MYELOSYRINGOSIS (syringomyelia; Greek syrinx a pipe, a tube + myelos a spinal cord) — the chronic progressing disease of a nervous system which is characterized by growth of a glia and formation of pathological cavities in a head and spinal cord.
About existence patol. cavities in a spinal cord it is known from 16 — 17 centuries. For the first time Olivier D'Anger (Ch. - River of Ollivier d'Angers, 1824) began to consider them as display of a disease, to-ruyu called «myelosyringosis». A little later Shtilling (V. of Stilling, 1859) at the same time found expansion of the central channel of a spinal cord, a cut called a gidromiyeliya. Etiology, pathogeny, patol. the anatomy and S.'s clinic were studied by F. Raymond, Zh. Sharko and A. Joffroy, E. Leyden, Morvan (A. M. of Morvan), V. K. Roth, L. S. A minor, V. A. Muratov, etc. to Shulttsa (F. Schultze) considered S. a blastomatosis and called a gliosis or a gliomatosis. S.'s symptomatology was in detail described by V. K. Roth, to-ry proved that the disease described in 1883 Morvanom (called by Zh. Sharko a disease of Mor-vana) is a myelosyringosis. F. Bremer, S. N. D avidenok, D. A. Shamburov added and systematized data on S., especially it concerns questions of symptomatology and communication of a disease with the dizrafichesky status. Treat its signs: the funneled or zheloboobrazny breast, a rachiocampsis, deformations of brushes and feet, a spider finger, different size of mammary glands, additional nipples, a vertical fold between eyebrows, bifurcation of a tip of language and an under lip, change of reflexes, is more often absence Achilles, spina bifida, the high sky, anomalies of pilosis, maldevelopment of teeth.
S.'s incidence fluctuates over a wide range as around the world, and on small regions. Irregularity of distribution of S. is explained with formation of populations with small migration of inhabitants, existence of related marriages, and also nek-ry geochemical features of soils — a hyperoxemia, the small copper content, zinc, phosphorus, boron increased by the content of aluminum and magnesium, the disturbances of embryonic development and mutagenesis promoting increase in populations.
The greatest incidence of S. is noted among the persons occupied with physical work; among country people it is higher, than in the city. Men are ill 3 times more often than women. More than 80% of patients make faces aged from 10 up to 30 years.
The etiology and a pathogeny
uses the Greatest recognition the dizontogenetichesky theory connecting emergence gliosis (see) and formation patol. cavities in tissue of a spinal cord with disturbance of embryonic development of a nervous system — a delay and abnormality of a smykaniye (dysraphia) of a neurotubule and disturbance of process of normal maturing of cells that leads to «jamming» in a seam of mesodermal and ectodermal elements, to a disproportion of a differentiation of various elements of a nervous system, an arrest of development at this or that stage or to surplus of growth of one elements to the detriment of others. Excess formation of spongioblasts is most often observed, from to-rykh various forms of a glia and an epidermal epithelium form (see. Neuroglia ). Cells it is sometimes long keep embryonal properties and readiness for proliferation, edges comes at influence of disturbing factors; in the subsequent the gliomatosis and formation patol is observed. cavities. Disturbance of development of a neurotubule throughout or its parts is possible. Defects of development of a spinal cord are followed by the disturbances of development of other bodies and fabrics which are shown externally dizrafichesky status.
The Dizrafichesky status is followed by weak adaptation opportunities of an organism, its unusual reactions to exogenous and internal disturbing factors.
Disturbances of development of a nervous system at S. are genetically determined. According to a number of scientists, the dizrafichesky status at S. is inherited on an autosomal domi-iantnomu or autosomal recessively type. S.'s cases occur at members of one family seldom that speaks low penetrantnostyo and small expressivity of a hmutantny gene.
