Indiana family struggling with rare diagnosis (PSP)

My online friend Joe Blanc posted this article today to the Society for PSP’s Forum. The article is about an Indiana family coping with progressive supranuclear palsy (PSP). The wife was diagnosed with anxiety, Alzheimer’s, and, finally, PSP. Swallowing is described as the main complication with PSP.

The concept of brain autopsy is raised in the article:

“Dr. Alberto Espay, assistant professor of Neurology at the University of Cincinnati, said it is very difficult to pinpoint PSP, and the only way to positively confirm a diagnosis is by an autopsy.”

If you are interested in making arrangements for a family member’s brain donation (or your own brain donation), please let me know. I can help!

Robin


Difficult Diagnosis 

Struggle with rare, fatal disease compels family to raise awareness
By Rebecca L. Sandlin
[email protected]noblesvilledailytimes.com

Chris Ryan doesn’t want other families to have to go through what hers suffered when her mother, Augie O’Herren, slowly began to deteriorate.
Augie and her husband, Roger O’Herren, had lived in Noblesville for years, and she was active in the community, Ryan said. But in 1994, at the age of 62, she began to have mysterious symptoms of “light-headedness,” followed by failing eyesight, poor balance and slowed speech. “It was such a long battle of trying to figure out what’s wrong,” Ryan said. “That’s the sad state. … So many different doctors’ opinions. It’s a science – nothing perfect.”

Augie was diagnosed with everything from anxiety to Alzheimer’s disease. But the family wasn’t satisfied with the doctors’ diagnoses.
Finally, they took Augie for an evaluation at the University of Chicago. There, a doctor told them her symptoms appeared in a pattern that suggested she had progressive supranuclear palsy, or PSP – a form of Parkinson’s disease.

According to a PSP Web site, PSP is a fatal, degenerative brain disease that has no known cause, treatment or cure. It causes complete disability by progressively impairing all movement, balance, vision, speech and swallowing. PSP patients may often show alterations of mood and behavior, including depression and progressive dementia.
It is estimated between 5,000 and 10,000 people are currently afflicted with the disease and as many as 10,000 more are undiagnosed or misdiagnosed.

Dr. Alberto Espay, assistant professor of Neurology at the University of Cincinnati, said it is very difficult to pinpoint PSP, and the only way to positively confirm a diagnosis is by an autopsy.

“We don’t have a readily-available blood test that we could use and say, ‘Yes – that’s what you have,” Espay said. “A high proportion of people that end up developing full-blown PSP may be misdiagnosed early on as Parkinson’s disease.”

Espay said the treatment of suspected PSP patients would be nearly the same as for Parkinson’s disease.

“We can then counsel patients as to what to expect, what to watch for so that the main sources of potential complications – namely, difficulty with swallowing … can be monitored and ideally kept at a point where they are not going to be losing the patient to complications,” he said.

Espay suggests patients go to a clinic that is dedicated to PSP research for a second opinion if they are uncomfortable with a doctor’s diagnosis.
Augie O’Herren finally succumbed to the disease in August 2006 at the age of 74. Her daughter, Kathleen O’Herren Huston, said she’s now “dancing, eating and reading – all the things she loved to do before this horrible disease took it away from her.”

Ryan said she hopes her family’s trial will help others with loved ones who are struggling with mysterious symptoms.

“Don’t give up – don’t just listen to one doctor, if your senses are telling you something different and what you’re reading is contrary,” Ryan said. “It did bring us some relief to know that we were not crazy – that all of her symptoms that she’s been complaining about actually do fit into something. … Keep on believing.”

Symptoms of PSP:
Gradual loss of certain brain cells causes slowing of movement and reduced control over walking
Balance problems
Difficulty speaking or thinking
Slow eye movement; complaints of not being able to read
Difficulty swallowing
Tight, small script when writing
On the Web
You can learn more about progressive supranuclear palsy by visiting www.psp.org. The O’Herren family journal is located at www.caringbridge.org/ visit/augieoherren.