Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome,(after the Canadian physicians who described it in 1963) is a brain disorder that causes serious problems with walking, balance and eye movements. Progressive supranuclear palsy results from deterioration of cells in areas of your brain that control movement.
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Progressive supranuclear palsy is a rare disorder that slowly worsens over time. Although it’s not life-threatening itself, progressive supranuclear palsy can lead to life-threatening complications, such as pneumonia and swallowing problems.
Because there’s no cure for progressive supranuclear palsy, treatment focuses on managing and improving the related signs and symptoms.
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.
It has been described as a tauopathy
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 (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 ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. 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 (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 (double vision) when reading.
*Behavioral and Cognitive impairment
*Imbalance and Difficulties walking
*Problems with speech and swallowing
*Loss of interest in pleasurable activities (apathy)
*Depression and anxiety
*Laughing or crying for no reason
True to its name, the signs and symptoms of progressive supranuclear palsy tend to become progressively worse as the disease advances.
We know that the symptoms of PSP are caused by a gradual deterioration of brain cells in a few tiny but important places at the base of the brain, in the region called the brainstem. One of these areas, the substantia nigra, is also affected in Parkinson’s disease, and damage to this region of the brain accounts for the motor symptoms that PSP and Parkinson’s have in common.
Scientists do not know what causes these brain cells to degenerate. There is no evidence that PSP is contagious, and genetic factors have not been implicated. No ethnic or racial groups have been affected more often than any others, and PSP is no more likely to occur in some geographic areas than in others.
There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Creutzfeldt-Jakob disease is one disease known to be caused by such an agent. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. This theory stems from a clue found on the Pacific island of Guam, where a common neurological disease occurring only there and on a few neighboring islands shares some of the characteristics of PSP, Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (Lou Gehrig’s disease). Its cause is thought to be a dietary factor or toxic substance found only in that area.
Another possible cause of PSP is cellular damage caused by free radicals, unstable molecules produced continuously by all cells during normal metabolism. Although the body has built-in mechanisms for clearing free radicals from the system, scientists suspect that, under certain circumstances, free radicals can react with and damage other molecules. A great deal of research is directed at understanding the role of free radical damage in human diseases.
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, 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.
Risk factors for progressive supranuclear palsy include:
*Age. Progressive supranuclear palsy typically affects people around the age of 60.
*Sex. Men are affected somewhat more often.
Complications of progressive supranuclear palsy result primarily from hindered muscle movements. These complications may include:
*Frequent falling, which can lead to head injuries, fractures and other injuries.
*Difficulty focusing your eyes, which also can lead to injuries.
*Problems with reading, driving a car, or other tasks requiring hand-eye coordination.
*Difficulty looking at bright lights.
*Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration). Aspiration can develop into pneumonia — the most common cause of death in people with progressive supranuclear palsy.
*Impulsive behaviors — for example, standing up without waiting for assistance — which can lead to falls.
Complications from progressive supranuclear palsy may eventually necessitate the use of a feeding tube due to choking hazards. To avoid injuries due to falling, a walker or a wheelchair may also be necessary.
Initial complaints in PSP are typically vague and an early diagnosis is always difficult. The primary complaints fall into these categories:
*Symptoms of dysequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness.
*Visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble.
*Various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood.
Progressive Supranuclear Palsy is often misdiagnosed because some of its symptoms are very much like those of Parkinson’s disease, Alzheimer’s disease, and more rare neurodegenerative disorders, such as Creutzfeldt-Jakob disease. In fact, PSP is most often misdiagnosed as Parkinson’s disease early in the course of the illness. Memory problems and personality changes may also lead a physician to mistake PSP for depression, or even attribute symptoms to some form of dementia. The key to establishing the diagnosis of PSP is the identification of early gait instability and difficulty moving the eyes, the hallmark of the disease, as well as ruling out other similar disorders, some of which are treatable.
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. A poor response to levodopa along with symmetrial onset can help differentiate this disease from PD.
