Brain Facts

Brain Facts

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Progressive Supranuclear Palsy

Description

Progressive supranuclear palsy (PSP) is a disorder characterized by symptoms similar to Parkinson's disease (including unsteady gait, stiff movements, and mild dementia). Symptoms usually begin between ages 50 and 60, with a range from the early forties to the late eighties and is more common in men than in women. Because its early symptoms may look similar to those of Parkinson's disease or Alzheimer's disease, it is often initially misdiagnosed as one of these two diseases.

PSP is a disorder caused by damage to certain nerve cells in the brain that results in progressive lack of coordination, stiffness of the neck and trunk, difficulties with eye movement, slow movements, cognitive dysfunction, and difficulty walking that can result in falls.

The cause is unknown, but the disease gets worse over time. It involves damage to multiple cells of the brain, with loss of the myelin sheath (the covering of the nerve cell that speeds nerve impulse conduction) in some nerves and destruction of the entire nerve in other areas.

People with this condition have deposits in brain tissues that resemble the deposits found in Alzheimer's disease. There is atrophy (loss of tissue) in most areas of the brain.

Symptoms may include:

Repeated falls
Loss of coordination, unsteady gait (walking pattern)
Stiffness and rigid movement in the neck, trunk, arms, legs  
Difficulty swallowing
A tremor
Pain or difficulty with bending the neck up or down
Slow or stiff movements
Vision difficulty
Inability to look up or down without bending the neck
Changes in facial expression
"Masking" - reduced expression
Deeply lined face
Jaw or face jerks or spasms
Pupils of the eyes different size
Uncontrollable eye movements
Generalized slowness of all movement with strength relatively preserved
Speech difficulties
Slow speech
Poor enunciation
Low voice volume
Mild dementia
Forgetfulness
Slowed thought processes
Apathy (indifference)
Difficulty manipulating knowledge
Personality changes, vague and mild


A neurological examination may show Parkinsonian movements with typical stiffness and lack of coordination. Eye movements are limited, particularly vertical movements. However, vision, hearing, sensation, and voluntary control of movement remain intact. Tests may be done to rule out other diseases. An MRI might show shrinking of the brainstem.


Treatment

Only a few drugs have been found to have any effect on the symptoms of PSP. Drugs that appear to have some benefit include dopa, amantadine, amitriptyline, desipramine, and yohimbine. Unfortunately, none of these drugs provide dramatic or long-lasting improvements, and each can cause serious side effects. Botulinum toxin can be used to treat eyelid spasms and other types of dystonia that sometimes occur in PSP. Artificial tears can help prevent drying out of the eyes that may occur from decreased blinking.

A speech therapist can teach safer swallowing techniques and advise on the need for a gastrostomy tube. The speech therapist can also offer the PSP patient alternative communication devices, such as pointing boards or computer-based systems. Books on tape provide an alternative when reading is no longer possible.

A weighted, soft-wheeled walker can help prevent falls. Caregivers can improve the safety of the person with PSP by keeping pathways in the home clear of objects such as toys, floor rugs, or low furniture that are difficult to see without looking down. An occupational therapist can advise on these and other home modifications to improve safety, comfort, and usability of the home environment.


Prognosis

PSP gets progressively worse but is not itself directly life-threatening. It does, however, predispose patients to serious complications such as pneumonia secondary to difficulty in swallowing. 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, however, most PSP patients live well into their 70s and beyond.

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