PROGRESSIVE SUPRANUCLEAR PALSY

Richardson-Steele-Olszewski syndrome; Steele-Richardson-Olszewski syndrome; Richardson Syndrome; Nuchal Dystonia Dementia Syndrome; Progressive Supranuclear Ophthalmoplegia; Supranuclear Palsy-Progressive; PSP

Progressive supranuclear palsy (PSP) is a rare progressive brain disorder that affects movement, gait, eye movements, speech, and cognition. The signs and symptoms are very different in each person, but may include personality changes, difficulty swallowing, jaw or face spasms, vision problems, loss of balance, frequent falls, problems with walking, poor coordination, and an unsteady, lurching gait. PSP primarily involves damage to the brain stem (the area of the brain that controls breathing, heartbeat, blood pressure, eye movement, and other autonomic functions) and the frontal lobes of the brain (the area of the brain that is associated with reasoning, planning, speech, movement, emotions, and problem solving). The most obvious sign of PSP is an inability to focus the eyes properly, which occurs because of lesions in the area of the brain that coordinates eye movements. People with PSP often show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia. PSP usually occurs in people over 60 years of age, and men are affected more often than women are. Additional features of the disorder includes a general loss of interest and enthusiasm (apathy) an increasing need for assistance with personal care and other activities of daily living.

Diagnostic testing: The diagnosis of PSP involves:

• •

  A clinical examination;

• •

  An evaluation of memory;

• •

  Assessment of balance and gait;

• •

  Evaluation of ocular movements with attention on the ability to move the eyes up and down;

• •

  Hearing assessment and evaluation of voluntary muscle movement; and

• •

  MRI of the brain.

PSP is often misdiagnosed because some of its symptoms are similar to Parkinson’s disease, Alzheimer disease and Creutzfeldt-Jakob disease. The key to properly diagnosing PSP is identifying early gait instability and difficulty moving the eyes. Neurological examinations may show uncontrollable eye movement, mild dementia, and visual difficulties with the inability to look up or down without bending the neck.

Physical findings: Signs and symptoms of PSP include:

• •

  Difficulty swallowing;

• •

  Jaw or face spasms;

• •

  Vision problems;

• •

  Loss of balance;

• •

  Frequent falls;

• •

  Problems with walking;

• •

  Poor coordination; and

• •

  An unsteady, lurching gait.

ICD-9: 333.0

PSP is slowly progressive with a decline in brain dysfunction over time. Initially, the motor and eye movement abnormalities are the major impairments, but as the disease progresses, cognitive and behavioral manifestations may become significant. In advance cases, the person with PSP may become wheel chair dependent or bedridden. Individuals with PSP are also at risk of pneumonia, choking from swallowing problems and injuries from falling.

There is no effective cure for this disorder. Drugs such as L-dopa, Amantadine, Amitriptyline, and Desipramine are prescribed to control symptoms. Speech therapy can provide instruction in swallowing techniques. Communication devices such as pointing boards or computer-based systems can be used when speech becomes ineffective. Walking devices can help to minimize falls.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

• •

  Clinical history and examination describing symptoms, signs, laboratory findings, and response to treatment;

• •

  Full neurological examination with emphasis on motor function and coordination, gait and balance, eye movements and gaze, and cognitive function;

• •

  Speech and language examination describing speech difficulties, low voice volume, slow speech, and poor ability to speak clearly (enunciate); and

• •

  Assessment of swallowing abilities.

Suggested Listings for Evaluation:

REMARKS