Progressive supranuclear palsy PSP is a rare brain disorder that causes problems with movement, walking and balance, and eye movement. It results from damage to nerve cells in the brain that control thinking and body movement.
Currently there is no effective treatment for PSP, but some symptoms can be managed with medication or other interventions. PSP affects movement, control of walking gait and balance, speech, swallowing, eye movements and vision, mood and behavior, and thinking. The pattern of signs and symptoms can be quite different from person to person. The most frequent first symptom of PSP is a loss of balance while walking.
Individuals may have abrupt and unexplained falls without loss of consciousness, a stiff and awkward gait, or slow movement. As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. These symptoms may include:.
The onset of both diseases is in late middle age. Although individuals with Parkinson's disease markedly benefit from the drug levodopa, people with PSP respond minimally and only briefly to this drug.
The exact cause of PSP is unknown, but research suggests that it involves a gradual deterioration of brain cells in a few specific areas in the brain, mainly in brain stem. The death of brain cells in one of these areas, the substantia nigra, accounts in part for the motor symptoms that PSP and Parkinson's have in common. The hallmark of PSP is the accumulation of abnormal deposits of the protein tau in nerve cells in the brain.
These deposits cause the cells to malfunction and die, which stops the flow of information to other nerve cells. PSP is usually sporadic, meaning that it occurs infrequently and without a known cause.
In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau to the nerve cell. Patients with atypical Parkinsonism often have some degree of difficulty speaking or swallowing, and drooling can be a problem. Psychiatric disturbances such as agitation, anxiety or depression may also be part of the clinical picture. Dementia with Lewy bodies DLB can cause changes in attention or alertness over hours or days, often with long periods of sleep two hours or more during the day.
Visual hallucinations — typically of small animals or children, or moving shadows in the periphery of the visual field — are common in DLB. Patients with progressive supranuclear palsy PSP may have difficulties with eye movements, particularly when looking downward, and with balance — when descending stairs, for instance.
Backward falls are common and may occur during the early course of the disease. Multiple system atrophy MSA can affect autonomic function, with urinary urgency, retention and incontinence, constipation, lightheadedness when standing orthostasis and significant otherwise unexplained erectile dysfunction in men. Patients may experience color and temperature changes in hands and feet, such as redness and coldness.
When MSA affects the cerebellum, patients may have ataxia, characterized by a wide-based unsteady gait, and lack of coordination in the hands, feet or both. The symptoms of corticobasal syndrome CBS often appear only on one side of the body. Symptoms usually begin after age 60 and can progress more rapidly than PD. Symptoms may include:. Individuals may experience slurred speech, swallowing difficulties and personality changes. Dementia often develops during the course of the disease.
Speech and physical therapy are important for the management of PSP. Learn more about PSP. MSA is a collective term for several rare disorders in which multiple systems in the nervous system deteriorate.
The average age of symptom onset is in the mids. Patients may respond to PD medications, but often the response is limited. Speech and physical therapy are important for the management of MSA. Learn more about MSA. LBD is a relatively common neurodegenerative disorder that results in progressive and fluctuating cognitive changes and functional deterioration.
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