A stiff person syndrome (SPR) It is a rare condition that is associated with stiffness and muscle spasms. The syndrome, which affects the nervous system, can present itself in different ways and, so far, there is no cure. The disease is considered to be autoimmune, although knowledge about the etiological mechanism requires further investigation. In general, other autoimmune diseases may be associated. The treatment consists of the use of medication and also physiotherapy and aims to improve the patient's quality of life.
Read too:Rheumatoid arthritis — an autoimmune disease that causes joint inflammation
Topics of this article
- 1 - Summary about the rigid person syndrome
- 2 - What is the rigid person syndrome?
- 3 - Symptoms of rigid person syndrome
- 4 - Diagnosis of the Rigid Person Syndrome
- 5 - Treatment of rigid person syndrome
Stiff person syndrome summary
Stiff person syndrome is an autoimmune problem that affects the nervous system.
It is estimated that the syndrome affects one individual in every million people.
The most common symptoms of the syndrome include muscle stiffness and spasms.
Symptoms can be triggered by stress and other stimuli, such as mechanical and auditory.
The treatment of the syndrome is not aimed at curing, but at controlling the symptoms and, consequently, improving the individual's quality of life.
What is rigid person syndrome?
Stiff-Person syndrome (SPR), also known as Stiff-Person syndrome and Moersch-Woltman syndrome, is a problem rare health that affects the central nervous system, more precisely the spinal cord. It is estimated that the prevalence of this pathology is 1/1,000,000, with the most affected age group being between 35 and 50 years. In general, other autoimmune diseases may be associated, such as thyroiditis and diabetes mellitus.
the syndrome was first described in 1956 by Moersch and Woltman, being defined as a condition characterized by persistent spasms involving several different muscles, especially those of the lower limbs and trunk. Initially, the syndrome was known as rigid man syndrome, however, it was later realized that this was not the best definition, since women are also affected. Thus, the syndrome came to be called rigid person syndrome.
The syndrome is believed to have an autoimmune component, but the etiology (science and study of the causes that provoke a disease) still remains uncertain. In about 60% to 80% of cases, the presence of autoantibodies against glutamic acid decarboxylase (anti-GAD) is verified. GAD is a enzyme necessary for the synthesis of GABA, an important inhibitory neurotransmitter that is present in the central nervous system.
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Symptoms of Stiff Person Syndrome
The rigid person syndrome stands out for causing progressive muscle stiffness and trigger muscle spasms. This characteristic clinical picture can occur spontaneously or be triggered by some stimulus, such as sudden sounds, touch or even emotional stress.
You muscle spasms are episodic, very painful and can even result in a fall. Regarding muscle stiffness, it usually starts in the thoracolumbar paraspinal musculature. Posteriorly, muscle tightness extends to the proximal region of the legs and abdominal musculature. By affecting the legs and abdominal muscles, the individual adopts a rigid and robotic gait.
It is worth mentioning that the accentuated rigidity of the muscles can also be responsible for causing fiber rupture, dislocations and even resulting in bone fractures. In addition, stiffness in the back muscles can cause back pain, hyperlordosis, and difficulty moving and balancing. In some cases, the person may even present loss of voice and subjective feeling of shortness of breath.
It is important to highlight that this syndrome can manifestif in six different ways, being them:
Classic form, which is characterized by affecting only the lumbar region and the legs.
Variant form, in which only one limb is affected with dystonic posture (fixed postural changes or lasting for many minutes).
Rare form, which is characterized by a rigidity that affects the whole body due to severe autoimmune encephalomyelitis.
Form in which there is functional movement disorder.
Form with dystonia and generalized parkinsonism.
Form with hereditary spastic paresthesia (group of hereditary diseases that causes gradual weakness with muscle spasms in the legs).
Read too: Lupus — an autoimmune disease that can affect the skin and other organs
Diagnosing Stiff Person Syndrome
The diagnosis of rigid person syndrome is made based on the analysis of symptoms presented by the patient and also complementary exams, such as magnetic resonance imaging, electroneuromyography and detection of antibody anti-glutamic acid decarboxylase (Anti-GAD).
Rigid person syndrome treatment
The rigid person syndrome is a disease that has no cure. However, with treatment, it is possible to improve the person's quality of life. The drugs used in the treatment act by increasing the action of GABA and immunosuppressants are also recommended.
It is worth noting that the physiotherapy can be very beneficial in people with the syndrome, helping mainly with issues such as imbalances and functional limitations.
By Vanessa Sardinha dos Santos
Biology teacher
Would you like to reference this text in a school or academic work? Look:
SANTOS, Vanessa Sardinha dos. "Stiff person syndrome"; Brazil School. Available in: https://brasilescola.uol.com.br/saude/sindrome-da-pessoa-rigida.htm. Accessed on July 6, 2023.
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