This disease opened quite long time. The first formation of bones was described by scientists Pikolomini and Etienne. They presented the results of researches in the 16th century. However, to the description of a disease it was still far. The following step to discovery of this pathology was made at the end of the 17th century by the scientist Bryunner who found in the newborn child with splittings in a backbone unclear cavities which lasted almost along all spinal cord. Finally the scientist Olivier Ange who could prove managed to find out everything only in the 19th century that process of education in a spinal cord of cavities represents separate pathology which received the name "siringomiyeliya". This term was formed from two Greek words, such as: syrinx - "emptiness" and myelos - "spinal cord".
This quite rare disease. After all it meets at 10 on 100 thousand people. And on supervision most often it men at the age of 30-35 years are ill. Women are ill less often. In rare instances the disease can develop at advanced age or in the childhood. Dependence of a disease on race or the place of residence was not noted, but cases when the disease came to light at all family members in practice met.
The disease first imperceptibly therefore the person can even not guess it develops. Unfortunately, this pathology is considered incurable, but sometimes it can pass into a remission stage nevertheless. The modern medicine in the presence of this disease allows to achieve more long full-fledged life. Thus it is necessary to begin treatment as it is possible earlier.
Siringomiyeliya usually extends only on department of a backbone. In rare instances pathology touches a medulla. Expansion of the spinal channel is the reason of formation of similar cavities. Thus liquid which on it flows, encounters certain obstacles that compels to bend around these obstacles for further course. As a result on places of these rounds cavities are gradually formed. Together with it some cages which do not take part in transfer of nervous impulses start developing. These all changes cause violation of full process of a momentum transfer from a brain in various bodies and back.
Unfortunately, the real reasons for which in an organism occur similar change still remain undecided. Siringomiyeliya can be congenital or acquired. The congenital type of a disease usually develops because of the available pathologies in an embryo, for example, when the spinal cord is incorrectly stuffed up.
Congenital pathology of certain brain structures can also become the reason of emergence of this disease. One of such pathologies is the syndrome of the Dandy Walker and Arnold-Kiari's anomaly. At congenital type of a siringomiyeliya, a cavity are usually formed in the top site of chest department. Sometimes formation of cavities is observed in the lower part of cervical department of a backbone. As for acquired like a disease, it quite often is result of an injury of a spinal cord or existence of a tumor in it.
The most frequent signs of a siringomiyeliya are sensitivity violations. Such violations are available for all patients. These symptoms are shown both in the form of irritation symptoms, and in the form of loss symptoms. At most of patients the top extremity pains, in a thorax and in cervicobrachial area are also noted. Pain usually amplifies under the influence of physical activity and overcooling, and decreases at rest.
Approximately at a half of patients paresteziya which are shown by feeling of a sleep, passing of electric current, crawling of goosebumps, and also cold sense and burnings are noted. Usually patients ask for the help when they have pains and a paresteziya. When carrying out inspection, violation of painful and temperature sensitivity is diagnosed for the patient. Thus at the patient tactile deep-muscular and kinestetichesky sensitivity usually remains. Points to violation of sensitivity that for the patient the got scratches, grazes and bruises of hands are painless. Disorders of sensitivity can be various degree of expressiveness and localization, but most often such frustration are noted on hands and a trunk in the form of so-called "semi-jacket" and "jacket". One patient can have two-three centers of the sensitive frustration having different degree of depth. The dissociated type of violation of sensitivity is usually connected with defeat of cages of forward white soldering or back horns of a spinal cord. If cavities appear in top cervical department of a backbone, violation of sensitivity on a face in zones of a zelder is observed. Violation of deep-muscular sensitivity usually happens only at patients whom process proceeds already for a long time.
Besides sensitive violations, at patients motive frustration are quite often shown. It should be noted that extent of similar violations is not so considerable as sensitivity violation. And these violations usually appear only at later stages of a disease. Because a place of formation of pathological processes most often is the cervicothoracic department of a spinal cord, the top extremities usually suffer much more often than the lower. Thus patients complain that in hands muscular force decreased. When carrying out diagnostics such pathologies are found in the patient as a hypotrophy of muscles, sluggish paresis of hands, hypotonia, loss and decrease tendinous nadkostnichnykh reflexes, and also reduction of separate muscle fibers. Squeezing or depravity of forward horns of a spinal cord is the reason of sluggish cuts.
At later stages of a disease, because of impact of processes of damages of neurons on the pyramidal ways at patients conduction symptoms start developing. These symptoms are shown in decrease in muscular force of feet, increase of a muscular tone. Also increase of segmentary reflexes and Oppengeym, Babinsky, Rossolimo's pathological signs and others is established. In certain cases on one extremity symptoms of at the same time spastic and sluggish paresis can be shown. Thus functions of bodies of a basin usually do not suffer.
Some experts in various areas of medicine are necessary for carrying out diagnostics and treatment of this disease. In particular, neurologists, neurosurgeons, radiologists and orthopedists are necessary. Treatment is usually carried out by a surgical way and its main direction stay of progressing of the available injuries of a spinal cord, and also preservation of its necessary functions is. Operation on a spinal cord thus is usually performed by means of its compression. In addition during operation manipulations necessary to make correction of deformation or for imposing of necessary shunts are carried out. For closing of cystous educations implantations of fabrics of a fruit are carried out.
Thanks to surgical intervention it is possible to stabilize or at least to improve symptoms at patients. Many experts incline to that it is necessary to carry out surgical treatment only when at the patient the progressing neurologic symptomatology is observed. In too time delay with operation when progressing a disease can lead to irreversible injuries of a spinal cord. Besides, it can become the reason of development of permanent neurologic violations.
As for drug treatment, it is quite often appointed for the purpose of reduction of hypostasis round a spinal cord. The patient for prevention of increase in pressure upon the damaged departments vigorous physical activity is recommended to avoid. If not to carry out treatment at the progressing symptoms, the patient can live of half a year about one year.