United States: The latest revelation by this world-adored singer, Celine Dion, showed that she struggled with a rare health ailment known as stiff-person syndrome.
More about Stiff-person syndrome
Stiff-person syndrome, or SPS, is an uncommon autoimmune disease characterized by the fact that the body’s defense mechanisms begin attacking tissues. These off-target cells, in the case of SPS, go on to attack the neurological system and use up a chemical messenger known as gamma-aminobutyric acid, or GABA, that informs muscles when they should be relaxed.
According to Dr. Marinos Dalakas, a Thomas Jefferson University neurologist who specializes in stiff-person syndrome, generally, muscles work in opposing pairs, where “when you contract a muscle, the opposite muscle—say, the tricep, when the bicep contracts—relaxes,” as Yahoo Life reported.
Dalakas added, “Otherwise, all of us would be stiff.” For people with SPS condition, occasionally, it leads to stiffness in the legs, arms, and torso, as well as muscle spasms.
Different symptoms for different people
As Dr. Scott Newsome, the head of the Johns Hopkins Stiff Person Syndrome Center, noted, similar to various autoimmune diseases, SPS also exists on a spectrum. Hence, its symptoms appear quite different in different people.
According to the experts, the most visible form of the disease “typically will present with insidious stiffness in the legs more than the torso,” where some people might show stiffness in the arms area, as Newsome noted.
Additionally, the symptoms mostly appear in middle age, however it can also be seen in elderly people and even children.
Moreover, the stiffness and muscle spasms might persist anywhere from a few minutes to several hours.
“Spasms can be prompted by triggers including good or bad stress, hot or cold weather, or tactile stimuli,” Newsome added.
With the progression of the condition, the stiffness starts to spread and becomes more regular. In such cases, people find it hard to stand steadily on their feet, which leads to falls, as well as a fear of falling also being generated, thereby leading an individual to avoid independent activities.
As Newsome added, “Anxiety is part of the disease,” and “People can have a lot of falls related to balance issues or mobility problems if they have severe spasms. That can lead to people not going outside of their houses.”
No cure for SPS
According to health experts, there is no cure for SPS. However, immunotherapy can be helpful in slowing down the overactivity of the immune system.
Newsome said these “are the types of treatments that I feel have the potentially greatest impact on preventing progression,” and, They don’t reverse time,” however these treatments stand helpless when the disease is diagnosed during the later stages, as Yahoo Life reported.
Other medications including anti-seizure, Botox injection, muscle relaxant such as tizanidine and Valium that belongs to the class of anti-anxiety or sedative are prescribed on a daily basis to help in managing stiffness as identified by the Stiff Person Syndrome Research Foundation.
Additionally, physical therapy, yoga, massage, and therapy or medication play a significant role in the treatment of anxiety and depression and also play a crucial role in the life quality of individuals with SPS, according to Newsome.
To add to it, he said, “Using a multi-pronged approach can help patients the most,” as Yahoo Life reported.