Help the development of the site, sharing the article with friends!

Rigid human syndrome, or generalized rigidity syndrome, is a disease of the nervous system characterized by constant muscle stiffness. Additionally, muscle cramps may occur, especially when influenced by various stimuli, e.g. noise. What are the causes and other symptoms of the rigid human syndrome? How is the treatment going?

Stiff man syndromeotherwisegeneralized stiffness syndromeor Moersch-Woltmann syndrome (SMS - Stiff man syndrome or SPS - stiff person syndrome is a neurological disease characterized by constant stiffness and sometimes painful muscle spasms, often triggered by various stimuli.

Rigid human syndrome - causes

Rigid human syndrome can be an autoimmune disease. Then, for unknown reasons, the immune system produces antibodies against glutamic acid decarboxylase (anti-GAD). GDA is an enzyme that is involved in the production of gamma-aminobutyric acid (GABA), responsible for maintaining proper muscle tone.

Rigid human syndrome is often associated with other autoimmune diseases such as diabetes, thyroiditis, vitiligo and pernicious anemia.

Rigid human syndrome does not belong to neurological paraneoplastic syndromes, but it may be neoplastic, i.e. the presence of neoplasm may contribute to its development. Generalized stiffness syndrome has been most frequently described in patients with breast cancer, small cell lung cancer and ovarian cancer. In the course of cancer, the immune system produces antibodies to destroy cancer cells, but these also mistakenly attack the structures of the nervous system, leading to the development of SMS. They are the so-called onconeural antibodies.

Rigid human syndrome - symptoms

  • severe, progressive muscle stiffness - especially in the spine (especially in the lower back or around the nape of the neck) and lower limbs. This stiffness may come and go at first, but then it persists and worsens. As a result, there are difficulties with moving (walking is slow, stiff), falls often occur
  • hypersensitivity to stimuli such as noise, touch, and emotional stress that can lead tomuscle cramps. Then the patient may avoid leaving the house for fear of these stimuli and possible contractions
  • incorrect body posture - the figure is often hunched and stiff

GOOD TO KNOW>>MUSCLE CONTRACTS - what are the proof of muscle spasms?

Rigid human syndrome - diagnosis

If rigid human syndrome is suspected, blood tests are performed to detect the presence of autoantibodies. The presence of anti-amphiphysin antibodies (a type of onconeuronal antibodies) indicates the cancerous basis of the disease. On the other hand, the presence of anti-GAD autoantibodies indicates a non-cancerous basis of the disease.

Electromyography (EMG) is also helpful, during which a constant, very rich bioelectric activity is observed.

Rigid man syndrome - treatment

If the cause of the disease is cancer, the first thing to do is to heal it.

In addition, the patient is given benzodiazepines. These are GABA-like drugs that relax the muscles, have anticonvulsant properties and have a calming effect.

Immunomodulating therapy is also used - the patient is administered intravenous immunoglobulins (IVIG). The doctor may also decide to perform plasmapheresis - a procedure to cleanse the blood of unwanted antibodies.

Help the development of the site, sharing the article with friends!

Category:

Neurology