What Journalists Need to Know About Stiff Person Syndrome

Journalists should be well-informed about Stiff Person Syndrome (SPS) to effectively communicate its complexities and challenges to the public. About one in a million people get stiff-person syndrome with one of three types: classic SPS, progressive encephalomyelitis with rigidity and myoclonus (PERM), and SPS variants. It affects twice as many women as men and usually develops in individuals aged 40 to 50. The complex nature of this syndrome causes many people to spend months or even years searching for the reasons for the symptoms, a situation not being helped by the fact that the symptoms can mimic some other autoimmune disorders. 

Here is what journalists should know about Stiff Person Syndrome before putting together any pieces associated with it.

Symptoms and Their Effects on Lifestyle

For those who live with stiff person syndrome, life can be a source of daily frustration.

The most prominent features of SPS involve painful muscle contractions and spasms typically initiated in the lower extremities and back. In most cases, the first muscles to become stiff are those of the abdomen, chest, and back. The symptoms can fluctuate in severity with no clear trigger or reason. The rigidity caused by the syndrome results in pain and an aching discomfort, and an abnormal posture may emerge as the stiffness increases, making the simple acts of walking and moving around a challenge.

Painful muscle spasms are also a feature in SPS, lasting anywhere from seconds to hours, and can involve the entire body or an acute pain focused on a specific area. Aside from difficulty moving, lifestyle is also affected because muscle spasms increase the risk of falls and subsequent injuries. Unfortunately, anxiety and agoraphobia can also rear its ugly head due to the unavoidable triggers while out in public, such as unexpected or loud noises, changes in temperature, and stressful events.

Due to mobility limitations, journalists will have to do whatever is necessary to accommodate the source if they are affected by SPS. They will very likely have to go to the source’s location. They may also have to have patience with last minute rescheduling if the individual with SPS is having a particularly debilitating day. There are plenty of individuals with SPS that can walk well enough to live without a wheelchair, but some do require one and that should be taken into account as well.

Causes and Treatment

SPS is believed to be an autoimmune disorder, wherein the body's immune system mistakenly attacks healthy tissues. Most individuals with SPS have unique antibodies, particularly anti-GAD65 antibodies, which interfere with the production of the neurotransmitter GABA, leading to nerve hyperexcitability and its resulting symptoms. When GABA levels are insufficient, nerve signals are not properly regulated, resulting in the symptoms associated with SPS. The common triggers of SPS symptoms are believed to be linked to disruptions in the GABA pathway. Treatment of symptoms involves medications such as benzodiazepines and muscle relaxants. Treatment targeting the immune system involves plasma exchange and intravenous or subcutaneous immunoglobulin.

Challenges Following Diagnoses

Here’s one story of many.

According to Time, A 74-year old patient named Maureen Materna is one of many who faced main roadblocks on the journey to diagnosing SPS. She lived an active and busy life and then one day started experiencing episodes of intense cramps in her feet and legs. Materna went to about 20 doctors over the next two years and felt acutely that her pain was not being taken seriously. She received mere (metaphorical) band aids on these issues until finally a neuromuscular specialist properly diagnosed her. Even now, Materna still comes across doctors that have no idea what stiff person syndrome is. Some have never even heard of it.

By understanding these key aspects of Stiff Person Syndrome, journalists can effectively inform the public about this rare but complex condition, providing valuable insights into its diagnosis, treatment, and impact on individuals' lives.