Syringomyelia

Syringomyelia is a chronic disease characterized by the formation of cavities in the dorsal and medulla oblongata with the development of extensive areas of pain and temperature sensitivity.

Etiology, pathogenesis. The causes of the disease are not clear. The process of growth of glial tissue with subsequent decay and cavity formation is localized mainly in the posterior horns of the cervical thickening of the spinal cord and spread to the brain stem.

Symptoms, course. The loss of pain and temperature sensitivity on large areas of the skin, most often on the hands and body ("jacket", "half jacket"), which causes numerous painless burns and injuries. Atrophy of the hands with reflexes, combined with spastic paresis of the legs and Horner's syndrome (ptosis, myosis, anhidrosis). This symptomatology is typical for the most common cervical disease. When the process spreads to the brain stem (syringobulbia), nystagmus, bulbar disorders (swallowing disorders, speech) and zones of dissociated anesthesia appear in the outer parts of the face. Less often, violations of sensitivity and atrophy occur in the lower parts of the trunk and legs. Significantly expressed trophic disorders-thickening and cyanosis of the skin on the hands, painless panarits with the mutation of terminal phalanges. Sometimes there are gross arthropathies (more often elbow and shoulder joints) with molten joint articular elements and their sequestration: the joint is sharply enlarged in volume, the motion is painless and accompanied by a kind of noise due to the fragments rubbing against one another (the neurodynamic joint of Charcot). The defeat of pyramidal beams in some cases, in addition to lower paraparesis, causes a disruption of the bladder function. As a rule, syringomyelia is accompanied by developmental anomalies (so-called dysraphic signs): kyphoscoliosis, inordinately long arms, polymathia, etc. The disease usually manifests itself in late childhood and lasts for many years. Progression is very slow, so patients are rarely immobilized. Cerebrospinal fluid is not changed.

Syringomyelic syndrome, indistinguishable sometimes from syringomyelia, is observed with bone anomalies of the craniovertebral transition, and also after the meningitis transferred.

Unlike syringomyelia, tumors of the spinal cord usually show more local and rapidly increasing symptoms. With amyotrophic lateral sclerosis, motor disorders are never accompanied by loss of sensitivity.

Treatment. If myelography reveals a blockade of the subarachnoid space, then a surgical emptying of the cyst is indicated. In the vast majority of cases, treatment is limited to massage and physiotherapy exercises. Patients are advised to be careful not to damage the skin. The effect of X-ray therapy of the affected area of ​​the spinal cord is questionable.

Forecast. The disease progresses slowly, but does not affect life expectancy. In far-advanced stages, a significant threat is a urological infection. With syringobulbia, breathing disorders are possible due to stridor caused by paralysis of the larynx.