Myelosyringosis

Description:

Myelosyringosis (Greek syrinx - reed, an empty pipe; myelon - a spinal cord) - the chronic dizembriogenetichesky disease which is characterized by emergence of cavities in gray matter of cervical or chest segments of a spinal cord and is frequent - in a myelencephalon (syringobulbia).

The disease usually begins to be shown at youthful age and proceeds for the rest of the life an individual.

Frequency: 8-9 on 100 000 population, is more often at men.

Myelosyringosis symptoms:

Most often cavities form in the field of back horns of a spinal cord with impact on sensitive neurons. Patients feel the persistent dull aching aches in the corresponding dermatomas and sclerotomes (in a neck, a shoulder girdle, an upper extremity, between shovels, in a thorax). Quite often a reason for the address to the doctor are painless burns of skin of an upper extremity or trunk. When progressing a disease atrophic paresis of the corresponding myotomes and neurotrophic frustration with osteoporosis and deformation of bones and joints develop (humeral, elbow, interphalanx on a brush). In the neurologic status the segmented dissociated anesthesia with loss painful and a thermoesthesia, safety of tactile and proprioceptive feeling comes to light. Often the zone of the broken sensitivity has an appearance of a symmetric or asymmetric jacket (semi-jacket) and takes a shoulder girdle and an upper back. The deep reflexes which are becoming isolated at the level of the struck segments of a spinal cord die away. However an animal force it is long remains. Only when the cavity takes also a front horn of a spinal cord, sluggish paresis and atrophies of muscles develop. In the lower extremities the hyperreflexia and other signs of defeat of pyramidal system, including Babinsky's symptom usually is defined. The pyramidal system partially suffers at the level of the available cavity in a back horn of a spinal cord or in tsentromedullyarny area at sharp expansion of the central spinal channel. Segmented anesthesia in such cases happens symmetric and bilateral (function of conductors of front commissure where axons of sensory cells of back horns cross is broken. The massive thickening of a brush - a heyromegaliya sometimes develops that is connected with defeat of vegetative cells of a side horn of upper chest segments. Skin is dry, its smoothness disappears. Atrophies extend to long muscles of a back that is shown by scoliosis or a kyphoscoliosis.

When forming a cavity in a myelencephalon painful also the thermoesthesia in a trifacial kernel zone is broken in the beginning (segmented anesthesia in Zelder's dermatomas), then symptoms of bulbar paresis with an atrophy of muscles of language, a soft palate and even one half of the face gradually join.

Myelosyringosis reasons:

Is inborn defect of development of a glia which patholologically breeds (gliomatosis) with the subsequent its disintegration and formation of a cavity in gray matter of segments of a spinal cord. The cells of a glia remaining on the periphery of such cavity setsernirut liquid which gradually leads to expansion of this cavity and degenerative destruction of neurons, adjacent to this cavity (sensitive neurons of a back horn, motor-neurons of a front horn of a spinal cord). The true myelosyringosis is quite often combined with other dizrafichesky changes of a skeleton and soft tissues: scoliosis and a smoothness of a chest kyphosis of a backbone, the high sky, the wrong ranks of teeth, a dysplastic structure of a facial skull, auricles, a shestipalost, asymmetry of papillas of the breast, additional rudimentary nipples, the doubled chin and a tip of language, etc. In an ebriogeneza defective is also a spinal cord which gliozny tissue was insufficiently differentiated by sites. In the course of ontogenesis as a result of provocative factors such as injury, an infectious disease, etc., this gliozny fabric is exposed to the pathological development noted above with formation of cavities. However such "true" myelosyringosis meets seldom. Much more often (in 2/3 cases) the myelosyringosis accompanies anomalies of a kranio-vertebralny joint (Arnold-Kiari's anomaly with omission of an almond of a cerebellum, a basilar impressiya, a platybasia, assimilation of the Atlas, Klippelya-Feyl's anomaly, etc.). At such anomalies the central spinal channel (gidromiyeliya) which at the level of separate segments of a spinal cord can extend and destroy aneurysmally gray matter of these segments with the corresponding clinical manifestations usually extends. Siringomiyelichesky cavities can form after a severe injury of a backbone and a spinal cord with a hematomyelia, and also at a heart attack of a spinal cord (a necrotic cyst). At a true myelosyringosis the disease gradually progresses.

Treatment of the Myelosyringosis:

To suspend increase in the sizes of a cavity at a true myelosyringosis x-ray radiation of the corresponding segments of a spinal cord is applied. At extensive damages of a spinal cord and myelencephalon use a radioiodine (with preliminary protection of a thyroid gland Lugol solution reception for fixing in cells of a thyroid gland usual, not radioactive, iodine. In the presence of large cavities in a spinal cord and the expressed accruing neurologic frustration in the form of a flaccid paralysis in hands and the central paresis in legs the possibility of neurosurgical intervention with the purpose to drain a siringomiyelichesky cavity is discussed. At a gidromiyeliya in connection with Arnold-Kiari's anomaly radiation therapy is not shown. Neurosurgical interventions are carried out on a kraniovertebralny joint (a decompression of a back cranial pole and a myelencephalon).

Conduct courses of drug treatment by dehydrating drugs, vitamins, neuroprotectors.