Rigid person syndrome is a very rare neurological disorder. It is a movement disorder that affects the central nervous system (brain and spinal cord). It is characterized by symptoms such as stiffness and spasms in the muscles of the trunk, legs and different muscle groups of the body. In the beginning, these symptoms may appear at regular intervals, while in later stages, the spasms and stiffness may remain constant. Treatment is usually aimed at controlling the symptoms and complaints.
Muscle spasms can occur suddenly or can be triggered by exposure to sounds, light touch, or an emotional event such as sadness or stress. For this reason, people are very sensitive and sensitive to sounds and touches. In later stages, the severity of the disease may increase, and in some cases, walking or movement may be restricted. In addition, this condition can reach degrees that prevent the patient from moving.
What are the Symptoms of Rigid Person Syndrome?
Symptoms of rigid person syndrome can appear suddenly. The severity of the symptoms may vary from person to person and depending on the progression of the disease. The contractions, pain and muscle spasms that people experience in this disease can last for hours.
It usually manifests itself with stiffness and contractions in the trunk and abdominal muscles. It can progress with pain in the joints and muscles, stiffness in the muscles and body aches.
Furthermore, the progression of symptoms and complaints may vary from person to person. In some cases, progression may occur after a few months, while in others it may take several years. As the symptoms progress, the person may have difficulty standing and walking.
The contractions can cause people who are standing or walking to suddenly fall. In these cases, psychological symptoms and disorders such as anxiety and depression may also be observed.
Since the resulting muscle spasms and the severity of these conditions are not at certain levels and cannot be predicted, people do not want to go out in public.
These people, who can lock themselves at home, may experience some psychological problems such as depression and anxiety as they stay away from their social relations, friends and environment.
Muscle spasms can occur without a cause or can be triggered by a loud noise, physical contact, being in a cold environment or exposure to a stressful situation that causes an emotional response.
Muscle spasms can affect only a specific area, or they can affect the whole body. The number of muscle spasms decreases during sleep.
Symptoms and signs may initially manifest themselves at regular intervals, but may remain constant in the later stages. In these cases, the person may reach levels where he/she cannot move.
What are the Causes of Rigid Person Syndrome?
There are many researches on the subject. However, as a result of the researches, there is no definitive result and explanatory data about the causes of rigid person syndrome.
Among the factors that may cause this condition, it is thought that there is an autoimmune disorder known as the immune system attacking its own cells.
The bodies of many people with the condition produce antibodies that attack an enzyme called glutamic acid decarboxylase (GAD).
The GAD enzyme is involved in the production of a neurotransmitter called gamma-aminobutyric acid (GABA), which helps control muscle movement.
It is thought that the immune system of people with the syndrome abnormally attacks the GAD enzyme in a way that reduces the amount of GABA in the body.
A lesser known cause is amphiphysin. Although it is not seen very often in people with this syndrome, it is thought to be one of the causes. This protein helps nerve cells to communicate with each other. Antibodies produced against this protein prevent nerve cells from communicating with each other.
However, the occurrence of the disease as a result of these conditions has not been conclusively proven.
Risk Factors for Rigid Person Syndrome
There are thought to be some risk factors that may increase the risk of developing the disease. Risk factors include diseases that people have and some types of cancer.
The diseases that are among the risk factors for solid person syndrome are as follows:
- Diabetes (Diabetes)
- Thyroiditis
- Vitiligo
- Persinous anemia
Some types of cancer are also thought to cause this disease. The presence of one of the following types of cancer is thought to increase the risk of developing this syndrome. The cancer types are as follows:
- Lung cancer
- Kidney cancer
- Thyroid cancer
- Colon cancer
- Thyroid cancer
It also increases the risk of certain types of cancer, including Hodgkin's lymphoma cancer.
Who Has Rigid Person Syndrome?
It is a rare neurological disorder known as autoimmune movement disorder. It is twice as common in women as in men. However, this disease is a very rare condition today.
Symptoms of the disease, which has a one in a million chance of occurrence, can occur at any age. It usually occurs between the ages of 30 and 60.
How is Rigid Person Syndrome Diagnosed?
The symptoms of this syndrome, which is a movement disorder and a neurological disease, are similar to some diseases and disorders.
Similar symptoms can be seen in disorders such as tetanus, MS and muscular dystrophy. For this reason, a number of tests and examinations can be performed by specialist doctors to diagnose the disease and prevent it from being confused with different diseases.
If the doctor suspects the disease, the tests that should be done in terms of the diagnosis of solid person syndrome are as follows:
Blood Test
The blood test looks for antibodies to GAD or amphiphysis in the blood and other symptoms that can cause different diseases. Usually these patients have antibodies against GAD.
Electromyography (EMG)
It is performed to measure electrical activity in the muscles and to determine if there is any condition affecting the nerves.
Lumbar Puncture (Spinal Tap)
It is a procedure in which fluid is taken from the spinal canal in the brain. It is done to check for the presence of antibodies against GAD and symptoms that can trigger different diseases.
Solid Person Syndrome Treatment
It is a disease that can occur in a single part or area of the body, but can spread throughout the body over time. The treatment of rigid person syndrome depends on the condition and the symptoms.
There is no definitive treatment for the disease. Long-term treatment is needed to control the problems, symptoms and complaints experienced in the disease and to maintain the quality of life of the person.
If the doctor deems it necessary, certain medications may be prescribed to treat and alleviate muscle stiffness and spasms. These medications given to prevent seizures can reduce the complaints and pain.
In some cases, anti-inflammatory and anti-inflammatory drugs may be given to prevent severe pain.
Other treatments include intravenous immunoglobulin (IVIG), plasmapheresis, rituximab and autologous stem cell transplantation.
The specialist doctor works on the necessary treatment method to minimize the symptoms seen in patients.
Non-drug treatment methods include different options such as physical therapy, heat therapy, acupuncture and massage. These are usually given in combination with medication.