The hydraulic theory of an origin of S. formulated in 1957 by W. J. Gardner et al. is at first sight a little dissonant with the given judgments. According to this theory in the embryonal period during the formation of a nervous system and the central channel of a spinal cord there is a defect of development of the fourth cerebral cavity; stenosis of a median aperture (Marangdi's opening) and lateral aperture (Lushki's opening). In this regard pressure in the fourth ventricle increases, and at each cordial systole cerebrospinal liquid opens the central channel, in the subsequent expands it and creates patol. cavities. Further increase in pressure of cerebrospinal liquid leads to a rupture of an ependyma of the central channel and formation patol. cavities in the neighbourhood with it in tissue of a spinal cord. The hydraulic theory, most likely, does not contradict the dizonto-genetic theory and opens the mechanism of only one of options of development of anomaly of a nervous system. She offers a pathogenetic explanation of polymorphism a wedge, S.'s manifestations, especially those its options, to-rye proceed with symptoms hydrocephaly (see).
In a crust, time it is possible to consider conventional that as a result of injuries, intramedullary tumors, hematomyelias, its softening owing to disturbance of blood circulation, acute or hron. a myelitis, intoxications in a spinal cord can be formed patol. cavities, but they have no relation to true S.
At a macroscopic research of a spinal cord a thickening of firm and web covers, their unions on certain sites among themselves and with a soft cover, flattening, a thickening or thinning of separate or all its segments are found. In nek-ry expanded segments of a spinal cord also consolidations and fluctuation are defined. Characteristic of S. single or multiple patol. cavities (fig. 1) are localized preferential in the central departments of segments of a spinal cord, sometimes and in a brain trunk; white commissure at the same time is, as a rule, kept. Cavities are in most cases not tied with the central channel, extend to back columns and the central departments of back cords; they meet and in a front cord. Their configuration is various: round, oval, slit-like, in the form of hourglasses, with one or several diverticulums. The sizes of cavities are also various: from narrow, found only during the pressing on the side, front or back surfaces of a spinal cord, to large, occupying almost all diameter of a segment. Patol. cavities can be so big that the spinal cord gets a form of a thin-walled tube and is easily torn at neosto-rozhnokhm its allocation from a firm cover. Most often cavities extend on 1 — 2, is more rare — on bigger number of segments. Observation is described, in Krom of a cavity stretched from a segment of Ssh to LIT. In isolated cases of a cavity reach a myelencephalon and a varoliyev of the bridge (the bridge of a brain, T.). Varoliyev Bridge, as a rule, is border of their distribution in the oral direction, however observation when the slit-like cavity stretched from sacral segments through all departments of a spinal cord, a myelencephalon, the right half of a varoliyev of the bridge, the right leg of a brain, the internal capsule, a kernel having a tail is known and came to an end at a front horn of a side cerebral cavity.
At S. the combination of the described changes to an atresia or narrowing median and lateral apertures of the fourth cerebral cavity, by expansion of ventricles of a brain and the central channel of a spinal cord, and also with the malformations characteristic of the dizrafichesky status, with neuroectodermal tumors (most often with spongioblastomas and ependymomas) and angiomas is quite often observed.
At microscopic examination in walls patol. cavities glial fibers, in nek-ry cases — groups of ependimotsit (as a rule are found, at the message of a cavity with the central channel). Around patol. cavities, near them or in a distance from them focal accumulations of astrocytes, generally fibrous are visible, among to-rykh also not numerous proto plasmatic meet. These accumulations of fibrous astrocytes reaching the considerable sizes — so-called gliomatozny pins — are defined at palpation of a spinal cord as sites of consolidation. Gliomatozny pins are localized preferential near the central channel, is more often dorsalny than it. In them decomposition products of glial fibers (Rosenthal's fiber), collagenic fibers, numerous vessels meet the thickened, sclerosed walls, spongiob flippers and other glial cells with signs of an incomplete differentiation, multinuclear huge gliotsita. There is an opinion that focal disintegration of gliomatozny pins leads to formation of cavities. Near cavities depression of fibers of white matter, their swelling, vacuolation and fragmentation, acute swelling of neurons, their retrograde degeneration is found, two-nuclear neurons, astrocytes meet two and three kernels, glial macrophages, the roundish or extended sites of white matter, in to-rykh fiber are deprived of a myelin. On certain sites of a spinal cord the myelolysis — dissolution of its substance under the influence of the cerebrospinal liquid getting from the central channel through sites where there is no ependyma is found. As nek-ry scientists believe, the myelolysis also leads to education patol. cavities.
