Progressive supranuclear palsy: causes and treatment Essay

Progressive supranuclear palsy (PSP), also called SteeleRichardsonOlszewski syndrome, is an uncommon neurodegenerative disease affecting brain cells.
Causes & Risk Factors
Throughout the world, an estimated 3 to 6 of every 100,000 individuals are afflicted with progressive supranuclear palsy. Middleaged and elderly people are at higher risk of developing progressive supranuclear palsy. The most common age of presentation with PSP is 60 years, although some patients may experience initial symptoms when they are over 40 years if age. More men than women tend to be affected by PSP.
PSP is caused by deterioration of cells above clusters of nerve cells (neurons and glial cells) termed nuclei in the midbrain and the brainstem, which control body movement, and the frontal lobe, which controls thinking. As the damage occurs above the clusters of nerve cells, it is called supranuclear. Because the disease results in weakness of certain parts of the body, it is termed palsy.
In patients with PSP, abnormal amounts of the protein, tau, have been seen within deteriorating brain cells. The clumps of tau protein interfere with the ability of microtubule structures within the nerve cells in the brain to send information via the axons of the nerve cells to other nerve cells in the brain and death of the cells. A theory is that abnormal clumps of tau may stimulate a connected cell to produce clumps of tau protein. Tau clumps also have been found in other neurodegenerative diseases, including Alzheimers disease.
Rare (under 1% of) examples of PSP occurring within certain families have been observed. Also, rare mutations that have been observed in the MAPT gene, which is responsible for synthesizing tau protein, have been found in some patients with PSP,
Symptoms
As the name progressive supranuclear palsy indicates, symptoms of the disease may worsen with time. Physical symptoms of PSP may include
Abnormal movement of the eyes, such as blinking, prolonged tearing of the eyes, difficulty opening the eyes, and a sensation of burning in the eyes
Sensitivity of the eyes to light
Abnormal speech, involving incorrect sounds (apraxia), which may occur early in disease. Speech may be slurred.
Difficulty swallowing
Rigidity of facial muscles, causing a frightened or surprised look on the face
Sleep disturbances
Problems with balance
Tremors in the legs, feet, or hands
Difficulty in walking
Emotional, behavioral, and cognitive symptoms of PSP may include
Personality changes, such as more irritability
Reduced interest in pleasurable activities
Impulsive behavior, including crying or laughing in the absence of apparent reasons
Depression and/or anxiety
Cognitive difficulties in memory, reasoning, solving, problems, and making decisions. The progressive dementia usually is mild.
Complications
Patients with progressive supranuclear palsy may become severely disabled by 3 to 5 years after the onset of symptoms. The most common cause of death is pneumonia. With good attention to medical and nutritional needs, it is possible for individuals with PSP to live at least a decade after the first symptoms of the disease.