, or diagonally in relation to each other...

 (double vision) when reading.

Cardinal manifestations:
  • Supranuclear ophthalmoplegia
  • Neck dystonia
  • Parkinsonism
    Parkinsonism is a neurological syndrome characterized by tremor, hypokinesia, rigidity, and postural instability. The underlying causes of parkinsonism are numerous, and diagnosis can be complex...

  • Pseudobulbar palsy
    Pseudobulbar palsy
    Pseudobulbar palsy results from an upper motor neuron lesion to the corticobulbar pathways in the pyramidal tract. Patients have difficulty chewing, swallowing and demonstrate slurred speech...

  • Behavioral and Cognitive impairment
  • Imbalance and Difficulties walking
  • Frequent Falls


There is currently no effective treatment or cure
A cure is a completely effective treatment for a disease.The Cure is an English rock band.Cure, or similar, may also refer to:-Film and television:* The Cure , a short film starring Charlie Chaplin...

 for PSP, although some of the symptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...

s 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
Parkinson's disease
Parkinson's disease is a degenerative disorder of the central nervous system...

 because of the slowed movements and gait difficulty, or as Alzheimer's disease
Alzheimer's disease
Alzheimer's disease also known in medical literature as Alzheimer disease is the most common form of dementia. There is no cure for the disease, which worsens as it progresses, and eventually leads to death...

 because of the behavioral changes. It is one of a number of diseases collectively referred to as Parkinson plus syndrome
Parkinson plus syndrome
Parkinson-plus syndromes, also known as disorders of multiple system degeneration, are a group of neurodegenerative diseases featuring the classical features of Parkinson's disease with additional features that distinguish them from simple idiopathic Parkinson's disease...

s. A poor response to levodopa along with symmetrial onset can help differentiate this disease from PD.

Genetics and causal factors

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
A haplotype in genetics is a combination of alleles at adjacent locations on the chromosome that are transmitted together...

, located on chromosome 17
Chromosome 17 (human)
125px|rightChromosome 17 is one of the 23 pairs of chromosomes in humans. People normally have two copies of this chromosome. Chromosome 17 spans more than 81 million base pairs and represents between 2.5 and 3 % of the total DNA in cells.Identifying genes on each chromosome is an active area of...

, 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
Pollution is the introduction of contaminants into a natural environment that causes instability, disorder, harm or discomfort to the ecosystem i.e. physical systems or living organisms. Pollution can take the form of chemical substances or energy, such as noise, heat or light...

 are being investigated as other possible contributors to the cause of PSP.


The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles, which are clumps of tau protein
Tau protein
Tau proteins are proteins that stabilize microtubules. They are abundant in neurons of the central nervous system and are less common elsewhere, but are also expressed at very low levels in CNS astrocytes and oligodendrocytes...

, 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 structurally similar when they occur in the cerebral cortex. Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration
Corticobasal degeneration
Corticobasal degeneration or Corticobasal Ganglionic Degeneration is a rare progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia. It is characterized by marked disorders in movement and cognitive dysfunction...

. 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
    Basal ganglia
    The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...

    , particularly the subthalamic nucleus
    Subthalamic nucleus
    The subthalamic nucleus is a small lens-shaped nucleus in the brain where it is, from a functional point of view, part of the basal ganglia system. Anatomically, it is the major part of subthalamus. As suggested by its name, the subthalamic nucleus is located ventral to the thalamus. It is also...

    , substantia nigra
    Substantia nigra
    The substantia nigra is a brain structure located in the mesencephalon that plays an important role in reward, addiction, and movement. Substantia nigra is Latin for "black substance", as parts of the substantia nigra appear darker than neighboring areas due to high levels of melanin in...

     and globus pallidus
    Globus pallidus
    The globus pallidus also known as paleostriatum, is a sub-cortical structure of the brain. Topographically, it is part of the telencephalon, but retains close functional ties with the subthalamus - both of which are part of the extrapyramidal motor system...

  • the brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides;
  • the cerebral cortex
    Cerebral cortex
    The cerebral cortex is a sheet of neural tissue that is outermost to the cerebrum of the mammalian brain. It plays a key role in memory, attention, perceptual awareness, thought, language, and consciousness. It is constituted of up to six horizontal layers, each of which has a different...

    , particularly that of the frontal lobe
    Frontal lobe
    The frontal lobe is an area in the brain of humans and other mammals, located at the front of each cerebral hemisphere and positioned anterior to the parietal lobe and superior and anterior to the temporal lobes...

  • the dentate nucleus
    Dentate nucleus
    The dentate nucleus is located within the deep white matter of each cerebellar hemisphere, and it is the largest single structure linking the cerebellum to the rest of the brain. It is the largest and most lateral, or farthest from the midline, of the four pairs of deep cerebellar nuclei, the...

     of the cerebellum
    The cerebellum is a region of the brain that plays an important role in motor control. It may also be involved in some cognitive functions such as attention and language, and in regulating fear and pleasure responses, but its movement-related functions are the most solidly established...

  • and the spinal cord
    Spinal cord
    The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...

