Celine Dion recently announced that she suffers from stiff-person syndrome, an incurable neurological disorder. Dion will celebrate her 55th birthday this Thursday; for years now, she has been dealing with this illness.
Sister Claudette shared with Le Journal de Montreal that the singer of “My Heart Will Go On,” Leahy, is currently listening to top researchers as part of her search for a cure.
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What is Stiff Person Syndrome?
Celine Dion made headlines worldwide when she revealed in December 2022 that she is living with stiff person syndrome, an uncommon neurological disorder characterized by muscle spasms, stiffness, and light/sound sensitivity triggers. There is no cure for stiff person syndrome; however, medication or other treatments can help manage its symptoms to control it over time.
SPS is thought to be caused by issues with the body’s production of GABA enzyme, an essential factor in slowing nerve cell activity by blocking specific signals. If insufficient GABA is available, nerve cells become overexcitable and send incorrect alerts directly to muscles, triggering spasms that may be caused by emotions or external stimuli like hearing the sound of a car horn or sirens. According to John Hopkins Medicine, SPS affects females more than males and is typically diagnosed between 30-50.
Doctors typically perform several steps to identify SPS, including reviewing medical histories and conducting comprehensive physical exams, neurological exams, blood tests, and spinal fluid analysis. SPS is a progressive condition; without treatment, it may progress into difficulty walking and performing daily tasks; posture issues may develop into hunching-over postures, while street noises or sudden movements such as car horns may trigger spasms that cause people to fall over.
Doctors can treat SPS using medication such as GABA receptor agonists such as benzodiazepines and muscle relaxers like baclofen. Neuropathic pain medications such as gabapentin and pregabalin may also help relieve painful spasms. Evidence suggests intravenous immunoglobulin, made up of antibodies from thousands of healthy individuals donated via donation drives, may benefit some people living with SPS.
The Cleveland Clinic notes that conservative therapies and medications are typically the best approach to treating dystonia. Stretching exercises, heat and water therapy, massage therapy, acupuncture, and pain relievers may all prove useful; in particular, acupuncture may assist with balance issues, while botulinum toxin injections may reduce spasms significantly.
What are the symptoms of Stiff Person Syndrome?
SPS symptoms often manifest with stiffness throughout the body, usually starting in the torso and spreading throughout. Muscles become enlarged while spasms often become very painful and persistent – often brought on by noise, touch, or emotion; this condition usually lasts hours before subsiding on its own.
SPS is an autoimmune condition in which the immune system attacks the body’s healthy tissues. Most individuals diagnosed with SPS possess specific antibodies called anti-glutamic acid decarboxylase antibodies in their blood that block an enzyme needed for producing Gamma-Aminobutyric Acid (GABA), which slows down muscle activity. As a result, nerves send uncontrollable signals to muscles, prompting them to contract uncontrollably and become stiff and rigid over time.
SPS may cause other symptoms, including pain, sleep disruptions, fatigue, and depression. Although its severity varies weekly and from person to person, SPS can become disabling over time. SPS increases your risk of falling, which could lead to severe injuries; difficulties with balance can cause dizziness or lightheadedness, making walking or getting up from bed difficult or impossible altogether.
SPS may not have a cure, but treatments can alleviate most common symptoms and help you live your best life. An SPS treatment plan usually combines anti-inflammatory drugs, muscle relaxers, immunosuppressants, and immunomodulator medication to ease stiffness, while immunosuppressants reduce antibodies that attack the body.
Psychiatric therapy may also assist, particularly if emotional distress is exacerbating symptoms. Psychotherapy teaches coping skills and helps mitigate triggers that cause muscle spasms. Physical therapy may also prove helpful; however, high-impact exercises could exacerbate symptoms further.
Since Celine Dion first shared her diagnosis of SPS on social media in December 2022, there has been an increased awareness and understanding of this condition characterized by more than just stiff muscles. According to Tara Zier of the SPS Foundation, Celine’s openness has led to more empathy towards this illness, which manifests as more than stiffness.
What is the treatment for Stiff Person Syndrome?
Though there is no cure for stiff person syndrome, treatments exist that can help ease discomfort. These may include medications to decrease rigidity, muscle relaxants, and immunotherapies. Medicines may also help manage emotional triggers that lead to spasms.
According to the National Institute of Neurological Disorders and Stroke, SPS is a neurological condition with “features of an autoimmune disease.” People living with SPS often exhibit fluctuating muscle rigidity that may be triggered by noise, cold temperatures, or sudden movement; spasms caused by this condition may result in falls, leading to further injuries and even more severe health complications.
While the cause of SPS remains unknown, it is thought to result from antibodies attacking healthy nerve cells in specific brain or spinal cord areas. SPS typically causes stiffness and difficulty moving; symptoms may include muscle spasms, balance issues, and depression or anxiety.
The disease can strike anyone at any age; however, women are twice as likely to develop it than men and typically present between ages 30-50. Furthermore, it often coincides with other autoimmune conditions like type 1 diabetes, thyroiditis, vitiligo, and myasthenia gravis.
Symptoms of the disorder can be effectively managed using medications like baclofen, dantrolene sodium, and diazepam; for more severe cases, immunotherapies may also regulate an individual’s immune response. Intervention may involve intravenous immunoglobulin, a medication to ease your body’s natural reaction to certain substances. Or it could include decreasing immune cell numbers that produce antibodies to stop those produced from attacking healthy tissue. Many patients have found relief through these interventions; however, chronic illness requires time to heal completely, and symptoms may resurface at any point in time. Psychotherapy has also proven effective for SPS patients, providing them with skills to cope with chronic illness and minimize its negative impacts. Some have even reported how regular exercise, massages, or acupuncture treatments help alleviate their symptoms.
What is the prognosis for Stiff Person Syndrome?
Muscle stiffness and spasms that come and go can lead to other symptoms, including unsteady gait and double vision. Although men and women alike are susceptible, older people often show more severe signs than their counterparts, usually starting between 30-60. However, any age may experience symptoms. Diagnosing this condition involves having fluid drawn from your spinal canal by a doctor for testing purposes before looking for antibodies against the GAD enzyme (which produces GABA as an inhibitory neurotransmitter that inhibits brain activity) from your spinal canal fluid for testing purposes; blood tests then check for antibodies against GAD enzyme (used to produce GABA), an inhibitory neurotransmitter used by your brain to slow brain activity down brain activity.
Doctors cannot treat your symptoms directly but can manage them to help you live a more normal life. Options available to treat the condition include medications and physical therapy; specific examples would consist of diazepam for muscle spasms, baclofen to relax muscle stiffness, and neurolytic pain medication like gabapentin or pregabalin to ease discomfort or sensitivity to touch and sound.
Other treatments for IBS-D include heat and water therapy, massage, acupuncture, exercise, and Botox injections. Unfortunately, its cause remains unclear but could include family history, specific infections, or stress. At the same time, some researchers believe it might even be an autoimmune disorder similar to multiple sclerosis or Parkinson’s disease.
An expert team should be selected when treating stiff person syndrome. The Cleveland Clinic’s Stiff Person Syndrome Center offers experts in ophthalmology, rheumatology, neurology, and neuromuscular disease to collaborate in treating this condition.
Stiff Person Syndrome can be debilitating and affect balance, walking, speaking, and skin health, causing itching and reddening, leading to fatigue and depression in some cases. Yet many can manage symptoms through medication and alternative therapies to overcome it.
