Siringomiyeliya (from Greek "syrinx" - an empty pipe and "myelon" - a spinal cord) - a chronic disease of the central nervous system at which in substance of a spinal cord, and sometimes and in a medulla cavities are formed. The true siringomiyeliya is connected with pathology of glial fabric, in other cases the disease is a consequence of anomalies of kranio-vertebralny transition. Application of MRT of a backbone considerably facilitated diagnostics of a siringomiyeliya. In cases of impossibility of carrying out MRT it is possible to visualize siringomiyelitichesky cavities by means of a miyelografiya. Siringomiyeliya is not curable, having begun at young age, she accompanies the patient all life. To patients symptomatic and neurotyre-tread therapy, in hard cases - surgical drainage of cavities is carried out.
Siringomiyeliya (from Greek "syrinx" - an empty pipe and "myelon" - a spinal cord) - a chronic disease of the central nervous system at which in substance of a spinal cord, and sometimes and in a medulla cavities are formed. Siringomiyeliya is not curable, having begun at young age, she accompanies the patient all life.
The true siringomiyeliya is caused by congenital defect of glial fabric at which there is an excess growth of cages of a glia in gray substance of chest and cervical departments of a spinal cord and in a brain trunk (siringobulbiya). Process of reproduction of glial cages can be started at traumatic damage or an infectious disease. Pathologically bred cages of a glia perish and in gray substance of a brain the cavities covered by glial cages are formed. These cages pass liquid which collects in a cavity and causes its gradual increase. At the same time there is a sdavleniye, a degeneration and death of adjacent nervous cages: motor and sensitive neurons. Steady progressing of a siringomiyeliya is caused by the continuous increase in the formed cavities which is followed by death of an increasing number of neurons. At a true siringomiyeliya congenital deformations and anomalies of development of a backbone often come to light: scoliosis, deformations of a thorax, the wrong bite, an asymmetric structure of a facial skull and other parts of a skeleton, the high sky, a dysplasia of auricles, additional nipples of mammary glands, the doubled language, a shestipalost, etc. The true siringomiyeliya has family character and occurs generally at men aged from 25 up to 40 years.
Approximately in 65% of cases of a siringomiyeliya it is not true, and is connected with anomalies of a junction of a skull and backbone (kranio-vertebralny joint). Similar anomalies of a structure lead to expansion of the spinal channel. Gray substance of those segments of a spinal cord which are at the level of considerable expansion of the spinal channel collapses that leads to emergence of a clinical picture, characteristic of a siringomiyeliya. Cavities similar by the fact that are formed at a true siringomiyeliya can be formed the died on the spot neurons as a result of a severe spinal injury, hemorrhage or a heart attack of a spinal cord.
In most cases siringomiyeliya of a cavity are formed in back horns of a spinal cord where there are sensitive neurons which are responsible for painful and temperature sensitivity. At the same time on skin big areas decide on loss of the corresponding types of sensitivity, most often they are located on the top extremities and a trunk and at unilateral defeat have an appearance of "semi-jacket", and at bilateral — "jackets". Because of violations of sensitivity at a siringomiyeliya patients are often injured and get burns that quite often is a reason for the first address to the doctor. But even before formation of obvious losses of sensitivity, the patient in these areas has sensitive violations in the form of pains and paresteziya (burning, crawling of goosebumps, etc.) that can also be a reason for visit of the doctor. The Propriotseptivny feeling and tactile sensitivity at a siringomiyeliya remain sokhranna. Long dull aches of the aching character in a neck, interscapular area, hands and a thorax are characteristic. Losses of sensitivity in the lower part of a trunk and legs meets quite seldom.
