Rigid Man Syndrome

There are a number of diseases in medicine that have been very little studied so far, and therefore cause significant difficulties both in terms of diagnosis and treatment. Such pathologies include Mersch-Woltman syndrome, or the so-called rigid person syndrome. The disease is characterized by gradually increasing muscle stiffness, equally spreading through the system of muscles of the arms, legs, and torso. This condition worsens over time, which sooner or later leads to systemic muscle rigidity.

Epidemiology

Since the syndrome is a disease with extremely difficult diagnosis, it is not possible to trace its spread in the world or in a particular country.

It has been noted that the rigid person syndrome begins to manifest itself in patients aged 30-40 years and older. Sick children and teenagers also occur, but much less frequently – such cases can be called sporadic.

Rigid person syndrome affects people of both genders.

The disease is not contagious. Most scientists are inclined to believe that the pathology has an autoimmune origin.

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Risk factors

Since the causes of rigid person syndrome have been studied very little, the following possible risk factors for the disease can theoretically be identified:

• osteochondrosis of the spinal column;
• neck and head injuries;
• cerebral hemorrhages, inflammatory processes (meningitis, encephalitis);
• Parkinson's disease.

An additional unfavorable factor may also be a failure in the functioning of the adrenal glands. However, there is no clear evidence of one or another theory of the origin of the disease.

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Pathogenesis

The pathogenesis of the disease has not yet been fully elucidated. It can be assumed that the cause of the syndrome is a disruption of the central nervous system: this is indicated by such signs as an absolute improvement in the condition of the muscular system during sleep, after artificial blockade of peripheral nerves, during general anesthesia and when taking diazepam. There are many reasons to think that the main provoking moment in the development of the rigid person syndrome is increased excitation of α-motor neurons, the causes of which are also still unknown.

Information obtained during physiological testing allows us to substantiate this problem as a consequence of impaired function of the spinal cord systems that control the performance of the spinal cord nerve cells.

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Symptoms of rigid-man syndrome

The first signs of the disease often go unnoticed or are ignored. At first, the patient may experience unobtrusive, infrequent pain and muscle discomfort in the back, abdominal press and cervical spine. However, over time, the discomfort increases, becomes permanent, and muscle twitching appears. Within a few months, the symmetrical muscles of the arms and legs are also involved in the process.

Rigid man syndrome is characterized by the dominance of hypertonicity in the extensor muscles. Due to constant muscle tension, curvature of the spine (usually lordosis) may develop. The thoracic region is usually straight, the head may be thrown back, the shoulders are turned upward. Sometimes cervicothoracic kyphosis can be observed.

The abdominal muscles are in a tense state (the “board” symptom).

Along with constant muscle tension, short-term spastic muscle contractions can be observed. Such spasms can occur in response to fear, touch, loud sound, temperature exposure, etc. If the irritant is applied repeatedly, the muscle "response" becomes less pronounced.

Spastic contractions also affect the muscles of the legs and back, spreading to the respiratory muscles, which affects the frequency and rhythm of respiratory movements. Laryngeal stenosis, obstructive stenosis of the esophagus, and dysphagia may develop.

The severity of spasms can vary, from minor to severe, which can even provoke a dislocation or fracture. Often the patient screams or falls on level ground due to a sharp muscle spasm. This condition can be combined with anxiety, severe sweating, rapid heartbeat, hypertension.

Lesions of the facial muscles and muscles of the hands and legs are rarely observed.

Due to the constant spastic condition, the ability to move in the lower back and hip joint is limited. It becomes difficult for the patient to stand up, sit down, and even turn on his side. If the neck muscles are affected, the patient is unable to move his head to one side or the other.

At an advanced stage of rigidity syndrome, as a result of muscle strain, a pathological position of the limbs is formed. The patient often cannot move independently, and falls without support.

Forms

There are several types of rigid person syndrome, or, as it is also called, spinal column rigidity syndrome. Thus, in neurology, the syndrome has a number of degrees:

• mild, smoothed degree (slight change in the curves of the back);
• moderate degree (straightened back and “board” symptom of abdominal muscles);
• severe degree of the syndrome (secondary disorders of the musculoskeletal system and joints);
• severe degree (changes in the functionality of internal organs).

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Complications and consequences

In the vast majority of cases, rigid person syndrome tends to gradually progress, although properly prescribed treatment can stabilize the patient's condition for a long time. Rarely, there are periodic improvements in the condition, which can last for months or even years. But most often, the condition steadily worsens and after some time the patient can no longer get out of bed.

In turn, a constant lying position in combination with damage to the respiratory muscles leads to the development of an inflammatory process in the lungs, which most often becomes the cause of the patient's death.

In addition, there are data on cases of sudden death, which may be a consequence of significant vegetative disorders.

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Diagnostics of rigid-man syndrome

Diagnosis of the disease is carried out taking into account all detected symptoms, complaints and research results.

