The National Institute of Neurological Disorders and Stroke states that stiff person syndrome is distinguished by muscle rigidity and spasms, heightened sensitivity to stimuli like sound and lights, and emotional distress that can lead to muscle spasms.
Dr. Emile Sami Moukheiber from the Stiff Person Syndrome Center at Johns Hopkins Medicine stated, “Falls due to severe spasms are highly prevalent. These spasms can be triggered by shocks, intense emotions, and cold weather.” It is often linked to muscular spasms, which can be quite intense. These spasms can result in falls, severe pain, and significant disability.
These spasms can be strong enough to fracture a bone and any fall can cause serious injuries.
Dion said in a video that the spasms affect “every aspect” of her daily life, sometimes causing difficulties while walking and preventing her from using her vocal cords to sing in the way she is accustomed to.
The symptoms of the syndrome can also lead to anxiety.
In a statement, Dr. Scott Newsome, director of the Stiff Person Syndrome Center, said in a video on the organization’s website: “They have an anxiety that is inherent to the disease and that anxiety actually feeds off the physical ailments of the disease that people may have, many patients, if not all.”
¿Quién sufre de los efectos del síndrome de la persona rígida?
Moukheiber stated that the disorder impacts approximately one million individuals, and the majority of general neurologists will only encounter one or two instances throughout their lifetime.
Newsome stated that the initial occurrence of rigid individual syndrome was documented in the 1950s, and traditionally the illness was referred to as ‘rigid male syndrome’.
In order to avoid confusion, the name was changed to rigid person syndrome and it has been discovered to affect twice as many women as men since then.
¿Cuáles son las causas del síndrome de la persona rígida?
According to the National Institute of Neurological Disorders and Stroke, the stiff person syndrome is believed to have features of an autoimmune disease.
In the brain, a significant neurotransmitter involved in the synthesis is affected by an enzyme that counteracts the GAD antibody, individuals with SPS have elevated levels, according to the institute, although the exact cause is not clear.
Newsome asserts that the average duration for an individual to obtain a diagnosis of stiff person syndrome is approximately seven years. Frequently, individuals, driven by the scarcity of the ailment and the vagueness of its indications, pursue medical assistance for persistent discomfort prior to acquiring neurologic treatment.
She said, “because in the initial exam, there are no distinctive characteristics of stiff person syndrome”, “sometimes, [patients] are labeled as crazy”.
¿Cuál es el enfoque de tratamiento para el síndrome de la persona rígida?
Medications, such as immunoglobulin, can help reduce sensitivity to triggers that could cause spasms or falls. Medications can also alleviate symptoms, but there is no known cure for the condition known as stiff person syndrome.
Botulinum toxin is also used in the Center for Stiff Person Syndrome, with analgesics, anxiolytics, and muscle relaxants being part of the treatment for this disease.
“If left untreated, the illness can cause a severe deterioration in daily life,” Moukheiber said.
However, with treatment, the symptoms can be managed.