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Progressive Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP)

PROGRESSIVE SUPRANUCLEAR PALSY

Progressive supranuclear palsy (PSP), which is also known as Steele- Richardson- Olszewski syndrome, is an uncommon brain disorder that causes permanent problems with the control of balance together with eye movement and thinking problems.

Symptoms

The symptoms of progressive supranuclear palsy usually vary from person to person and they include inability to control eye movements like shifting the gaze upward or downward. This key symptom helps differentiate PSP from Parkinson’s disease. It can also cause blurred vision, double vision, prolonged or infrequent blinking and difficulty opening the eyes.
Other symptoms of PSP include loss of balance while walking which results in unexplained falls or an awkward gait. Some patients have slowness and stiffness of movements which is similar to that seen in persons with Parkinson’s disease. There may also be dysphagia or difficulty swallowing and dysarthria or difficulty speaking.
Cognitive symptoms of PSP include impaired executive functions like distractibility, impaired planning, pathetic problem solving and poor financial judgment. The memory may also become impaired and there may be progressive dementia though mild.
Behavioral and emotional symptoms associated with this condition include inability to control behavior and emotions especially in social settings which often results in embarrassment. Other symptoms include depression, apathy, alterations of mood and increased irritability. Some patients also develop prolonged forced laughing or crying.
Progressive supranuclear palsy arises as a result of degeneration of cells in the areas of the brain that coordinate eye movements, body movements and thinking. Cells in these regions have also been found to have collections of an abnormal form of the protein tau. The exact cause of this degeneration is not known.

Diagnosis

Diagnostic tests that may be done for persons with PSP include magnetic resonance imaging (MRI) to determine if there is shrinkage in the areas of the brain affected by this condition; however, the condition is diagnosed based on the history and a thorough neurological exam by an experienced neurologist.

Treatment

There is no cure for progressive supranuclear palsy though antiparkinsonian medications like levodopa are used to manage the slowness, stiffness and balance problems associated with it.  The benefit of Levodopa tends to be modest. This levodopa can also be combined with anticholinergic agents but its effects are usually temporary, lasting for less than three years.
Antidepressant medications like Prozac, Elavil and Tofranil are also used to manage some patients with PSP.
Botulinum toxin type A which is commonly known as Botox, is used to treat the excessive eye closing and eyelid spasms. This medication which is produced by the bacteria Clostridia botulinum, is injected into the muscles around the eyes to paralyze them since it blocks the release of the neurotransmitter or signaling chemical known as acetylcholine which causes muscle contractions. Repeat injections are required since the effects usually last for a just a few months.
Non-drug treatment for PSP includes the use of weighted canes or wheelchairs to counteract the tendency to fall backwards. Bifocal or special glasses with prism lenses are also prescribed to aid looking downwards.
Surgical treatment includes gastrostomy or jejunostomy which is done when the difficulties in swallowing pose a risk of choking. In this operation the surgeon places a tube through the skin of the abdomen into the stomach for feeding purposes.
It is important to treat PSP because even though it begins slowly, it progressively gets worse and it can lead to complications like choking and aspiration pneumonia from inhaling food. Other serious complications of this condition include fractures and head injury from the falls.

Research

Progressive supranuclear palsy research reveals that coenzyme Q10 can be of some benefit in managing this condition, but no large trials have been completed to verify this.

Support

Support groups for patients with progressive supranuclear palsy include CurePSP Foundation for PSP, CBD and related brain diseases whose website is http://www.psp.org/. Their address is 30E. Padonia road, suite 201, Timonium, MD 21093. They can be reached by phone at 800 457 4777 or via email at info@curepsp.org

 

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September 17, 2014