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 won’t run for reelection after being diagnosed with a brain disorder she described as ‘Parkinson’s on steroids’.
The Democrat from Virginia had revealed in April she had Parkinson’s disease, which sees parts of the brain gradually waste away, affecting movement and speech.
Doctors later changed her diagnosis to progressive supranuclear palsy (PSP), a similar but much rarer disorder that impacts just 20,000 people in America.
For comparison, around 1 million people in the US have Parkinson’s.
The main difference is that PSP causes patients to deteriorate much more rapidly, causing harsher symptoms, particularly with speech, coordination and eating.
People diagnosed with PSP die within a decade of their diagnosis, whereas Parkinson’s patients can expect to live between 10 and 20 years after diagnosis.
Rep Jennifer Wexton is pictured above with her family. The congresswoman revealed her new diagnosis this week of a rare neurological condition called progressive supranuclear palsy (PSP)
PSP is different from Parkinson’s in that it usually begins later in a person’s life and worsens quickly. Problems with speech and swallowing are much more common and severe in PSP patients compared to those with Parkinson’s and it is rare for PSP patients to develop a tremor, a hallmark sign of Parkinson’s disease
PSP, a form of ‘Parkinson’s on steroids,’ may be under- or misdiagnosed because its symptoms closely resemble those of Parkinson’s disease. However, there are several symptoms that can differentiate the two.
Problems with speech and swallowing are much more common and severe in PSP patients compared to those with Parkinson’s and it is rare for PSP patients to develop a tremor, a hallmark sign of Parkinson’s disease.
Another distinction is that some treatments successful in symptom management for Parkinson’s patients often fail in PSP patients.
While people with Parkinson’s disease may respond to medications or deep brain stimulation, those treatments are rarely helpful in people with PSP.
PSP symptoms worsen very quickly, leaving sufferers severely disabled within five years. With Parkinson’s disease, there are five stages that progress much slower, approximately every two to five years. Most people who have had the disease for 18 to 20 years are in a wheelchair.
The cause of PSP is unknown, but some research suggests it involves damage to cells in specific areas of the brain, mainly the brain stem, according to the National Institute of Neurological Disorders and Stroke (NINDS).
PSP often occurs randomly without a known cause, though in extremely rare cases, it arises from a gene 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 on steroids’ and won’t seek reelection
Rep. Jennifer Wexton announced she won’t seek reelection after receiving a diagnosis of 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, become damaged or die. Normally these cells produce dopamine, but when they deteriorate and produce less of the hormone, it causes movement problems.
Similar to PSP, scientists do not know what causes these neurons to die.
PSP symptoms can include difficulty controlling eyes and eyelids, loss of balance, slurred speech, difficulty walking or swallowing, changes in judgment, forgetfulness, personality changes and difficulty finding words.
The condition can lead to serious complications such as pneumonia, choking or head injuries from falls.
Aspiration pneumonia, when food or liquid is breathed into the airways or lungs instead of being swallowed, is the most common cause of death in people with PSP.
People with the condition also have a higher risk of falls and head trauma that could lead to death.
There are no current tests or brain imaging to definitively diagnose the condition and a doctor will often review your medical history and perform physical and neurological tests.
Additionally, doctors will perform thorough exams to help rule out other similar disorders.
In few cases, brain imaging of a PSP patient may show shrinking at the top of the brain steam, which can help doctors examine brain activity in certain areas.
At this time, there is no cure for the condition. There are also no effective treatments to stop or slow the progression of PSP and symptoms usually do not respond well to drugs.
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 limber.
Ms Wexton revealed in April she had received a Parkinson’s diagnosis, but in a statement issued Monday, said her symptoms were failing to be managed. With addition medical opinions and tests, doctors modified her diagnosis to PSP.
The congresswoman said: ‘This diagnosis is a tough one. There is no ‘getting better’ with PSP. I’ll continue treatment options to manage my symptoms, but they don’t work as well with my condition as they do for Parkinson’s.’
Ms Wexton said she will not seek reelection when her term is up and will instead ‘spend my valued time’ with her husband and sons.