The syndrome of a rigid person

In medicine, there are a number of diseases that are still very little studied, and therefore cause considerable difficulties, both in terms of diagnosis and treatment. Such pathologies include the Mersha-Voltman syndrome, or the so-called rigid person syndrome. For the disease is characterized by a gradually increasing stiffness of the muscles, equally spreading through the system of muscles of the hands, legs, trunk. This condition is worsened 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 a single country.

It is noticed that the syndrome of the rigid person begins to manifest itself in patients from 30-40 years old and older. Sick children and adolescents also occur, but much less often - such cases can be called sporadic.

The syndrome of a rigid person affects people regardless of gender.

The disease is not contagious. Most scientists tend to the autoimmune origin of the pathology.

[1], [2], [3], [4], [5]

Risk factors

Since the causes of the syndrome of rigid man have been studied extremely little, the following possible risk factors for the disease can be theoretically determined:

• osteochondrosis of the spine;
• trauma to the neck and head;
• cerebral hemorrhage, inflammatory processes (meningitis, encephalitis);
• Parkinson's disease.

An additional adverse factor may be a malfunction in the adrenal glands. However, there is no clear evidence of any theory of the origin of the disease.

[6], [7], [8]

Pathogenesis

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

The information obtained during the physiological tests allows us to justify this problem as a result of the impaired function of the stem-spinal systems that control the performance of the nerve cells of the spinal cord.

[9], [10], [11]

Symptoms of the syndrome of a rigid person

The first signs of the disease often go unnoticed, or ignored. Initially, the patient may experience unobtrusive, infrequent pain and muscle discomfort in the back, abdominal and cervical spine. However, over time, discomfort increases, acquires a permanent character, there are muscle twitchings. Within a few months, the symmetrical muscles of the arms and legs are involved in the process.

The syndrome of a rigid person is characterized by the dominance of hypertonia in the extensor muscles. Because of the constant muscle tension, the curvature of the spine can develop (usually lordosis). The thoracic region is usually straight, the head can be thrown back, the shoulders turned upward. Sometimes it is possible to observe cervicothorax kyphosis.

The muscles of the abdominal press are in a stressed state (a symptom of the "board").

Simultaneously with constant muscular tension, one can notice short-term spastic contractions of muscles. Such spasms can arise in response to fright, touch, sharp sound, temperature influence, etc. If you apply the stimulus repeatedly, the muscle "response" becomes less pronounced.

Spastic contractions also affect the muscles of the legs and back, extending to the respiratory musculature, which affects the frequency and rhythm of the respiratory movements. Possible development of stenosis of the larynx, obstructive narrowing of the esophagus, dysphagia.

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

Rarely is the damage to facial muscles and muscles of the hands and legs.

Because of the constant spasticity, the possibility of movements in the lower back and hip joint is limited. The patient becomes difficult to get up, sit down and even turn on his side. With the defeat of the neck muscles, the patient is unable to move his head in one direction or the other.

At the advanced stage of a syndrome of rigidity as a result of an overstrain of a musculature the pathological position of extremities is formed. The patient often can not move independently, in the absence of support falls.

Forms

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

• easy smoothed degree (slight change in the bends of the back);
• middle degree (straightened back and symptom of the "board" of abdominal muscles);
• expressed degree of the syndrome (secondary disorders of the musculoskeletal system and joints);
• a severe degree (changes in the functionality of internal organs).

[12], [13], [14]

Complications and consequences

In the overwhelming majority of cases, the syndrome of rigid person has a tendency to gradual progression, although correctly prescribed treatment can permanently stabilize the patient's condition. Periodic relief of the condition is rare, which can last for months and even years. But most often the condition is steadily deteriorating and after a while the patient can no longer get out of bed.

In turn, the constant recumbency in combination with the defeat of the respiratory muscles leads to the development of an inflammatory process in the lungs, which most often causes the death of the patient.

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

[15]

Diagnostics of the syndrome of a rigid person

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

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

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

Conventional tests can not detect the syndrome of a rigid person, but help to identify concomitant diseases, which can somewhat ease the diagnosis. For example, a general blood test will determine anemia, inflammatory processes in the body.

Assignment is possible:

• general analysis of urine;
• analysis of electrolytic blood composition;
• hormone level studies (thyroid hormones).

A biopsy of the muscle tissue is performed to identify such nonspecific changes as fibrosis, atrophy, degenerative disorders, puffiness, ischemia of muscle fibers.

[16],

Differential diagnosis

Differential diagnosis may be needed to distinguish the syndrome of a rigid person from the syndrome of Isaac. At the last, electromyography reveals myochemia. In some cases, exclusion of spasms in the pyramid syndrome is required (with high-refractive stresses absent) and muscle tension during supranuclear palsy (this disease has much in common with Parkinsonism and dystonia).

In severe spastic conditions, it is necessary to differentiate with tetanus, for which the phenomenon of trism is characteristic.

[17]

Treatment of the syndrome of a rigid person

Treatment of the syndrome is medicated. As a rule, all treatment procedures are aimed at improving the patient's condition and prolonging his life.

The main therapeutic approach is implemented using GABA funds. Such drugs inhibit the increased activity of spinal motoneurons and reduce muscle spasms.

	Clonazepam	Diazepam
Dosing and Administration	The average dose of tablets is 4 to 8 mg per day.	Tablets take an average of 2.5-10 mg, up to 4 times a day.
Contraindications	Respiratory failure, glaucoma, severe depressive condition.	Epilepsy, glaucoma, nocturnal apnea.
Side effects	Drowsiness, fatigue, muscle weakness, indigestion.	Drowsiness, apathy, pain in the head, dizziness.

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

If the above drugs did not improve the dynamics of the syndrome, then proceed to the application 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 inhibition and drowsiness.

A qualitative improvement in the state of patients can be achieved with the combined use of baclofen and diazepam, with the appointment of minimal quantities of drugs to reduce adverse events.

Also for treatment can be used corticosteroids:

• Methylprednisolone 500 mg intravenously, with a slow decrease in dosage to 5 mg orally 24 hours a day. The drug is not prescribed for persistent hypertension, nephritis. With diabetes, special care is required when using the medication.

With prolonged use of Methylprednisolone, obesity, hair, and osteoporosis may develop.

There is a good effect in the appointment of injections of immunoglobulin - a safe drug with a minimum of side effects (rarely - headache, fever, nausea, allergies).

If all of the above schemes were found to be ineffective, then proceed to the continued use of cytostatic agents, such as cyclophosphamide or Azathioprine. The scheme of using cytostatics is prescribed strictly individually.

Prevention

Not 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 a test for the detection of antinuclear antibodies. Such antibodies give rise to the development of the disease. Thus, it is possible to find out whether there is a risk of getting an autoimmune process.

In the rest, one should adhere to the general recommendations for the prevention of diseases:

• balanced diet;
• support the normal operation of the intestine;
• a sufficient number of vitamins and trace elements in food, as well as vitamin D;
• counteraction to stressful situations;
• absence of bad habits.

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

[18], [19], [20], [21], [22]

Forecast

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

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

It is assumed that in individuals who have a syndrome, the autoimmune process is redirected against the central nervous system and some organ systems. There are descriptions of cases of the disease, which are related to oncological changes in the body.

If you take into account all of the above, then the prognosis of such a disease as the syndrome of a rigid person, can not be considered favorable. At least, at present medicine can not restore the body affected by autoimmune processes.

[23], [24]