stiff person syndrome symptoms

A healthcare provider may use several tests to rule out these conditions and look for specific signs of stiff person syndrome. Because stiff person syndrome compromises my immunity, I can't see many people these days. Date 06/2024. In part, this is because of the unpredictability of the disease, but patients also have lower levels of the neurotransmitter GABA, which regulates anxiety. Generally, SPS is suspected based on characteristic symptoms. She enjoys cooking, yoga, reading, music and walking her two rescue dogs. Quincy, MA 02169 Because of its symptoms, SPS is often misdiagnosed as: To obtain a diagnosis, your doctor will perform a physical examination and ask you about your medical history. The team at Indiana University Health ran every test they could think of trying to uncover the reason why my muscles felt so tight, but still felt at a loss about what was behind my pain. Doctors havent yet pinpointed an exact cause of stiff person syndrome. Sudden withdrawal of medication in individuals with SPS may result in a life-threatening situation with overwhelmingly severe muscle spasms. Phone: 203-263-9938 Reaching a diagnosis for Stiff Person Syndrome can be difficult. msn back to msn home lifestyle. Your healthcare team will devise a management plan thats unique to you and your symptoms. Stiff-person syndrome typically progresses over 10 to 20 years, but doctors say they have treatments to offer patients cope with the degenerative disease. 2002;17:853-866. Email: [emailprotected], Some current clinical trials also are posted on the following page on the NORD website: Medscape Updated: May 30, 2017. amphiphysin antibodies) is usually appropriate. Muscular rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the muscle spasms. Elevated GAD titers, up to 10 times above normal, also are seen in diabetes but in SPS the titers are very high (at least 10 times above the range seen in diabetes) or are present in the spinal fluid. People with classic SPS may have frequent muscle spasms and walk with a stiff gait, and they can be in pain most of the day. The stiffness can cause you to fall when standing or walking, and this falling down has been described as log-like since you become very rigid and cant stop yourself or break the fall. Cancer is rarely associated with SPS (in less than 5% of cases). Generally, doctors focus on mitigating pain, relaxing muscles, and suppressing a person's overactive autoimmune response. Is It (Finally) Time to Stop Calling COVID a Pandemic? Suite 310 Some people may be too disabled to walk or move. Fewer than 5,000 people in the U.S. have this disease. IVIg contains immunoglobulins (natural antibodies your immune system produces) donated by thousands of people with healthy immune systems. In some extreme cases, spasms of the chest muscles can affect a persons ability to breathe. Most people with SPS have elevated (higher) levels of GAD antibodies. PERM is more rapidly progressive than other forms of SPS; onset of symptoms usually occurs over several weeks. They can help connect patients with new and upcoming treatment options. People with stiff person syndrome frequently experience painful muscle spasms that can carry on for hours. Could the hunger hormone help improve heart function in people with heart failure? Meghan Holohan is a contributing writer who covers health and parenting for TODAY.com. GAD plays a role in making a neurotransmitter called gamma-aminobutyric acid (GABA), which helps control muscle movement. INTERNET Muscular dystrophy refers to a group of more than 30 disorders that impair the bodys ability to build and restore muscle. Dystonia is a neurological movement disorder that causes muscle contractions or spasms. If you notice new symptoms or side effects of your medications, talk to your healthcare provider. Stiff person syndrome is so rare that in the U.S., fewer than 5,000 living people have been diagnosed with it. Some people may be too disabled to walk or move. National Center for Advancing Translational Sciences, Moersch-Woltman syndrome; SMS; SPS; Stiff man syndrome; Stiff person syndrome and related disorders. The twitching or jerking of muscles cannot be controlled by the person experiencing it. Meinck HM, Thompson PD. Neurological disorders affect the brain, spinal cord, and nerves. For the latest news, weather, sports, and . If your provider suspects stiff person syndrome, tests to confirm the diagnosis may include: Treatment for stiff person syndrome is based on your symptoms. Symptoms can take several months to a few years to develop. Most people with SPS improve with medications, but it can still be difficult to manage triggers that cause muscle spasms. If you have stiff person syndrome, youll need to see your healthcare team regularly to check if your treatment is working and to monitor the progression of your symptoms. You might become afraid to leave the house since the muscle spasms and stiffness are so unpredictable. These symptoms can lead to difficulty walking and, over time, even greater disability. The main symptoms of Stiff Person Syndrome are muscle stiffening in the torso