The clinical picture
the Clinical picture is characterized by polymorphism of symptomatology. At the same time degree of manifestation of symptoms of a disease varies from almost inaudible to rough with essential disturbances of functions.
S.'s symptomatology forms against the background of the dizrafichesky status with malformations of skin, hypodermic cellulose, muscles, bones, internals, a nervous system. Their nature, degree of manifestation, frequency of manifestations, combinations are very variable.
In some cases there can be no accurate external signs of the dizrafichesky status.
Preferential defeat of gray matter of cervicothoracic department is characteristic of typical cases of S. (a classical form) spinal cord (see) with the advent of a triad of symptoms: the dissociated loss painful and a thermoesthesia on segmented type on hands and an upper part of a trunk (see. Sensitivity ), atrophic paresis of hands (see. Paralyses, paresis ), trophic and vasomotor frustration. Most often disorders of sensitivity — pain of various localization and character, quite often painful, followed by burning sensation meet, paresthesias (see), the segmented dissociated type of loss of superficial sensitivity, a thicket in the form of «jacket» or «semi-jacket». Pains and paresthesias arise sometimes long before emergence of objective symptoms of a disease. Sometimes patients see a doctor concerning painless wounds, burns, suppurations.
Atrophic paresis is more expressed in distal departments of hands and is shown externally by development of a so-called sharp-clawed or monkey brush. Partial distribution of a muscular atrophy is characteristic that explains frequent discrepancy between expressiveness of thinning of muscles and extent of motive disturbances. At distribution of process on side cords of a spinal cord there are symptoms of defeat of conduction paths with the advent of conduction disturbances of sensitivity and development of spastic paresis of legs. From cherepnomozgovy (cranial, T.) nerves V, VII, IX, X, XII couples are often involved in process. Vegetative and trophic disturbances are presented widely and polimorfno: disorganization of peripheric circulation, a hyperhidrosis is significantly expressed (see), Bernard's syndrome — Horner can be observed (see. Bernard — Horner a syndrome ). In skin and its appendages atrophic and dystrophic changes are found, the hyperkeratosis and disturbances of pigmentation (fig. 2) are noted. Nails lose norkhmalny gloss, become opaque, fragile, ribbed. Felons are noted, to-rye proceed without pains. Trophic changes of bones, joints can be observed. Are especially characteristic a kyphoscoliosis of chest department of a backbone (see. Scoliosis ), deformations of a thorax in the form of a costal hump (fig. 3). The expressed destructive changes of bones which sometimes are followed patol are noted. changes at a small injury; the neurodystrophic osteoarthropathies proceeding without pain syndrome; the rough destructive changes of a bone tissue which are combined with its hypertrophy come to light.
Osteoarthropathies at S. develop preferential in an upper extremity. Damage of one joint meets more often, but are in rare instances observed change and several joints (see. Arthropathy ).
In development of an osteoarthropathy at S. radiological distinguish three stages — latent, a stage of destruction (preferential lytic) and a stage of reactive changes (preferential giperostotichesky). The latent stage is shown osteosclerosis (see) adjacent to joint surfaces of departments of bones, an uzuration of subchondral departments, narrowing of a joint crack owing to thinning of a cartilaginous cover or its expansion owing to accumulation of an exudate. Further there are otloma of edges of an epiphysis, a rassasyvaniye of the departments of bones, nearest to joints, and the whole epiphysis, metaphyses and even parts of a diaphysis often disappear.
Along with osteolytic processes there are also expressed giperostotichesky changes in a look excessive, a bizzare shape of beddings of a bone tissue, okosteneniye of soft circumarticular tissues. Destruction and creation of a bone tissue happens randomly, chaotically. At the same time there are changes and in the copular device — the joint capsule, sheaves relax that promotes excessive mobility in a joint, looseness, sharply expressed shift of bones of a joint — patol. to dislocation (see). Distinguish two forms of osteoartroiatiya — osteolytic and giperostotichesky, to-rye proceed according to preferential destructive, lytic, changes or with hyperostosis (see) — a considerable osteogenesis, with a fancy thickening of a bone and consolidation of its structure (fig. 4).
