Celine Dion, the famous Canadian singer who performed My heart Will go on, the main theme of the movie Titanic, has surprised her followers on Instagram by announcing that she suffers from stiff person syndrome, a rare autoimmune disease that affects a out of every million people, and which appears to be responsible for the muscle spasms he suffers from. “I’ve had health problems for a long time, but I wasn’t ready to say anything before,” says the singer in a video recorded in English and French in which she addresses her audience to announce that she will delay the tours she planned to do in 2023 for different European countries.
What is stiff person syndrome and what are its causes?
But what is this disease that has prevented the interpreter from fulfilling the commitments made? The rigid person syndrome –RPS for its acronym in English– is a neurological disorder that especially affects the female sex –twice as many women suffer from it as men– and the National Institute of Neurological Disorders and Cerebrovascular Accidents of the United States indicates that it causes fluctuating muscle stiffness in the torso and extremities, increased sensitivity to stimuli such as touch, noise, or light, and muscle spasms.
The origin of this disorder is unknown, but it is considered that it could be due to an autoimmune reaction, which occurs when the individual’s own immune system attacks their organs and tissues; in this case, the antibodies produced by the body would attack the nerve cells in the spinal cord that are responsible for controlling muscle movement.
Stiff person syndrome mainly affects the brain and spinal cord (central nervous system), and has been associated with other autoimmune diseases such as diabetes, vitiligo, thyroiditis or pernicious anemia, and some types of cancer, such as breast, lung, thyroid, kidney, colon, and Hodgkin lymphoma.
Symptoms and Diagnosis of Stiff Person Syndrome
The symptoms that characterize the rigid person syndrome generally begin to manifest between the ages of 30 and 60, and are:
- Intermittent muscle spasms, which over time become continuous.
- Muscle stiffness, which gets progressively worse.
- Difficulty walking or moving. Those affected may even have problems going outside because when exposed to noise they can increase spasms or suffer falls.
- Exacerbated sensitivity to stimuli such as noise, touch or light, which cause the patient to startle.
- abnormal postures.
Anxiety and depression that can develop due to the difficulties they experience in carrying out their daily tasks or interacting socially, but also due to a decrease in the levels of neurotransmitters that help maintain a good mood.
The disease can be confused with others such as Parkinson’s, fibromyalgia, multiple sclerosis, anxiety, or some type of phobia, and although the symptoms may lead one to suspect that it is this syndrome, electromyography is used to confirm the diagnosis -which measures the electrical activity of muscles and nerves – and a blood test to check for elevated levels of certain antibodies, such as glutamic acid decarboxylase (GAD), an enzyme involved in the production of the neurotransmitter GABA (gamma-aminobutyric acid). )
How is stiff person syndrome treated?
There is currently no cure for stiff person syndrome, so treatment is aimed at relieving symptoms as much as possible and muscle relaxants, especially diazepam, or baclofen, are often used to treat muscle stiffness and spasms. Corticosteroid drugs can also be helpful, but their long-term use has many side effects.
Immunoglobulins are another therapeutic option, and a solution with different antibodies from donors can be administered intravenously, which alleviate symptoms for long periods of time (up to a year).
Sometimes rituximab, a drug that modifies the activity of the immune system, or plasmapheresis, a medical technique in which toxic substances in the blood, including abnormal antibodies, are filtered out. To control this syndrome, immunotherapy is also being tested, which stimulates the body’s natural defenses, as well as physiotherapy and psychotherapy.