When a customer wrote us to say that TheraSpecs provide her husband with great relief from the light sensitivity caused by PSP, we were curious to learn about the disorder. PSP is an abbreviation of progressive supranuclear palsy.

What is progressive supranuclear palsy?

PSP causes serious, progressive problems with controlling gait and balance, complex eye movement, and thinking. More common in men than women, PSP is rare neurological disorder – only about 20,000 Americans have it, according to the National Institutes of Health. The full name of the disorder indicates that it worsens over time (progressive), causes weakness (palsy), and damages a particular part of the brain (supranuclear). PSP can look like Parkinson’s disease, but they are distinct illnesses. Given its similarity to other movement disorders and because hallmark symptoms may be late to develop, or may never develop at all, PSP can be difficult to diagnose.

What are the symptoms of progressive supranuclear palsy?

• Loss of balance: Often, losing balance when walking is the first symptom. PSP may cause people to fall or have a stiff, awkward gait. Sometimes people say their falls appear to be caused by dizziness.
• Personality and mood changes: Loss of interest in activities that were once enjoyable, irritability, forgetfulness, sudden tears or laughter, apathy and angry outbursts are all possible, though the symptoms vary from one person to the next.
• Mental difficulties: Loss of memory or slowed thinking can be symptoms of PSP.
• Vision/visual problems: People with PSP have trouble controlling eye movements, which can make vision appear blurred and cause difficulty making eye contact. PSP can also cause difficulty in controlling eyelids, causing involuntary eye closures, long or frequent blinks, and trouble opening eyes. Light sensitivity is another symptom of PSP.
• Slurred speech
• Difficulty swallowing

What causes progressive supranuclear palsy?

PSP symptoms are caused by slow deterioration of brain cells in some specific areas of the brain, mainly in the brainstem. Although scientists don’t completely understand why brain cells deteriorate, people with PSP have an accumulation of tau, which is an abnormal protein. Genetic factors have not been identified in most people; there is also no evidence it is contagious. Several factors have been speculated to cause PSP, though none have been proven. Speculated causes include a virus-like illness that does not produce symptoms for years, a random genetic mutation, exposure to some unknown chemical that slowly damages implicated areas of the brain, or damage from free radicals.

How is progressive supranuclear palsy diagnosed?

Early diagnosis of PSP is difficult. Key symptoms for diagnosis are gait instability and difficulty moving eyes, both of which are characteristic symptoms of PSP. It is often misdiagnosed because symptoms are similar to those of Parkinson’s, Alzheimer’s, and other neurodegenerative disorders.

What’s the difference between progressive supranuclear palsy and Parkinson's disease?

Although symptoms of the two diseases can be similar, there are some key differences. Parkinson’s usually causes people to bend forward; people with PSP usually stand straight or tilt their heads backward and tend to fall backward. Speech and swallowing difficulties are more common and severe in PSP than in Parkinson’s, they also tend to show up earlier in the disease. Eye movement difficulties are characteristic of PSP, but are close to normal in Parkinson’s. Tremor is common in Parkinson’s and rare in PSP. The drug levodopa can be very helpful in treating Parkinson’s, but is ineffective for PSP.

What’s the treatment for progressive supranuclear palsy?

Currently, there is no established treatment for PSP, though researchers are investigating options. Some symptoms can be managed with medication or non-drug treatments. For example, Botox can help with excessive eye closing, weighted walking aids can prevent falls, eyewear can assist with the difficulty looking down and/or relieve light sensitivity. Physical and occupational therapy can improve balance. If swallowing becomes too difficult, a gastrointestinal tube can be used for nutrition.

References:

• National Institute of Neurological Disorders and Stroke: Progressive Supranuclear Palsy Fact Sheet. Retrieved October 29, 2014 from http://www.ninds.nih.gov/disorders/psp/detail_psp.htm.
• Mayo Clinic. Progressive Supranuclear Palsy. Retrieved October 29, 2014 from http://www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/basics/definition/con-20029502.