and limbs, along with episodes of violent muscle spasms. Our Information Specialists are available to you by phone or by filling out our contact form. In: NORD Guide to Rare Disorders. Although currently, no therapy makes stiff person syndrome go away, treatment can help minimize the severity of symptoms and improve quality of life. High doses of diazepam will suppress the characteristic EMG results. Johnston MV, Adams HP, Fatemi A, editors. Common symptoms of problems in the nervous system include trouble moving, speaking, swallowing, breathing, or learning. While my health care team is great, I often have to be assertive when explaining how I feel and what I need because its such a rare disorder. Prominent SPS symptoms include muscle stiffening and painful muscle spasms that can be provoked by emotional stress or, again, outside stimuli. A doctor may make a diagnosis of SPS when there is no better explanation for the symptoms and exam findings. But I also hope for better research so that others don't have to live with so much pain. The left side of my body felt so tight that Id ask my daughters to help stretch it. I once thrived on pressure but now I spend my days managing my health. In some people, the spasms are brought on by certain exercises or touch. It may. Published March 15, 2023 3:19 a.m. PDT. It commonly causes muscle stiffness and painful spasms that worsen over time. "Electrophysiology testing and antibody testing in serum or cerebrospinal fluid assist in making the correct diagnosis," says Dr. McKeon. Affected individuals may also benefit from baclofen, usually given in addition to benzodiazepines. GARD does not currently have information about the cause of this condition. Its important to make sure youre getting the support you need and to stay attentive to your health. Blood is removed from an affected individual and blood cells are separated from plasma. Plasmapheresis may be of benefit in individuals with SPS. No treatment eliminates or cures SPS, but medications and other therapies can help manage symptoms and improve quality of life, and some treatments may help prevent disease progression. With partial SPS, tight muscles and spasms are limited to a specific area, usually one leg. March 15 may be the ninth annual International Stiff Person Syndrome Awareness Day, but it is the first time the event is attracting significant public attention, thanks to Cline Dion's . I'm still learning to navigate life with this condition. My thinking even becomes sharper after my infusion. On good days, I try to read and cook, though I often have to sit down because its tough to stand for a long time. I wish I hadnt lived in denial and I sought help earlier. As the disease progresses, stiffness of the leg muscles develops, and is often more pronounced on one side than the other (asymmetrical). People with SPS can have other symptoms, which are thought to be related to the type of SPS they have. International Parkinson and Movement Disorder Society. Affected individuals should talk to their physician and medical team about their specific case, associated symptoms and overall prognosis. In some cases, SPS can be fatal. Philadelphia, PA. 2000:761-762. Stiff-person syndrome (SPS) is a rare acquired neurological disorder characterized by progressive muscle stiffness (rigidity) and repeated episodes of painful muscle spasms. Another less common type of the syndrome is SPS plus, which is best described as a combination of classic features (spasms and stiffness) and symptoms that suggest brainstem and/or cerebellar dysfunction. Electromyography or EMG is also sometimes recommended to study the electrical activity of skeletal muscles. Stiff Person Syndrome. Testing for stiff-person syndrome is done only at Mayo Clinic Laboratories. (For more information on this disorder, choose paraneoplastic neurologic syndromes as your search term in the Rare Disease Database.). People with SPS often develop mental and emotional symptoms alongside physical symptoms, which can greatly affect their quality of life. Early on, stiffness may come and go, but it gradually becomes fixed. The symptoms of SPS often progress slowly and can lead to disability. Spasms may occur randomly or be triggered by a . Follow her on Twitter to see her recent stories. Some individuals may need to use an assist device such as a cane, walker or wheelchair. Here are some answers to common questions about SPS. The nervous system is made up of the brain, spinal cord, and nerves. "This neurological disorder can affect anyone at any age," says Dr. Andrew McKeon, a Mayo Clinic neurologist and expert in autoimmune neurology and movement disorders. Affected individuals may also experience low-grade fever, difficulty in swallowing (dysphagia), difficulty breathing, alteration in the rhythm of the heartbeat, and convulsions. Floeter MK. 1982;45:1159-1160. Share. Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms. Stiff-person syndrome often occurs in people with type 1 diabetes, certain autoimmune disorders, or certain kinds of cancer. Symptoms of the following disorders can be similar to those of SPS. Ive had to stop working and I have been living with my daughter. A study funded by the National Institute of Neurological Disorders and Stroke (NINDS) showed that intravenous immunoglobulin (IVIg) treatment is effective in reducing stiffness, sensitivity to noise, touch, and stress and for improving gait and balance for people with SPS. Follow your doctors instructions regarding medication. People worry they will experience a bout of spasms or stiffness in public. As my immune system is compromised, I have not seen a lot of friends in real life during the COVID-19 epidemic, but I still try to keep in touch with people. Over time, walking can become more and more difficult. People with SPS often have heightened sensitivity to noise, sudden movements, and emotional distress, which can set off muscle spasms. SNRIs have previously been shown to worsen the EMG activity and clinical symptoms in SPS. We link primary sources including studies, scientific references, and statistics within each article and also list them in the resources section at the bottom of our articles. The symptoms of tetanus usually last for three to four weeks. Murinson, BB, Butler, M, Gleason, S, et al. Stiff man syndrome and related conditions. Stiff Person Syndrome. Spasms may occur randomly or be triggered by a variety of different events including a sudden noise or light physical contact. Stiff person syndrome (SPD) is a rare neurological disorder that causes periods of muscle pain, stiffness, and spasms. Consider participating in a clinical trial so clinicians and scientists can learn more about SPS and related disorders. Antibody titers are important for the diagnosis of SPS. Some experts feel that immune-based therapies can help limit the progression of SPS for some people, and they advocate for these treatments long term. Antibodies to GAD-65 are associated with several other disorders including diabetes. Dupond JL, Essalmi L, Gil H, Meaux-Ruault N, Hafsaoui C. Rituximab treatment of stiff-person syndrome in a patient with thymoma, diabetes mellitus and autoimmune thyroiditis. J Neurol Neurosurg Psychiatry. The stiffness and muscle spasms are extremely similar to those found in classic stiff-person syndrome. Learn about symptoms, cause, support, and research for a rare disease. Some people with stiff person syndrome who have higher levels of disability may need help walking and moving around in other ways, and their activities can become limited. People with stiff-person syndrome often experience rigidity in their torso and limbs, as well as severe muscle spasms that can cause them to fall down. How can I or my loved one help improve care for people with s, those who are healthy or may have an illness or disease. Celine Dion has. National Institute of Neurological Disorders and Stroke. Scientists don't yet understand what causes SPS, but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord. For months, doctors didn't know what was causing my extreme muscle pain, fatigue and stomach upset. Difficulty walking, and the person might space the feet wide to feel more steady, A stiff or rigid posture due to ongoing spasms in the back or trunk, Unsteadiness and falling because of sudden spasms, which can lead to injuries, Shortness of breath if SPS affects muscles in the chest, Exaggerated curve (hyperlordosis) in the lower back that develops over time due to muscle tightness, and changes in the spine alignment can lead to spinal cord compression (, Anxiety and agoraphobia due to falls caused by spasms, Oral muscle relaxers, such as diazepam, clonazepam, baclofen or tizanidine, Oral medications that are not muscle relaxers but that have some effect on the GABA neurotransmitter, including gabapentin, pregabalin and tiagabine, Botulinum toxin, which is sometimes used when there are distinct areas of muscle spasm/tightness, Intravenous (injected into a vein) or subcutaneous (given as an infusion under the skin) immunoglobulin, Intravenous immunosuppressant therapy (e.g., rituximab), Oral immunosuppressant therapy (e.g., mycophenolate mofetil, azathioprine), Physical therapy that focuses on stretching, deep tissue myofascial techniques, balance/gait, ultrasound therapy and heat therapy, Transcutaneous electrical nerve stimulation. Symptoms can take several months to a few years to develop. Stiff person syndrome (SPS) is a rare chronic condition that causes muscle stiffness and painful muscle spasms. N Engl J Med. Policy. The increased risk of falls also becomes a growing concern as SPS worsens. When cancer is found in people with SPS, it is most commonly breast or lung cancer, and it is typically identified within a few years after SPS symptoms start. SPS is an extremely rare disorder. Immune-based therapies for stiff person syndrome include: Together with medications, nonmedication therapies can make stiff person syndrome symptoms more manageable. Symptoms of SPS may include stiff muscles in the torso, arms, and legs, says NINDS. Symptoms may start to appearas an Adult. We rely on our muscles all day, every day, to carry our coffee, type on our computers, and walk to the grocery store. 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