Author: Dr. Ricardo Kotliroff
Progressive supranuclear palsy (PSP), also called Steele-Richardson-Olszewski Syndrome, is an uncommon neurodegenerative disease that affects brain cells.
Progressive Supranuclear Palsy Causes & Risk Factors
An estimated 3 to 6 of every 100,000 individuals throughout the world are afflicted with PSP, and middle-aged and elderly people are at higher risk of developing it. Sixty is the most common age that PSP presents, although some patients may experience initial symptoms starting as young as 40. Typically, men tend to be affected by PSP than women.
PSP is caused by cellular deterioration above nerve cell clusters, (neurons and glial cells, termed nuclei) in the midbrain and the brainstem, which is responsible for controlling body movement, and the frontal lobe, which controls thinking. Damage that occurs above the clusters of nerve cells is called supranuclear, and because the disease results in weakness of certain parts of the body, it is termed palsy.
Patients with PSP have been observed to have abnormal amounts of the tau protein inside deteriorating brain cells. These clumps of tau proteins interfere with the microtubule structures’ ability to send information (via axons of the nerve cells to other nerve cells in the brain), as well as cause cell death. One theory suggests that abnormal clumps of tau proteins may stimulate connected cells to produce clumps of tau protein. Tau clumps have also been found in other neurodegenerative diseases, including Alzheimer’s Disease.
Rare (under 1% of) cases of PSP occurring within certain families have been observed. Also, rare mutations in the MAPT gene, which is responsible for synthesizing tau protein, have been found in some patients with PSP.
Progressive Supranuclear Palsy Symptoms
As the name Progressive Supranuclear Palsy indicates, symptoms of the disease may worsen with time. Physical symptoms of PSP may include:
- Troubles with the eyes including: abnormal eye movement, such as blinking, prolonged tearing, difficulty opening the eyes, burning sensations, and sensitivity to light
- Abnormal speech involving: incorrect sounds (apraxia), which may occur early in disease, and slurred speech
- 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 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, problem solving, and decision making.
Progressive dementia is usually mild.
Patients with Progressive Supranuclear Palsy may become severely disabled withing 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 start to appear.
Although the condition of PSP itself is not fatal, some of its complications are dangerous and may even be life-threatening.
Complications from PSP symptoms may include:
- Abnormal movement of the eyes and an inability to aim the eyes correctly may affect vision. Some patients have problems looking down, or have blurred or double vision. Problems with visual focus may result in spilling of food. Additionally, due to the appearance of poor eye contact with others, PSP patients may seem uninterested during conversations. More seriously, vision problems can cause injuries.
- Difficulty swallowing may result in a risk of choking or aspirating (i.e., inhaling) food or liquids into the airway. Pneumonia may develop from aspiration of food or liquid. Importantly, Pneumonia is the most common cause of death in patients with PSP. To avoid choking, physicians may suggest surgical implantation of a feeding tube.
- Some patients may experience stiffness and awkward movements. Serious, permanent problems with balance may interfere with the ability to walk, increasing the risk of falling. Early in the disease, some patients experience a tendency to fall backwards. Falling can cause injuries to the head or fractures in other parts of the body.
- Impulsive behaviors, including not waiting for help in mobility, can result in falling.
Proper diagnosis by a neurologist or movement disorder specialist is important because physicians may sometimes confuse the symptoms of Progressive Supranuclear Palsy with those of Parkinson’s Disease, Alzheimer’s disease, or other forms of dementia.
Diagnosis may include taking a medical history, physical examination, neurological examination, and radiological tests such as magnetic resonance imaging (MRI) or positron emission tomography (PET) to find out whether shrinking in the upper part of the brainstem, a sign of PSP, has occurred; to identify other abnormalities in the brain; to measure brain activity in areas where degeneration is known to have occurred; and to exclude other conditions, such as stroke.
Types of Progressive Supranuclear Palsy
Patients with Progressive Supranuclear Palsy may be classified into two groups. PSP-Parkinsonism and PSP-Richardson. People with PSP-Parkinsonism may respond temporarily to the medication, Levodopa, before their illness progresses to characteristics of the PSP-Richardson group, the classical type of PSP.
Progressive Supranuclear Palsy Treatment
Conventional treatment may include certain medications, glasses with bifocal or prism lenses to improve issues with looking down, evaluations for speech and swallowing, walking aids (such as canes, walkers, or wheelchairs), physical and occupational therapy, and feeding tubes (gastrostomy, surgical implantation of a tube through the abdominal skin leading into the intestine) for patients with difficulty swallowing and/or risk of choking.
Currently, there is no known “cure” for Progressive Supranuclear Palsy. Thus, conventional treatment is limited to the management of signs and symptoms, and enhancing the comfort of the patient. However, conventional treatment by physicians allows survival of patients with Progressive Supranuclear Palsy to be at least 10 years following the presentation of initial symptoms.
Conventional medications include:
- Eyedrops to keep eyes moist.
- Injections of low doses of Botulisum toxin type A (Botox) into the muscles surrounding the eyes to block chemical signals causing contraction of muscles, thereby reducing spasms of the eyelids.
- Medicines for Parkinson’s Disease to increase concentrations of dopamine, a chemical in the brain that involved in controlled movements of smooth muscles. For example, Levodopa may reduce tremors. However, the effects of these medicines last only 2 to 3 years.
- Antidepressant medicines may relieve pain, reduce drooling, and improve mood.
- Medicines to aid sleep.
Alternative therapy for PSP includes homeopathy, which seeks to treat the patient, rather than just the disease. Homeopathy is an effective method of healing acute and chronic conditions, as claimed by millions of patients worldwide and thousands of homeopathic doctors.
Our proprietary therapeutic methods are based on a belief that all humans possess certain foundational energy sources within their core, and that a person’s energy balance is tied directly to his/her state of health and wellbeing. When these energy sources are disturbed, whether from internal or external forces, disease occurs.
Our homeopathic treatments seek to neutralize these disturbances and restore a person’s natural, optimal energy balance.
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