Canadian singer Celine Dion announced last week that she had a “very rare neurological syndrome”, which forced her to postpone concerts she was scheduled to perform in Europe.
The star appeared affected in a video clip she posted on Instagram, in which she explained that she She has had “health problems for a long time”. “I was recently diagnosed with a very rare neurological disease, Stiff Person Syndrome,” she said.
Sensitivity to sound and lights
Because of this disease, which translates into a progressive stiffness of the muscles, Celine Dion began to suffer from walking difficulties, while the disorder also prevented her from “using her vocal cords” in the way she wanted, according to the singer.
According to information published by CNN, “stiff person syndrome” is a rare neurological condition that may take years to diagnose and design a specific treatment for.
According to the National Institute of Neurological Disorders and Stroke in the United States, “stiff person syndrome” is characterized by muscle rigidity and spasms, increased sensitivity to stimuli such as sound and lights, and emotional distress that can cause muscle cramps.
The condition is often associated with muscle spasms, which can be very severe. “This condition can cause falls, severe pain and significant disability,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine. “Falls due to severe spasms are very common. These spasms can be triggered by Extreme emotions and cold weather,” according to what was reported by “Al-Sharq Al-Awsat” newspaper.
These spasms may be strong enough to break bones, and any fall can lead to serious injury.
Symptoms of the syndrome can also cause anxiety. “Many, if not all, patients have illness-related anxiety, and that anxiety is actually fueled by the physical ailments that people can develop,” said Dr. Scott Newsom, director of the Stiff Person Syndrome Center.
Mokheiber said the condition affects about one person in a million, and most neurologists will only see one or two cases in their lifetime.
The first case of stiff person syndrome was reported in the 1950s. Historically, the disease was referred to as “stiff man syndrome”. Since then, it has been found to affect twice as many women as men, and the name has been changed to Stiff Person Syndrome to avoid confusion.
Stiff person syndrome is thought to have hallmarks of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke.
Although the exact cause isn’t clear, according to the institute, “people with the syndrome have elevated levels of GAD, an antibody that works against an enzyme involved in the formation of an important neurotransmitter in the brain.”
Because the disease is rare and its symptoms are vague, people often seek care for chronic pain before they seek neurological care. On average, it takes about seven years for someone to receive a diagnosis of stiff person syndrome. In this context, Newsom said, “Sometimes, patients are described as insane, because on early examination, the hallmarks of stiff person syndrome are not present.”
There is no known cure for stiff person syndrome, but medications may relieve symptoms. Immunoglobulin medications can help reduce sensitivity to light or sound stimuli, which may help prevent falls or seizures.
Pain relievers, anti-anxiety medications, and muscle relaxants may be part of treatment for this condition. The Stiff Person Syndrome Center also uses botulinum toxin injections.
In this context, Mukheiber said, “If the disease is left untreated, it can cause severe impairment in daily life,” but with treatment, symptoms can be controlled.