What is progressive supranuclear palsy? Democrat Congresswoman Jennifer Wexton diagnosed with rare disease described as ‘Parkinson’s on steroids’
Congresswoman Jennifer Wexton said Monday she will not run for office after being diagnosed with a brain disorder she described as “Parkinson’s on steroids.”
The Virginia Democrat revealed in April that she suffered from Parkinson’s disease, which sees parts of the brain gradually waste away, affecting movement and speech.
Doctors later changed his diagnosis to progressive supranuclear palsy (PSP), a similar but much rarer disorder that affects only 20,000 people in America.
For comparison, approximately 1 million people in the United States have Parkinson’s disease.
The main difference is that PSP causes patients to deteriorate much more quickly, causing more severe symptoms, including speech, coordination and eating.
People with PSP die within ten years of their diagnosis, whereas patients with Parkinson’s disease can expect to live between 10 and 20 years after diagnosis.
Rep. Jennifer Wexton is pictured above with her family. The MP revealed this week her new diagnosis of a rare neurological condition called progressive supranuclear palsy (PSP).
PSP is different from Parkinson’s disease in that it usually appears later in life and gets worse quickly. Speech and swallowing problems are much more common and severe in PSP patients than in those with Parkinson’s disease, and it is rare for PSP patients to develop tremors, a characteristic sign of Parkinson’s disease.
PSP, a form of “Parkinson’s on steroids,” can be underdiagnosed or misdiagnosed because its symptoms closely resemble those of Parkinson’s disease. However, several symptoms can differentiate the two.
Speech and swallowing problems are much more common and severe in PSP patients than in those with Parkinson’s disease, and it is rare for PSP patients to develop tremors, a characteristic sign of Parkinson’s disease.
Another distinction is that some treatments effective in managing the symptoms of Parkinson’s patients often fail in PSP patients.
Although people with Parkinson’s disease may respond to medications or deep brain stimulation, these treatments are rarely helpful in people with PSP.
Symptoms of PSP worsen very quickly, leaving patients severely disabled within five years. In Parkinson’s disease, there are five stages that progress much more slowly, approximately every two to five years. Most people who have had the disease for 18 to 20 years use a wheelchair.
The cause of PSP is unknown, but some research suggests that it involves damage to cells in specific areas of the brain, primarily the brainstem, according to the National Institute of Neurological Disorders and Stroke (NINDS).
PSP often occurs randomly with no known cause, although in extremely rare cases it results from a genetic mutation that provides faulty instructions to brain cells.
Other theorized causes include exposure to unknown environmental factors that damage the brain and cellular damage caused by free radicals or reactive molecules produced by cells.
Virginia Democratic Rep. Jennifer Wexton, 55, reveals she has Parkinson’s disease on steroids and won’t seek re-election
Rep. Jennifer Wexton announced she will not run for re-election after being diagnosed with a rare form of progressive Parkinson’s disease.
Parkinson’s disease occurs when nerve cells or neurons in an area of the brain that controls movement, called the basal ganglia, are damaged or die. Normally, these cells produce dopamine, but when they deteriorate and produce less of the hormone, it causes movement problems.
As with PSP, scientists don’t know what causes these neurons to die.
Symptoms of PSP may include difficulty controlling the eyes and eyelids, loss of balance, slurred speech, difficulty walking or swallowing, changes in judgment, forgetfulness, personality changes and difficulty finding words.
This disease can lead to serious complications such as pneumonia, choking, or head injuries from falls.
Aspiration pneumonia, when food or liquids are inhaled into the airways or lungs instead of being swallowed, is the most common cause of death in people with PSP.
People with this condition are also at higher risk of falls and head injuries that can lead to death.
There are currently no tests or brain imaging that can definitively diagnose the condition, and a doctor will often review your medical history and perform physical and neurological tests.
Additionally, doctors will perform thorough examinations to help rule out other similar disorders.
In a few cases, brain imaging of a PSP patient may show narrowing at the top of the brain steam, which can help doctors examine brain activity in certain areas.
Currently, there is no cure for this disease. There is also no effective treatment to stop or slow the progression of PSP and symptoms generally do not respond well to medication.
However, walking aids can reduce the risk of falling, special glasses called prisms can reduce difficulty looking down, and supervised physical activity can keep joints flexible.
Ms Wexton revealed in April that she had been diagnosed with Parkinson’s disease, but in a statement on Monday she said her symptoms were not being managed. With additional medical advice and testing, doctors changed his diagnosis to PSP.
The MP said: “This diagnosis is difficult. There is no “improvement” with the PSP. I will pursue treatment options to manage my symptoms, but they don’t work as well with my disease as they do with Parkinson’s.
Ms Wexton said she would not seek re-election once her term ends and would instead “spend my precious time” with her husband and sons.