Strympel's disease: symptoms and treatment

Struumpel's disease is a pathology characterized by the gradual development of weakness in the lower extremities, against the background of an increase in muscle tone in them. This is a hereditary disease that can be diagnosed absolutely at any age, beginning with early childhood and ending with deep old age.

General information

Stroumpel's disease is a chronic progressive pathology of the nervous system that belongs to the group of heterogeneous ailments. It is characterized by bilateral damage to the so-called pyramidal pathways in the anterior and lateral cords of the spinal cord. Dominant forms of ailment are formed during the first few years of a person's life, and recessive forms - at a later age and characterized by a heavy current.

This pathology was first described in detail by A. Strumpel in 1866. The specialist noted the family nature of the course of the disease. In medical literature, you can find other names of pathology (Strympel's paralysis, family spastic paraplegia).

The course of the disease is defined as slowly progressing. If it occurs at an early age, it is more malignant. In the case of late development, hypertension and hyperreflexia often prevail over various motor disorders. The prognosis, according to experts, is favorable for patients. The degree of loss of normal working capacity primarily depends on the severity of dysfunction of the central nervous system.

According to the International Classification, Strumentel's disease (ICD-10) is encoded under the cipher G11.4.

Main reasons

The exact causes of the development of this disease are currently unknown. Specialists in this field only express various hypotheses, but none of them has yet found scientific confirmation.

Heredity of the disease

This ailment can only be inherited. In total, experts identify several of its types:

• Autosomal dominant (in a married couple where one of the parents suffers from this pathology, the probability of having a child with such a diagnosis is 50 to 100%).
• Autosomal recessive (the probability of the birth of an unhealthy child in a married couple, where one of the parents is already sick, can be from 0 to 50%).
• Coupled with the X-chromosome (most often an ailment is diagnosed in males).

In medical practice, special cases are known (for example, the syndrome of Beer, which manifests itself atrophy of the optic nerve), when the symptoms of the disease very much resemble family spastic paraplegia, but are nevertheless independent diseases.

How is Stryumpel's disease manifested? Symptoms

The development of the disease and the manifestation of symptoms occur gradually. Patients initially complain of painful discomfort in the legs, a constant sense of stiffness and convulsive contractions. After a while bending of the legs in the knee joints becomes more difficult, then such pathological changes increase. Some can not tear the foot off the floor. Moreover, it is deformed (shortened and becomes slightly above the norm). In the late stages there are serious impairments of the functions of the pelvic organs in the form of constant urge to urinate.

Forms of the disease

Based on the features of the pathology, in medical practice its following forms are distinguished:

• Uncomplicated (in the clinical picture, weakness predominates in the muscles of the lower limbs without accompanying disorders).
• Complicated (except for the above signs in patients also diagnosed with heart disease, deformation of the foot , etc.).

Depending on the presence of confirmed cases, the ailment among close relatives is distinguished:

• Family illness Stryumpel (inherited from parents to children).
• Idiopathic variant of the disease (in this case the family history of pathology can not be detected).

Establishing diagnosis

When symptoms appear, it is very important not to engage in self-medication, but to seek help from a doctor. At the reception the specialist first collects the patient's anamnesis, listens to complaints and asks questions (when there was weakness in the legs, whether such relatives had similar pathologies, etc.).

The next stage is a neurological examination. It involves an assessment of the strength of the muscles in the hands and feet, the search for pathological reflexes. Additionally, computer and magnetic resonance imaging of the spinal cord can be assigned. Such a study allows for maximum detailed study of its structure and to identify possible thinning of pyramidal tracts. Note that in most cases, CT and MRI do not show any pathological changes.

How else can Striemple's disease be confirmed? Diagnosis of the disease also includes the study of sensory potentials. Such a method allows to estimate the degree of carrying out of sensitive impulses all over the spinal cord. A DNA analysis is mandatory for detecting the presence of a gene mutation.

Special attention should be paid to differential diagnosis, which in some cases causes difficulties. This disease is extremely important to differentiate with Mills syndrome - degenerative CNS lesion, characterized by the expansion of the cerebral ventricles and atrophy of the frontal and precentral convolutions. Clinically, this illness manifests itself slowly. In the last stages of the development of the disease, bilateral paralysis is observed.

Family spastic paraplegia is important to differentiate with the so-called spinal form of multiple sclerosis. Here it is necessary to take into account the state of abdominal reflexes, sphincters, the course of the entire disease. Particular attention in this case is given to the patient's anamnesis. Only after the delivery of all tests and tests can confirm the disease Stryumpel.

Treatment

The modern medicine can not offer effective methods of treatment of this disease. Therapy is reduced to reducing symptoms and improving the quality of life of the patient.

Patients are prescribed drugs for the gradual reduction of muscle tone ("Midokalm", "Baclofen", "Isoprotan"), tranquilizers ("Sibazon", "Nosepam", "Chlosepid"), vitamins of group B.

Patients with this diagnosis are shown physiotherapy procedures, coniferous baths, paraffin applications on the lower limbs. In addition, a positive result can be seen after reflexotherapy, acupressure and the course of therapeutic physical training.

Orthopedic therapy involves the use of special orthoses that help a person move around.

It is important to note that in each specific case, the course of therapy and associated procedures are assigned individually. The doctor should take into account not only the results of the examination, but also the general condition of the patient, the severity of clinical symptoms and the presence of other diseases. Priority is given to the selection of medicines and to apply them strictly in the prescribed dosage. Otherwise, the probability of an unfavorable forecast increases.

Stroumpel's disease is quite a complex pathology from a psychological point of view. Relatives and relatives should provide all-round moral assistance. The courses of the above-described symptomatic treatment are recommended to be held at least twice a year. With constant and adequate therapy, the progression of the disease can be slowed down. This means that a person can lead a habitual way of life and even work for a long time.

Complications and consequences

Strympel's disease is dangerous not only for its clinical picture. A person with such a diagnosis sooner or later loses the ability to fully move without help. In addition, the ailment leaves its imprint on labor adaptation due to a pronounced neurological defect in the form of weakness of the leg muscles. As a consequence, patients can not work, they gradually lose interest in life. Such people need help not only from a specialized specialist, but also from a psychologist.

Preventive measures

Modern medicine can not, unfortunately, offer adequate methods of preventing this disease, since its main causes are not known.

On the other hand, experts are sure that one should try to lead a correct lifestyle, eat well, give preference to healthy foods, and not smoke. Sports activities have a strong effect on the whole body. If close relatives have a history of Strympel disease, it is advisable to consult with the doctor on this matter and, if necessary, to undergo a full examination several times a year. This approach will not only allow timely detection of pathology, but also take all measures to reduce its progression.

Conclusion

In this article, we talked about what Stryumpel's disease is. Photos of patients with this diagnosis can be seen in specialized medical reference books. Undoubtedly, this pathology requires not only a qualified approach to treatment, but also a constant moral support from relatives.

Scientists from all over the world continue to actively study this disease, its main causes of development and adequate methods of treatment. Even today, there are speculations about a particular gene and its mutation, which directly affects the appearance of the disease.

We hope that all the information presented here is actually useful for you. Be healthy!