At S. elbow joints most often are surprised, is slightly more rare — humeral. Radiocarpal and interphalangeal joints are surprised seldom. In fingers of a brush changes of hl meet. obr. in the form of ossifluence of nail phalanxes, and also destruction of interphalangeal joints.
The most expressed changes are observed in bones of elbow joints. In shoulder joints destructive bone changes are less expressed, more often they are shown by partial destruction of a head of a humeral bone and a joint hollow of a shovel (fig. 5). Usually, despite big destructive bone changes, osteoporosis is not noted.
Damage of joints in the form of osteoarthropathies at S. often is followed by calcification and ossification of a bizzare shape and various density soft, hl. obr. circumarticular, fabrics, in some cases extremely plentiful.
Except osteoarthropathies, at S. are observed also an osteopathy — trophic extraarticular changes in a diaphysis of long tubular bones, hl. obr. in bones of a forearm, in the form of the local hyperostosis and a rassasyvaniye bringing to patol. to changes and formation of nearthroses (fig. 6). So, sometimes in an ulna the zones of reorganization and ossifluence (fig. 7) leading to disturbance of an integrity of a bone and a spontaneous change are observed. Peculiar changes of a skeleton in the form of increase in bones of a brush — so-called heyromegaliya are described.
The mental status is violated, memory, ability to generalization decreases, lability of the emotional and strong-willed sphere, decrease in mental capacities is noted.
At S. are often observed also gastritis (see), peptic ulcer (see), changes of sokratitelny ability and metabolism of a myocardium, insufficiency of pituitary and adrenal system with essential disturbance of somatotropic function of a hypophysis, sexual dysfunction, etc. Genital ability at women is kept, however viability of the born children low.
Cerebrospinal liquid (see) it is in most cases not changed, increase in its pressure, moderate increase in protein content (0,4 — 0,8 g/l), change of a proteinaceous range with reduction of prealbumine, alfa-1-and beta 1 - globulinovykh fractions, increase in content of gamma-globulin, emergence of additional proteinaceous components, antibodies to tissues of a brain is possible; increase in activity of aminotransferases (see), increase in content of atsetilkholinopodobny substances, sugar, copper, manganese, reduction in the amount of chlorides is noted.
In blood the content of crude protein is a little reduced, the disproteinemia, increase in content of fibrinogen, copper, creatine, glutathione (especially its oxidized phase), a gipoaminoatside-miya, lowering of the level of ceruloplasmin, increase in maintenance of Ig M are observed. Antigenic properties of proteins of serum are broken, increase of an antiserum capacity to fabrics is noted. The hyper aminoaciduria is characteristic (see. Aminoatsiduriya ).
The first symptoms of a disease are very various and to some extent predetermine the subsequent a wedge, a picture of a disease. At S. men proceeds heavier. At children pyramidal insufficiency is less often observed (see. Pyramidal system ) it is more weakly involved in patol. process bulbar department, disorders of sensitivity are insignificant, but bigger extent; development of a bone skeleton is sharply changed, vegetovascular frustration are brightly expressed.
Allocate three types of a current of S. — stationary, at Krom increase of symptoms, slowly progressing with slow increase of symptoms and quickly progressing with bystry increase and distribution of process to c is not noted. in the N of page leading to the expressed disorders of functions within 2 — 5 years.
Except typiforms of S. described above atypical forms are possible. E.g., at a syringobulbia patol. changes preferential develop in areas of a myelencephalon and varoliyev of the bridge, more often on the one hand, taking kernels of the glossopalatine, wandering, hypoglossal, additional nerves and the lower kernel of a spinal way of a trifacial. Phonation, articulation, swallowing are broken, there are an atrophy of language, a painful and temperature hypesthesia of the person on segmented type more sharply expressed in lateral departments, a nystagmus. Disorder of hearing and defeat of a facial nerve are sometimes noted.
The found seldom lumbosacral form is characterized by the same symptoms, as classical S., but frustration in the lower extremities are localized.
At a thoracolumbar form the dissociated disturbances of sensitivity in the field of chest segments of a spinal cord come to light, the spastic lower paraparesis, pelvic frustration, vegetative and trophic disturbances in legs are observed.
