Progressive Supranuclear palsy (PSP)

aka: Dementia-nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy – progressive supranuclear

History:

PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson’s disease. It is sometimes referred to as Steele-Richardson-Olszewski syndrome, reflecting the combined names of the scientists who defined the disorder. Although PSP gets progressively worse, no one dies from PSP itself.

Definition:

Progressive supranuclear palsy (PSP) is a condition causes serious and progressive problems with control of gait and balance, along with complex eye movement and thinking problems.

One of the classic signs of the disease is an inability to aim the eyes properly, which occurs because of lesions in the area of the brain that coordinates eye movements. Some individuals describe this effect as a blurring. Affected individuals often show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia.

Affected areas include the part of the brainstem where cells that control eye movement are located, and the frontal lobes of the brain which lead to personality changes.

Presentation and Symptoms

  • Changes in expressions of the face
  • Deeply lined face
  • Difficulty moving the eyes or lack of control over the eyes
  • Different size pupils
  • Difficulty swallowing
  • General slowness of movement (bradykinesia)
  • Jaw or face jerks or spasms
  • Loss of coordinationunsteady gait (walking pattern)
  • Mild-to-moderate dementia
    • Difficulty using knowledge
    • Forgetfulness
    • Indifference (apathy)
    • Slowed thought processes
  • Personality changes
  • Repeated falls
  • Slow or stiff movements
  • Speech difficulties
    • Low voice volume
    • Poor ability to speak clearly (enunciate)
    • Slow speech
  • Stiffness and rigid movement in the neck, middle of the body, arms, legs
  • Tremor
  • Vision difficulty — unable to look up or down without bending the neck
    There is no known cure.

Etiology:

unknown.

Biochemistry:

Diagnosis:

1) symptoms of disequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness;

2) visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble;

3) slurred speech; and

4) various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood.

People with this condition have deposits in brain tissues that look like those found in patients with Alzheimer’s disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.

PSP is often misdiagnosed because some of its symptoms are very much like those of Parkinson’s disease, Alzheimer’s disease, and more rare neurodegenerative disorders, such as Creutzfeldt-Jakob disease. PSP is most often misdiagnosed as Parkinson’s disease early in the course of the illness. Memory problems and personality changes may also lead a physician to mistake PSP for depression, or even attribute symptoms to some form of dementia.

The key to diagnosing PSP is identifying early gait instability and difficulty moving the eyes.

Prognosis

PSP gets progressively worse but is not itself directly life-threatening. It does, however, predispose individuals to serious complications such as pneumonia secondary to difficulty in swallowing (dysphagia). The most common complications are choking and pneumonia, head injury, and fractures caused by falls. The most common cause of death is pneumonia. With good attention to medical and nutritional needs, it is possible for most individuals with PSP to live a decade or more after the first symptoms of the disease.

Management:
Symptom management, palliative care and comfort measures.

Levodopa or other drugs, such as benztropine or trihexyphenidyl, which block the action of a nervous system chemical called acetylcholine (anticholinergic medications) may temporarily reduce some symptoms, such as rigid limbs or slow movements.

Excessive eye closing can respond to botulinum injections.

A gastrostomy (or a jejunostomy) may be necessary for feeding when dysphagia is severe and the patient is at high risk for aspiration.

Source: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001772/

http://www.ninds.nih.gov/disorders/psp/detail_psp.htm

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