In the rigid person syndrome, the individual presents intense rigidity that can manifest itself in the whole body or only in the legs, for example. When these are affected, the person can walk like a soldier because he cannot move his muscles and joints very well.
This is an autoimmune disease that usually manifests between 40 and 50 years of age and is also known as Moersch-Woltmann syndrome or in English, Stiff-man syndrome. Only about 5% of cases occur in childhood or adolescence.
Rigid person's disease syndrome can manifest in 6 different ways:
- Classic shape where it affects only the lower back and legs; Variant shape when limited to just 1 limb with the dystonic or back posture; Rare shape when stiffness occurs throughout the body due to severe autoimmune encephalomyelitis; When there is movement disorder functional; with generalized dystonia and parkinsonism eCom hereditary spastic paraparesis.
Usually the person who has this syndrome does not only have this disease, but also has other autoimmune diseases such as type 1 diabetes, thyroid disease or vitiligo, for example.
This disease can be cured with the treatment indicated by the doctor but the treatment can be time consuming.
Symptoms
The symptoms of rigid person syndrome are severe and include:
- Continuous muscle spasms that consist of small contractures in certain muscles without the person being able to control, and Marked stiffness in the muscles that can cause rupture of muscle fibers, dislocations and bone fractures.
Due to these symptoms the person may have hyperlordosis and pain in the spine, especially when the back muscles are affected and may fall frequently because he is unable to move and balance properly.
Intense muscle stiffness usually arises after a period of stress as a new job or having to perform jobs in public, and muscle stiffness does not happen during sleep and deformities in the arms and legs are common due to the presence of these spasms, if the disease is not treated.
Despite the increase in muscle tone in the affected regions, the tendon reflexes are normal and therefore the diagnosis can be made with blood tests that look for specific antibodies and electromyography. X-rays, MRIs and CT scans should also be ordered to exclude the possibility of other diseases.
Treatment
The treatment of the rigid person must be done with the use of drugs such as baclofen, vecuronium, immunoglobulin, gabapentin and diazepam indicated by the neurologist. Sometimes, it may be necessary to stay in the ICU in order to ensure the proper functioning of the lungs and heart during the disease and the treatment time can vary from weeks to months.
Plasma transfusion and the use of anti-CD20 monoclonal antibody (rituximab) can also be indicated and has good results. Most people diagnosed with this disease are cured on receiving treatment.