S.'s cases meet the clear symptomatology testimonial of presence of two or more patol. centers.
the Diagnosis is established on the basis of a characteristic complex of frustration painful and a thermoesthesia at persons with the dizrafiche-sky status. Are of great importance in diagnosis a computer tomography of a head and spinal cord (see. Tomography computer ), and also results of a research of cerebrospinal liquid (see), pnevmomiyelografiya (see), an electromyography (see), an electroencephalography (see), immunochemical and biochemical researches.
At rentgenol. a research inborn changes of a skeleton, a platybasia (see Kraniovertebraljny anomalies), spina bifida (see), Klippel's syndrome — Feylya can come to light (cm. Klippelya — Feylya a disease ), a kyphoscoliosis (see Scoliosis). On tomograms of a backbone in some cases it is possible to find expansion of bone borders of the vertebral channel in the sagittal direction. On miyelo-grams diffusion expansion of a spinal cord is often noted. Pnevmomiyelografiya in S.'s diagnosis is applied seldom. Changes in bone and joint system at S. have characteristic rentgenol. the picture consisting of sharply expressed dystrophic and destructive changes, patol. intra joint and extraarticular changes (see), mutual shifts of the destroyed joint surfaces of bones (patol. dislocations), calcifications and patol. osteogeneses.
Differential diagnosis carry out with hematomyelia (see), an intramedullary tumor, neuritis of a brachial plexus — plexitis (see), leprosy (see), amyotrophic side sclerosis (see), back tabes (see). At a hematomyelia the wedge, symptoms are connected with an injury of a spinal cord, develop sharply and are followed by disturbance of the general condition of the patient; in the subsequent the disease regressing. Distinguishes existence of the persistent pains which are not giving in to usual types of therapy, their peculiar parestetichesky nature, localization of pains in zones gipestezin and vegetative and trophic frustration of segmented type, lack of painful points and symptoms of a tension, existence of signs of the dizrafichesky status from S.'s plexitis. It is also necessary to differentiate S. with vertebrogenny radicular syndromes. Young age, absence rentgenol. the data indicating loss of an intervertebral disk, existence of dizrafichesky signs, the permanent losses of sensitivity which are going beyond cervical segments, lack of painful points and symptoms of a tension confirm rather S., than the syndromes mentioned above.
Differential diagnosis of S. with a leprosy sometimes presents great difficulties. Local disturbances of sensitivity, nevus pigmentosus, eyebrow shedding, thickening of peripheral nerves are characteristic of a leprosy.
The intramedullary tumor differs from S. in absence at the patient of signs of the dizrafichesky status; it progresses with the advent of conduction and pelvic disturbances quicker; its extent on a longitudinal axis of a spinal cord is less, than defeat at Page.
The forms C. which are followed by an atrophy of muscles should be differentiated with an amyotrophic side sclerosis, for to-rogo symmetry of muscular atrophies, existence of a hyperreflexia, quickly progressing disease is characteristic. At S. decrease in an animal force and restriction of movements are usually preceded by hypotrophies, painful and dizrafichesky symptoms, vegetative and trophic disturbances.
Treatment complex. It is necessary to approach its appointment individually taking into account character of a current and activity of process. The best results are received at early the begun regularly carried out complex therapy, edges use of amino-acid drugs, hydrolyzates of proteins (Hydrolysinum, Aminopeptidum, Amincrovinum, a hydrolyzate of casein) on 200 — 250 ml-pelno intravenously or subcutaneously 1 time in 3 — 4 days (four injections on a course), vitamins of group B includes, dezintoksikatsioniy therapy (Unithiolum, 5% solution on 5 ml intramusculary daily, to 15 injections on a course; cuprenil, 0,25 g 2 — 3 times a day), radiation therapy for the purpose of delay of proliferation of gliozny elements in iatol. center.