The expressed neurotrophic violations are characteristic of a siringomiyeliya: thickening of skin and its cyanosis, bad zazhivlyaemost even of insignificant wounds, deformations of joints and bones, osteoporosis. Patients with a siringomiyeliya often have characteristic changes of brushes: fingers are thickened, skin dry and rough, numerous hems from wounds and burns are visible, often there are suppurated wounds or fresh burns, felons are not rare. At defeat of side horns of verkhnegrudny segments of a spinal cord the heyromegaliya — the expressed brush thickening is observed. Trophic damages of joints (is more often humeral and elbow) at a siringomiyeliya are followed by fusion of their bone elements with formation of cavities. Sharp increase in the affected joint is characteristic, at the movement pain is absent and the noise caused by friction of bone fragments in a joint is heard.
When progressing a siringomiyeliya the cavities formed in a spinal cord increase and can take its forward horns that leads to decrease in muscular force, violation of movements and emergence of muscular atrophies. The so-called sluggish paresis of extremities which is characterized by decrease in a muscular tone, atrophies and decrease in deep reflexes develops. At a siringomiyeliya of cervical department of a spinal cord Horner's syndrome is noted (omission of a century, expansion of a pupil, zapadeny an eyeball). When involving in process of the motive carrying-out ways the lower paraparesis which sometimes is followed by violation of an urination is noted.
If at a siringomiyeliya the cavity is formed in a brain trunk (siringobulbiya), the disease begins with violation of sensitivity in external departments of the person. Later gradually violations of the speech, swallowing, breath (bulbarny paresis) accrue, atrophies of language, a soft palate and a half of the face develop. At a siringomiyeliya there can be an accession of a secondary infection to development of bronchial pneumonia or urological diseases (uretrit, pyelonephritis). Development of bulbarny paralysis at a siringobulbiya can lead to respiratory standstill and the death of the patient.
Diagnostics and treatment of a siringomiyeliya
Radiological methods of diagnostics can reveal trophic manifestations of a siringomiyeliya in the form of osteoporosis, destructions of bone elements of a joint, etc. Precisely allows to diagnose a siringomiyeliya backbone MRT, and at impossibility of its carrying out - a miyelografiya. These methods of inspection give the chance to see siringomiyelichesky cavities in a spinal cord and a trunk.
In an initial stage of a siringomiyeliya when there is an active reproduction of a glia, apply the treatment methods directed to suppression of this process. Such treatment allows to stop progressing of a disease and to reduce its manifestations. For this purpose apply X-ray therapy — radiation of the struck segments of a spinal cord and treatment by radioactive iodine or phosphorus which have property to collect in quickly breeding cages of a glia and to irradiate them "from within". Before treatment of a siringomiyeliya radioactive iodine to the patient appoint solution of Lugol from which iodine fills cells of a thyroid gland and by that protects them from penetration of radioactive iodine.
Medicamentous therapy of a siringomiyeliya is carried out by the neurologist and the bendazola consists in application of dehydrating means (acetazoleamide, furosemide), vitamins, neuroprotectors (glyutaminovy acid, piracetam). For knocking over of a pain syndrome at a siringomiyeliya analgetics (metamizol, aminophenazone) and ganglioblokator are shown ().
Rather new method in treatment of a siringomiyeliya is therapy neostigminy, improving carrying out nervous impulses. But it is not directed to a cause of illness, and only allows to improve neuromuscular carrying out temporarily. The combination of such therapy to UVCh or radonic bathtubs is possible.
Surgical treatment of a siringomiyeliya is discussed at the big and increasing neurologic deficiency in the form of the central paresis of legs and peripheral paresis of hands. Backbone operations are made for drainage of siringomiyelichesky cavities, a decompression of a spinal cord, removal of solderings.
Forecast and prevention of a siringomiyeliya
As a rule, the true siringomiyeliya is characterized by slowly progressing current and does not reduce life expectancy. Patients keep working capacity the long period of time. However at emergence of infectious complications development of sepsis is possible. Hard the siringobulbiya as involvement in process of the respiratory center and the wandering nerve lead to a lethal outcome proceeds.
Methods of primary prevention of a siringomiyeliya do not exist yet. Secondary prevention is directed to prevention of progressing of a disease, the prevention of infections, injuries and burns.