The most important stage for making a correct diagnosis is considered to be instrumental diagnostics, and, in particular, electromyography. This type of study helps to detect the presence of constant hidden motor activity even when trying to relax the muscles. Under the influence of sensory stimuli (sharp sound, electrical stimulus, etc.), electromyographic activity increases.

The disappearance of muscle tension is observed during sleep (especially during the so-called "fast" sleep phase). The same effect can be observed after an intravenous injection of diazepam or muscle relaxants, or during general anesthesia.

Conventional tests cannot detect rigid person syndrome, but they can help identify associated diseases, which can make diagnosis somewhat easier. For example, a general blood test can detect anemia and inflammatory processes in the body.

Possible appointment:

• general urine analysis;
• blood electrolytic analysis;
• Hormonal level tests (thyroid hormones).

Muscle tissue biopsy is performed to detect non-specific changes such as fibrosis, atrophy, degenerative disorders, edema, and muscle fiber ischemia.

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Differential diagnosis

Differential diagnostics may be necessary to distinguish rigid person syndrome from Isaac's syndrome. In the latter, myokymia is detected on electromyography. In some cases, it is necessary to exclude spasms in pyramidal syndrome (high reflexes are absent in RPS) and muscle tension during supranuclear palsy (this disease has much in common with parkinsonism and dystonia).

In severe spastic conditions, differentiation should be made with tetanus, which is characterized by the phenomenon of trismus.

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Treatment of rigid-man syndrome

The syndrome is treated with medication. As a rule, all treatment procedures are aimed at improving the patient's condition and prolonging his life.

The main therapeutic approach is carried out using GABA agents. Such drugs inhibit the increased activity of spinal motor neurons and reduce muscle spasms.

	Clonazepam	Diazepam
Method of administration and dosage	The average dose of tablets is 4 to 8 mg per day.	The tablets are taken on average 2.5-10 mg, up to 4 times a day.
Contraindications	Respiratory failure, glaucoma, severe depressive state.	Epilepsy, glaucoma, sleep apnea.
Side effects	Lethargy, fatigue, muscle weakness, dyspepsia.	Drowsiness, apathy, headache, dizziness.

Treatment with such drugs begins with a minimum dosage, after which the amount of medication is gradually increased to the optimal effective level.

If the above-mentioned drugs do not improve the dynamics of the syndrome, then they move on to the use of Baclofen.

• Baclofen is prescribed with a gradual increase in dosage, bringing it to 100-120 mg per day (morning, afternoon and night). One of the common side effects when taking Baclofen is considered to be lethargy and drowsiness.

A qualitative improvement in the condition of patients can be achieved with the combined use of Baclofen and Diazepam, with the prescription of minimal quantities of drugs to reduce side effects.

Corticosteroids may also be used for treatment:

• Methylprednisolone 500 mg intravenously, with a slow reduction in dosage to 5 mg orally every other day. The drug is not prescribed for persistent hypertension, nephritis. In diabetes mellitus, special caution is required when using the drug.

With long-term use of Methylprednisolone, obesity, hair growth, and osteoporosis may develop.

A good effect is observed when prescribing injections of immunoglobulin - a safe drug with a minimum number of side effects (rarely - headache, fever, nausea, allergies).

If all the above schemes are considered ineffective, then they switch to long-term use of cytostatic agents, such as Cyclophosphamide or Azathioprine. The scheme for using cytostatics is prescribed strictly individually.

Prevention

Without knowing the true causes of the disease, it is very difficult to determine preventive measures. However, if we assume the autoimmune nature of the disease, then any person can undergo an examination to detect antinuclear antibodies. Such antibodies initiate the development of the disease. Thus, it is possible to find out whether there is a risk of developing an autoimmune process.

Otherwise, you should follow general recommendations for disease prevention:

• rational nutrition;
• support for normal bowel function;
• sufficient amount of vitamins and microelements in food, as well as vitamin D;
• counteracting stressful situations;
• absence of bad habits.

A healthy and clean organism can often overcome many factors, from the adverse effects of external factors to hereditary predisposition. This allows you to minimize the risk of developing autoimmune processes.

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Forecast

The prognosis of the disease may largely depend on the presence of background diseases. For example, a very common concomitant pathology is insulin-independent diabetes mellitus. In this case, the death of patients from hypoglycemic coma is not uncommon.

Also, patients with rigid person syndrome often have pathologies of the endocrine system (in particular, the thyroid gland), anemia, retinopathy, skin diseases, etc. The listed diseases are usually associated with autoimmune reactions.

It is assumed that in people who have the syndrome, the autoimmune process is redirected against the central nervous system and some organ systems. There are descriptions of cases of the disease that are associated with oncological changes in the body.

Taking into account all of the above, the prognosis for such a disease as rigid person syndrome cannot be considered favorable. At least, at present, medicine has no way to restore an organism affected by autoimmune processes.

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