Radiation is carried out on gamma and therapeutic installations, betatrons and linear accelerators. Irradiate only the struck segments of a spinal cord. Radiation is carried out 5 weekly from two juxtaspinal fields with a tilt angle of the central bunch for cervical department of a spinal cord 60 °, chest and lumbar departments — 45 °. Single focal dose 90 I am glad (0,9 Gr), total — 900 — 1100 I am glad (9 — 11 Gr). At damage of a medulla radiation is carried out with direct or two side occipital fields in a single dose 50 — 70 I am glad (0,5 — 0,7 Gr); a total dose to 400 — 500 I am glad (4 — 5 Gr) at a rhythm of radiation 2 — 3 times a week. Repeated courses of radiation therapy are conducted during the progressing of process, but not earlier, than in 1 — 1V2 years.
Radiation therapy (see) is contraindicated at the leukopenia, pregnancy open for a form of a pulmonary tuberculosis.
Use also radioactive drugs 131 I and 32 P, to-rye are selectively absorbed by gliozny tissue of a brain. 131I activity of 50 — 100 m of curie enter 2 times a week to total activity 500 — 800 mkyur. Conduct not less than 4 — 5 bucketed courses between them 1 — 1,5 years. 32P appoint on 150 — 170 mkyur at an interval of 3 — 4 days to total activity 450 — 500 mkyur. In 3 months the course of treatment is repeated. Radonic bathtubs have more sparing effect on an organism and do not cause by-effects, but concede to radiation therapy on firmness of a favorable effect. At the expressed pain syndrome good results are received at use acupuncture (see). At disturbance of microcirculation use of Prodectinum, nicotinic to - you is shown. Antikholinesterazny drugs, massage are shown, to lay down. physical culture. Courses of complex treatment it is necessary to carry out 2 — 3 times a year to stabilization of process. It is possible to recommend a dignity. - hens. treatment with use of natural radioactive waters.
As the indication to operational treatment serves steady progressing of a disease with considerable pains and conduction symptoms. The purpose of operation — elimination or reduction of a compression of a spinal cord due to removal of liquid from a cystous cavity and prevention of its repeated accumulation after operation. The area of a local thickening of a spinal cord corresponding to an arrangement of a cavity is established with the help miyelografiya (see) or computer tomography.
Later laminectomies (see), openings of a firm cover of a spinal cord, a puncture of a siringomiyelitichesky cavity with extraction of a part of its contents longwise cut a wall of a cavity in the most thinned site, usually otstupya on 3 — 4 mm from the centerline (Puusepp's operation offered in 1926). For the purpose of prevention of a smykaniye of a dissect wall of a cyst various modifications of this operation are developed, at to-rykh for drainage enter a strip of a firm cover of a spinal cord, silk or catgut threads, rubber or polyethylene tubes or a distal piece of the crossed blizraspolozhenny nervous root of a spinal nerve then the firm cover of a spinal cord and soft tissues are sewn up layer-by-layer tightly into a cavity. When the siriigomiyelitichesky cavity is reported with gidromiyelitichesky expansion of the central channel or there is a gidromiyeliya which is reported with a cavity of the fourth ventricle, carry out a laminectomy in upper cervical area and delete a lower part of scales of an occipital bone. After opening of a firm cover of a brain bare a lower part of the fourth ventricle and tampon the site of the message of the last with a gidro-miyelitichesky cavity (expansion of the central channel of a spinal cord) a piece of muscular tissue to dia. 0,5 cm, exsected from operational area (Gardner's operation offered in 1957).
In the postoperative period hold the same events, as after removal of tumors of a spinal cord and a back cranial pole. Special attention should be paid to prevention of decubituses and burns owing to often available for sick disturbances painful and a thermoesthesia; at a delay of an urination catheterization is shown.
The forecast and Prevention
the Forecast for life rather favorable, concerning recovery — adverse. Working ability of patients can be kept it is long at the correct employment.
Prevention includes medikogenetichesky consultation (see. Medicogenetic consultation ), early identification of initial forms of a disease, dispensary observation (see. Medical examination ) behind persons with sharply expressed dizrafichesky status. Sick S. and their relatives, especially in the presence at them signs of the dizrafichesky status is (risk group), it is necessary to acquaint with factors, to-rye can be an incitement to development of a disease, an aggravation of symptoms (injuries, accidental wounds, household and professional intoxications, burns, frostbites, a physical overstrain, disturbance of food). Rational vocational guidance and employment, creation are necessary for patients favorable a dignity. - a gigabyte. conditions, full protein-rich and vitamins food.
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