Progressive Supranuclear Palsy

Signs and Symptoms

The signs and symptoms of progressive supranuclear palsy (PSP) vary from patient to patient but loss of balance while walking is the most common first symptom. Patients may have unexplained falls or a stiffness and awkwardness when walking. Sometimes the falls are described as attacks of dizziness, which can lead to an incorrect diagnosis of an inner ear problem.

Other common early symptoms include a loss of interest in usual hobbies or recreational activities, increased irritability and forgetfulness. Patients may suddenly laugh or cry, be apathetic or have occasional angry outbursts for no apparent reason.

As the disease progresses, many patients develop blurring of vision and problems controlling eye movements. PSP patients have trouble voluntarily shifting their gaze downward and can have trouble controlling their eyelids. This can lead to involuntary closing of the eyes, prolonged or infrequent blinking, or difficulty in opening the eyes. Another common visual problem is an inability to maintain eye contact during a conversation. This can give the mistaken impression that the patient is hostile or uninterested.

Diagnosis

An early diagnosis of progressive supranuclear palsy (PSP) is difficult because initial complaints typically are vague. PSP often is misdiagnosed because some symptoms are very much like those of Parkinson's disease, Alzheimer's disease and more rare neuro-degenerative disorders, such as Creutzfeldt-Jakob disease. Memory problems and personality changes also may lead a doctor to mistake PSP for depression or even attribute symptoms to some form of dementia.

It is important that your neurologist conduct a thorough clinical evaluation to establish an accurate diagnosis of PSP. This involves an interview with you and another person such as a spouse, relative or close friend to provide examples of behavior and daily activities, physical testing for mobility and vision, and neuropsychological testing for evaluation of thinking and reasoning.

Treatment

Currently, there is no effective treatment for progressive supranuclear palsy (PSP), although scientists are searching for better ways to manage the disease. In some patients the slowness, stiffness and balance problems may respond to Parkinson's disease medications but the effect is usually temporary. Speech, vision and swallowing difficulties usually do not respond to drug treatments.

Simple lifestyle changes may benefit patients with PSP. For example, patients frequently use weighted walking aids to deter their tendency to fall backwards. Bifocals or special glasses called prisms are sometimes prescribed for PSP patients to remedy their difficulty of looking down. Formal physical therapy has not proven beneficial but certain exercises can be performed to keep the joints limber.

When a patient experiences severe difficulty swallowing and choking is a risk, a surgical procedure called a gastrostomy may be necessary. This surgery involves the placement of a tube through the skin of the abdomen into the intestine for feeding purposes.

Being a caregiver for a PSP patient can be physically and emotionally demanding. Support groups can play an important role in coping with the disease and maintaining quality of life for the patient and caregiver.