    , particularly the area where some control of the bladder and bowel resides.

Some consider PSP, corticobasal degeneration
Corticobasal degeneration
Corticobasal degeneration or Corticobasal Ganglionic Degeneration is a rare progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia. It is characterized by marked disorders in movement and cognitive dysfunction...

, and frontotemporal dementia
Frontotemporal dementia
Frontotemporal dementia is a clinical syndrome caused by degeneration of the frontal lobe of the brain and may extend back to the temporal lobe...

 to be variations of the same disease. Others consider them separate diseases. PSP has been shown to occasionally co-exist with Pick's disease
Pick's disease
Pick's disease, is a rare neurodegenerative disease that causes progressive destruction of nerve cells in the brain. Symptoms include loss of speech , and dementia. While some of the symptoms can initially be alleviated, the disease progresses and patients often die within two to ten years...


Classification and treatment

PSP cases are often split into two subgroups, PSP-Richardson, the classic type, and PSP-Parkinsonism, where a short-term response to levodopa can be obtained.

Two studies have suggested that rivastigmine
Rivastigmine is a parasympathomimetic or cholinergic agent for the treatment of mild to moderate dementia of the Alzheimer’s type and dementia due to Parkinson's disease. The drug can be administered orally or via a transdermal patch; the latter form reduces the prevalence of side effects, which...

 may help with cognitive aspects, but the authors of both studies have suggested a larger sampling be used.,


Patients with PSP usually seek or are referred to occupational therapy
Occupational therapy
Occupational therapy is a discipline that aims to promote health by enabling people to perform meaningful and purposeful activities. Occupational therapists work with individuals who suffer from a mentally, physically, developmentally, and/or emotionally disabling condition by utilizing treatments...

 and physiotherapy for balance and gait
Gait is the pattern of movement of the limbs of animals, including humans, during locomotion over a solid substrate. Most animals use a variety of gaits, selecting gait based on speed, terrain, the need to maneuver, and energetic efficiency...

 problems with reports of frequent falls. Unfortunately, evidence-based approaches to rehabilitation in PSP are lacking, and currently the majority of research on the subject consists of case reports involving only a small number of patients.

Case reports of rehabilitation programs for patients with PSP generally include limb-coordination activities, tilt-board balancing, gait training
Gait training
In its most general form, Gait training is the act of learning how to walk. However, the term is more often used in reference to a person learning how to walk again after injury or with a disability...

, strength training with progressive resistive exercises and isokinetic exercises and stretching of the neck muscles. While some case reports suggest that physiotherapy can offer improvements in balance and gait of patients with PSP, the results cannot be generalized across all patients with PSP as each case report only followed one or two patients. The observations made from these case studies can be useful, however, in helping to guide future research concerning the effectiveness of balance and gait training programs in the management of PSP.

Individuals with PSP are often referred to occupational therapists to help manage their condition and to help enhance their independence. This may include being taught to use mobility aids
Mobility aids
Mobility aids are devices designed to assist walking or otherwise improve the mobility of people with a mobility impairment.There are various walking aids which can help with impaired ability to walk and wheelchairs or mobility scooters for more severe disability or longer journeys which would...

. Due to their tendency to fall backwards, the use of a walker, particularly one that can be weighted in the front, is recommended over a cane. The use of an appropriate mobility aid will help to decrease the individual’s risk of falls and make them safer to ambulate independently in the community.
Due to their balance problems and irregular movements individuals will need to spend time learning how to safely transfer in their homes as well as in the community. This may include arising from and sitting in chairs safely.

Due to the progressive nature of this disease, all individuals eventually lose their ability to walk and will need to progress to using a wheelchair.

Notable cases

  • Nobel Laureate Dr Abdus Salam
    Abdus Salam
    Mohammad Abdus Salam, NI, SPk Mohammad Abdus Salam, NI, SPk Mohammad Abdus Salam, NI, SPk (Urdu: محمد عبد السلام, pronounced , (January 29, 1926– November 21, 1996) was a Pakistani theoretical physicist and Nobel laureate in Physics for his work on the electroweak unification of the...

     (1926–1996), a world renowned scientist and first and only Muslim Pakistani man to win the Nobel Prize in Physics (1979) for his gauge unification of weak and electromagnetic interactions, which forms the basis of the Standard Model in particle physics.
  • Teel Bivins
    Teel Bivins
    Miles Teel Bivins served as United States ambassador to Sweden between 2004 and 2006. He was confirmed by the U.S. Senate on May 21, 2004, and sworn in at Washington D.C., on May 26. He presented his credentials to King Carl XVI Gustaf in Stockholm on June 9...

     (1947–2009), a former U.S. Ambassador to Sweden and a former member of the Texas State Senate from Amarillo
    Amarillo, Texas
    Amarillo is the 14th-largest city, by population, in the state of Texas, the largest in the Texas Panhandle, and the seat of Potter County. A portion of the city extends into Randall County. The population was 190,695 at the 2010 census...

    , Texas
    Texas is the second largest U.S. state by both area and population, and the largest state by area in the contiguous United States.The name, based on the Caddo word "Tejas" meaning "friends" or "allies", was applied by the Spanish to the Caddo themselves and to the region of their settlement in...