Difference between PSP & Parkinson’s Disease:
Both PSP and Parkinson’s disease cause stiffness, movement difficulties, and clumsiness. However, patients with PSP usually stand straight or occasionally even tilt their heads backward (and tend to fall backward), while those with Parkinson’s disease usually bend forward. Problems with speech and swallowing are much more common and severe in PSP than in Parkinson’s disease, and tend to show up earlier in the course of the disease. Both diseases share other features: onset in late middle age, bradykinesia (slow movement), and rigidity of muscles. Tremor, almost universal in Parkinson’s patients, is rare in PSP. Although Parkinson’s patients markedly benefit from the drug levodopa, patients with PSP respond poorly and only transiently to this drug.
There is currently no effective treatment for PSP, although scientists are searching for better ways to manage the disease. In some patients the slowness, stiffness, and balance problems of PSP may respond to antiparkinsonian agents such as levodopa, but the effect is usually temporary. The speech, vision, and swallowing difficulties usually do not respond to any drug treatment.
Another group of drugs that has been of some modest success in PSP are antidepressant medications. The most commonly used of these drugs are fluoxetine (Prozac), amitriptyline (Elavil), and imipramine (Tofranil). The anti-PSP benefit of these drugs seems not to be related to their ability to relieve depression.
Non-drug treatment for PSP can take many forms. Patients frequently use weighted walking aids because of their tendency to fall backward. Bifocals or special glasses called prisms are sometimes prescribed for PSP patients to remedy the difficulty of looking down. Formal physical therapy is of no proven benefit in PSP, but certain exercises can be done to keep the joints limber.
A surgical procedure that may be necessary when there are swallowing disturbances is a gastrostomy. A gastrostomy (or a jejunostomy) is a minimally invasive procedure which is performed when the patient has difficulty swallowing or when severe choking is a definite risk. This surgery involves the placement of a tube through the skin of the abdomen into the stomach (intestine) for feeding purposes. Surgical procedures such as fetal brain cell implantation and pallidotomy, which are being tested as treatments for Parkinson’s disease, are not effective in PSP.
Life Style & Home Remedies:
To minimize the effects of progressive supranuclear palsy, you can take certain steps at home. They may include:
*Eyedrops. Eyedrops may help ease dry eyes that can occur as a result of problems blinking. They can also be helpful for persistent tearing.
*Fall-avoidance aids. Installing grab bars in hallways and bathrooms or using a walker that’s weighted can help you avoid falls. Making home modifications, such as removing scatter rugs or other items that are hard to see without looking downward, also can help with balance and vision problems. When possible, avoid climbing stairs.
As 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
Studies to improve the diagnosis of PSP have recently been conducted at the National Institute of Neurological Disorders and Stroke (NINDS). Experiments to find the cause or causes of PSP are currently under way. Therapeutic trials with free radical scavengers (agents that can get rid of potentially harmful free radicals) are being planned for the future.
In addition, there is a great deal of ongoing research on Parkinson’s and Alzheimer’s diseases at the National Institutes of Health and at university medical centers throughout the country. Better understanding of those common related disorders will go a long way toward solving the problem of PSP, just as studying PSP may help shed light on Parkinson’s and Alzheimer’s diseases.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose
- Movement Disorders (findmeacure.com)
- Parkinson’s Disease (findmeacure.com)
- New genes for risk and progression of rare brain disease identified (eurekalert.org)
- FDA Grants Orphan Drug Designation for Mithridion’s MCD-386CR to Treat Progressive Supranuclear Palsy (prnewswire.com)
- Three possible susceptibility genes found in neurodegenerative disorder (medicalxpress.com)
- FDA Grants Orphan Drug Designation For Mithridion’s MCD-386CR To Treat Progressive Supranuclear Palsy (bioresearchonline.com)
- Possible susceptibility genes found in neurodegenerative disorder (eurekalert.org)
- CurePSP-Funded Study Identifies Three New Genes Linked To Progressive Supranuclear Palsy (medicalnewstoday.com)
- Possible susceptibility genes found in neurodegenerative disorder (sciencedaily.com)
- Questions to Ask Your Doctor About Bell’s Palsy (everydayhealth.com)