Hope for people with rare disorder called stiff person syndrome

By Danny Halpin via SWNS

A rare neurological disorder called stiff person syndrome could be treated by replacing plasma in the blood, according to a new study.

The debilitating illness, also known as SPS, is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms

Researchers found that the radical treatment - known as plasmapheresis or therapeutic plasma exchange - eased the symptoms of many patients and allowed them more freedom of movement.

Symptoms of SPS include stiffness and spasms that usually begin in the leg and torso muscles but can over time affect the arms and face.

Most people with these disorders are women and symptoms can come and go during the early stages but eventually become constant.

Other people find they are unsteady as they walk and sometimes fall.

Study author Dr. Scott Newsome, a neurologist at Johns Hopkins University in Baltimore, MD, said: “Stiff person syndrome spectrum disorders are rare, and while there are some treatments for varying forms of these disorders, they are not always effective at reducing symptoms or preventing worsening of function.

“Finding a new way to treat a debilitating and painful disease that has few side effects or risks is a big win."

“The findings of our research help promote awareness of a treatment that may lead to relief for some people living with stiff person syndrome spectrum disorders.”

For the study, the scientists looked at the medical records of 36 people with SPS who were treated using plasmapheresis.

This cleans the blood by replacing the blood plasma with albumin, a product made from donated plasma to treat certain diseases.

Researchers found that this therapeutic plasma exchange was a safe and tolerable form of treatment for people with SPS.

Four of the people studied had complications such as catheter infection or bleeding, but no deaths or anaphylaxis were reported.

Additionally, 20 people, or 56 percent, saw improvement with this treatment and 12 people needed fewer medications to treat their symptoms three months after.

Researchers also reviewed other cases and identified an additional 42 people with stiff person syndrome who had the same treatment and found that 69 percent reported a temporary improvement in their condition.

Dr. Newsome said: “While the main focus of the study was safety, and we showed that complications from this treatment in people with stiff person syndrome are rare and manageable, it’s very important that many people saw improvements or maintained the same level of function.

“Further studies could confirm these results and help inform doctors when to use this treatment for stiff person syndrome spectrum disorders.”

Dr. Newsome’s research is due to be presented at the American Academy of Neurology Summer Conference in San Francisco later this month.