    , died at the age of sixty-one of progressive supranuclear palsy, first diagnosed in 2006, while he was in Stockholm, Sweden
    Sweden , officially the Kingdom of Sweden , is a Nordic country on the Scandinavian Peninsula in Northern Europe. Sweden borders with Norway and Finland and is connected to Denmark by a bridge-tunnel across the Öresund....

  • Leonard Krieger
    Leonard Krieger
    Leonard Krieger was an American historian of modern Europe, particularly known as an author on Germany. He was influential as an intellectual historian, and particularly for his discussion of historicism...

     (1918–1990), was an American historian of modern Europe, particularly known as an author on Germany. He was influential as an intellectual historian, and particularly for his discussion of historicism. Krieger taught at Columbia University and The University of Chicago until his death from PSP.
  • Actor Dudley Moore
    Dudley Moore
    Dudley Stuart John Moore, CBE was an English actor, comedian, composer and musician.Moore first came to prominence as one of the four writer-performers in the ground-breaking comedy revue Beyond the Fringe in the early 1960s, and then became famous as half of the highly popular television...

    , 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
    Lee Philips
    Lee Philips was an actor and director.Philips' acting career started on Broadway, and peaked with a starring role as Michael Rossi in the film adaptation of Peyton Place opposite Lana Turner....

    , the 1950s actor-turned-director of such shows as Peyton Place
    Peyton Place (film)
    Peyton Place is a 1957 American drama film directed by Mark Robson. The screenplay by John Michael Hayes is based on the bestselling 1956 novel of the same name by Grace Metalious.-Plot:...

    and The Ghost & Mrs. Muir
    The Ghost & Mrs. Muir (TV series)
    The Ghost & Mrs. Muir is a situation comedy based on the 1947 film The Ghost and Mrs. Muir, which was based on the 1945 novel by R.A. Dick. It starred Hope Lange as Carolyn Muir, a young widow who rents Gull Cottage, near the fictional fishing village of Schooner Bay, Maine along with her two...

    , also died after suffering from this disease.
  • American singer Teresa Brewer
    Teresa Brewer
    Teresa Brewer was an American pop singer whose style incorporated elements of country, jazz, R&B, musicals and novelty songs. She was one of the most prolific and popular female singers of the 1950s, recording nearly 600 songs. Born Theresa Breuer in Toledo, Ohio, Brewer died of a neuromuscular...

     ("Music! Music! Music!
    Music! Music! Music!
    "Music! Music! Music!" is a popular song written by Stephen Weiss and Bernie Baum and published in 1949.The biggest-selling version of the song was recorded by Teresa Brewer on December 20, 1949, and released by London Records as catalog number 604. It became a #1 hit and a million-seller in 1950...

    ), 76, died of this disease on October 17, 2007.
  • Film Director
    Film director
    A film director is a person who directs the actors and film crew in filmmaking. They control a film's artistic and dramatic nathan roach, while guiding the technical crew and actors.-Responsibilities:...

     and Playwright
    A playwright, also called a dramatist, is a person who writes plays.The term is not a variant spelling of "playwrite", but something quite distinct: the word wright is an archaic English term for a craftsman or builder...

     Joshua Logan
    Joshua Logan
    Joshua Lockwood Logan III was an American stage and film director and writer.-Early years:Logan was born in Texarkana, Texas, the son of Susan and Joshua Lockwood Logan. When he was three years old his father committed suicide...

  • Abe Pollin
    Abe Pollin
    Abe Pollin was the owner of a number of professional sports teams including the Washington Capitals in the National Hockey League , the Washington Mystics in the Women's National Basketball Association , and the Washington Wizards in the National Basketball Association...

    , D.C. sports mogul, longest tenured owner of an NBA franchise (since 1964) Washington Wizards
    Washington Wizards
    The Washington Wizards are a professional basketball team based in Washington, D.C., previously known as Washington Bullets. They play in the National Basketball Association .-Early years:...

    , 85, died of this disease on November 24, 2009.
  • Dr. Anne Turner, who was the basis of the BBC drama Short Stay in Switzerland about assisted suicide.
  • Jon Hassler
    Jon Hassler
    Jon Hassler was an American writer and teacher known for his novels about small-town life in Minnesota. He held the positions of Regents Professor Emeritus and Writer-in-Residence at St...

    , a famous book writer
  • Richard Rainwater
    Richard Rainwater
    -Early life:The son of a wholesale grocer, he grew up in Fort Worth, Texas. He graduated from the University of Texas with a degree in mathematics, where he was a member of the Tau chapter of the Kappa Sigma fraternity. He was recognized by the national organization in 1996 as Kappa Sigma Man of...

    , an investor

Support groups

Several international organizations serve the needs of patients with PSP and their families and support research. The Foundation for PSP, CBD and Related Brain Diseases is based in the US and the PSP Association (PSP-Europe Association) is based in the UK. The PSP-France association is based in Paris.

External links

The source of this article is wikipedia, the free encyclopedia.  The text of this article is licensed under the GFDL.