What it’s like to live – and even thrive – with rigid personality syndrome

You probably haven’t heard the name “Stiff Personality Syndrome” until the Superstar singer came along Celine Dion announced her battle with a rare and often misdiagnosed disease.

Now a local woman will tell us what it’s like to live – even thrive – with wasting syndrome.

In December 2022, in a message to her fans, Dion spoke of the one-in-a-million people battling the same illness she battled offstage.

“I was like, ‘Oh my God, I feel so bad for her,'” Moira Pope said. “But I was very happy because I knew she had a huge voice and it would be recognised.”

Pope, 54, was diagnosed in 2021.

She has always been active, but says: “I did notice that I was falling, but I put it down to tripping or going too fast. I fell up and down the stairs.’

They assumed multiple sclerosis – then stenosis of the cervical spine. But the operation did not help her walk.

Dr. David Randall is a neurologist at Advocate Health Care.

“There is no single absolute characteristic that separates it from other diseases, so people look for things like: Did they have a stroke? Did they have spinal cord injuries? Do they have Parkinson’s disease?’ he said. “So there are other neurological conditions that can have some difficulty walking and feeling stiff.”

“Everybody asks, ‘What do you have?'” Pap said. “And then you have to talk about it forever because you have to talk about how rare it is.”

Most patients often go years without a proper diagnosis.

“I’ve had an MRI, a CT scan, a PET scan, I mean all of it, many times,” Papp said.

Signs of SPS don’t necessarily show up on imaging, but there are biomarkers that help doctors make the call. Pep’s blood has an elevated level of protein – GAD-65.

“As far as we know, it’s an autoimmune disease, and it recognizes a protein that we need as foreign,” Randall said. “And it attacks that protein, and it stops controlling muscle tension.”

Papp’s treatment regimen includes weekly infusions of immunoglobulin, a drug that works on an overactive immune system.

“Someone can just be walking and their muscles get really tight and they fall to the ground,” Randall said. “And that’s why there’s injuries involved as well.”

“I learned that a tough man has to move,” Pope said. “You can’t sit and lie in bed. Even if you don’t feel like moving, you have to move. So I have a new group of friends at the health club because I’m there every day.”

To learn more about Tough Man Syndrome, visit stiffperson.org

Even the muscles involved in breathing, swallowing and speaking can be affected. According to Dion, the muscle spasms affected her mobility and vocal cords.

Although Pep had never sung before her diagnosis, she has now taken it up on her own and recently performed on stage for the first time.

“Maybe there’s something in me that says, you know what? You have to live life to the fullest!” she said.

Tough man syndrome usually progresses over 10-20 years. But doctors say they are happy to be able to offer the treatment to patients with the degenerative disease.