Progressive Supranuclear Palsy (PSP): Symptoms, Diagnosis and Outlook

Progressive supranuclear palsy (PSP) is a rare brain disease caused by damage to nerve cells in the brain. It affects movement — especially walking and balance — as well as thinking and eye movement. It is progressive: it gets worse over time.

Causes and risk

The cause of PSP is unknown; in rare cases it is due to a mutation in a particular gene. One of its hallmarks is abnormal clumps of tau, a protein found in the nervous system, inside brain nerve cells. Tau also builds up in the brain in some other diseases, including Alzheimer’s disease.

PSP usually affects people over 60, though it sometimes begins earlier, and it is more common in men.

Symptoms

Symptoms differ a great deal from person to person. They may include:

• loss of balance while walking — often the first symptom;
• speech problems;
• trouble swallowing;
• blurred vision and difficulty controlling eye movements;
• changes in mood and behavior, including depression and apathy, a loss of interest and enthusiasm; and
• mild dementia.

Diagnosis

There is no specific test for PSP, and it can be hard to diagnose because its symptoms resemble those of other diseases, such as Parkinson’s and Alzheimer’s. A health care provider will take a medical history and do physical and neurological exams, and may order an MRI or other imaging.

Treatment

There is currently no effective treatment. Medicines may ease some symptoms, and non-drug aids such as walking aids and special glasses can help. People with severe swallowing problems may need a gastrostomy, surgery to place a feeding tube into the stomach.

Outlook

PSP worsens over time, and many people become severely disabled within three to five years of its onset. The disease is not life-threatening in itself, but it is still dangerous because it raises the risk of pneumonia, choking from swallowing problems, and injuries from falls. With good attention to medical and nutritional needs, many people with PSP live 10 years or more after their first symptoms.

Sources

Based on "Progressive Supranuclear Palsy," MedlinePlus, National Library of Medicine, with information from the National Institute of Neurological Disorders and Stroke; a work of the United States government in the public domain.