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Progressive Supranuclear Palsy (PSP)

By Hector A. Gonzalez-Usigli, MD, Instituto Mexicano del Seguro Social, Guadalajara ; Alberto Espay, MD, University of Cincinnati

Progressive supranuclear palsy is characterized by muscle stiffness (rigidity), problems moving the eyes, and a tendency to fall backward.

  • Progressive supranuclear palsy progresses faster and results in more severe muscle rigidity and disability than Parkinson disease.

  • The diagnosis is based on symptoms.

  • No effective treatment exists, but drugs used to treat Parkinson disease sometimes provide temporary relief.

Progressive supranuclear palsy, which is much rarer than Parkinson disease, affects many parts of the brain, particularly the basal ganglia and the brain stem. (The basal ganglia help smooth out muscle movements. The brain stem regulates critical body functions, such as breathing, heart rate, and swallowing, and helps adjust posture.) Brain cells in these areas degenerate, but why they do is usually unknown.

Locating the Basal Ganglia

The basal ganglia are collections of nerve cells located deep within the brain. They include the following:

  • Caudate nucleus (a C-shaped structure that tapers to a thin tail)

  • Putamen

  • Globus pallidus (located within the putamen)

  • Subthalamic nucleus

  • Substantia nigra

The basal ganglia help smooth out muscle movements and coordinate changes in posture.

Symptoms

Symptoms of progressive supranuclear palsy usually begin after age 40.

The first symptom may be difficulty looking up without bending the neck or difficulty climbing up and down stairs.

People with the disorder cannot roll their eyes downward, fix their eyes on a stationary object, or follow a moving object. They may have blurred or double vision. The upper eyelids may pull back, producing a look of astonishment.

Muscles become rigid, and movements are slow. Walking is unsteady, with a tendency to fall backward. Speaking and swallowing are difficult.

Other symptoms include insomnia, agitation, irritability, apathy, and rapid changes in emotion.

In the late stages, depression and dementia are common. Compared with Parkinson disease, progressive supranuclear palsy progresses faster, responds less well to treatment, and results in more severe muscle rigidity and disability, usually within 5 years. Usually, death, often due to infection, occurs within 10 years after symptoms begin.

Diagnosis

  • A doctor's evaluation

  • Usually magnetic resonance imaging

The diagnosis of progressive supranuclear palsy is based on symptoms.

Magnetic resonance imaging (MRI) is usually done to check for other disorders that may be causing the symptoms.

Treatment

  • Sometimes drugs used to treat Parkinson disease

  • Physical and occupational therapy

No effective treatment for progressive supranuclear palsy exists.

The drugs used to treat Parkinson disease sometimes provide temporary relief.

Physical and occupational therapists can provide exercises to help keep joints limber and help people function better. They can also recommend strategies and safety measures to reduce the risk of falls.

Because progressive supranuclear palsy is fatal, people with this disorder should prepare advance directives, indicating what kind of medical care they want at the end of life.

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* This is the Consumer Version. *