BACK TABES (tabes dorsalis; synonym: tabes, the progressing locomotory ataxy of Dyushenn) — one of forms of the late progressing neurosyphilis.
It is allocated in separate a wedge, a form in 1858 by Dyushenn. The village of the village develops at 2 — 3% of patients with syphilis, generally when treatment was insufficient or was not carried out at all. A span from infection syphilis (see) to the first the wedge, S.'s manifestations by the village makes from 6 to 30 years (more often 10 — 15 years).
the Causative agent of syphilis, a pale treponema, in nervous tissue or cerebrospinal liquid of sick S. of page find extremely seldom. Nevertheless the unity of an etiology of all forms of early and late neurosyphilis in a crust, time does not raise doubts. Disputes on whether S. belongs to page to syphilitic defeats of a nervous system literally or to a so-called metasyphilis [the term is offered by P. J. Moebius in 1876], are property of history. At the end of 19 century the fr. syphilologist A. Fournier formulated the thesis: there are no tabes back and a general paralysis (see) without syphilis. In the subsequent, with introduction of standard serological tests (Wassermann reaction, Kan and Legislative Assembly — Vitebsk), as a result of works of X. Noguchi (1913), the found causative agent of syphilis in tissue of a spinal cord of sick S. of page, use of specific reactions to syphilis (reaction of an immobilization of pale treponemas, reaction of an immunofluorescence, reaction of immune sticking) this wedge. the postulate received biol. confirmation. The solution of a question of unity of an etiology of all forms of syphilis did not give, however, answers to questions of what long stage of latency of development of S. of page, the progressing current of this form of late neurosyphilis, S.'s resistance of page to specific treatment is connected with. On one of the existing hypotheses S.'s emergence by the village is connected with change of biological properties of a pale treponema in the course of the course of syphilis under the influence of fabric and immune factors of an organism of the patient therefore the activator gets defined neyrotropizkhm. According to other hypothesis which does not have more or less firm justifications yet existence of different types of pale treponemas, including with the neurotropic properties predetermining defeat of a nervous system is supposed.
the Pathogeny is insufficiently clear. The greatest difficulties are caused by an explanation of preferential localization patol. process in back roots of spinal nerves, back cords of a spinal cord and their continuations in a brain trunk; also the conditions promoting transition of early neurosyphilis to S. of page are not completely clear. Selectivity of defeat of separate systems of nerve fibrils, according to M. S. Maher-gulisa (1947), is result of a combination of a number of pathogenetic factors — anatomo-mechanical and biological. Features of circulation of cerebrospinal liquid within subarachnoid (subarachnoid) space of a spinal cord and feature of an anatomic syntopy of a meninx, roots of spinal nerves and spinal nodes concern to the first. Delay of circulation of cerebrospinal liquid in chest and lumbar departments of a subarachnoid space, and also in its part separated by a gear sheaf (see the Meninx, the Spinal cord, anatomy), can promote accumulation of pale treponemas and products of their life activity in these parts and to cause infektsionnotoksichesky damage to back cords of a spinal cord and back roots of spinal nerves. Defeat of back roots, thus, according to M. S. Marguli-s, is not the only reason of a degeneration of back cords, the pathogeny a cut is difficult. J. Nageotte and Richter (N. of Richter), on the contrary, considered that at S. of page patol. process begins in a radicular nerve, and the secondary degeneration of back cords is a direct consequence of infektsion-but-toxic defeat of an extradural part of a back root. Also another spoke, so-called funicular, the theory based on the assumption that changes in back roots of spinal nerves are a consequence of their retrograde degeneration. However the convincing evidence of this concept is not obtained.
In modern ideas of S.'s pathogeny of page especially important part is assigned biol. to a factor — to the autoimmune reactions arising in response to defeat of nervous tissue in the early period of neurosyphilis. To a crust, time does not have a consensus of rather provocative role of injuries, intoxications (as a rule, alcoholic), somatic burdeness, long hron. infections in S.'s development by page in patients with syphilis.
Macroscopically spinal cord (see) it is flattened and thinned, its back cords and back roots of spinal nerves are thinned and wrinkled. Microscopically in an initial stage of S. of page reveal a pia-arachnitis (see. Meningitis ) around an intradural part of back roots and in the field of back cords with infiltration by lymphocytes and plasmocytes, and also proliferative changes in walls of vessels, connecting fabric, in peri-and endonevriya of back roots. The changes described above are clearer in lower parts of a spinal cord — sacral and lumbar. In a stage of the expressed S. of page defeat is characterized by a degeneration of the central shoots of cells of spinal nodes, at the same time nodes are usually poorly changed. Especially considerable degenerative changes note in an extradural part of back roots and in a zone of their introduction in a spinal cord where they are squeezed by fibrous and reinforced covers and the expanded regional glia (see. Neuroglia ). Degenerative changes (disintegration of a myelin cover and axial cylinders of the axons forming back cords) of fibers of roots extend up back cords of a spinal cord. In a final stage of process on site of atrophied fibers of back roots and cords the fibrillar glia and connecting fabric expands. In lumbar and sacral departments of a spinal cord disintegration of a hmiyelin begins in a medial part of back cords. In chest and cervical segments of a spinal cord of a degeneration all area of back cords (fig. 1) is exposed. It is found also in peripheral shoots of cells of the spinal nodes which are a part of formations of a peripheral nervous system (textures, nerves) and also in various departments of century of N of page. In many cases reveal a degeneration of fibers craniocereberal (cranial, T.) nerves in their intracranial piece. At different stages of S. of page infiltrative, proliferative and cicatricial changes in covers of a brain, and also defeat of vessels of a meninx can be found.
A clinical picture
Men get sick with S. page approximately by 4 — 6 times more often than women. The first wedge, symptoms usually appear aged after 30 — 40 years. Occasionally at inborn syphilis the wedge, S.'s symptoms of page can come to light aged up to 10 years. The classical description a wedge, S.'s symptoms of page in their consecutive development Is given by V. Erb, G. O captivity a game and Schaffer (To. Schaffer) distinguishing an initial stage of development of a disease, a stage of the expressed S. of page (atactic) and final stage (paralytic).
Early symptoms of S. of page — characteristic paresthesias (see) and tabic pains. Paresthesias are shown by a pricking, feeling of crawling of goosebumps and numbness, arises in lower distal parts more often, is more rare — upper extremities or on a trunk. Paresthesias on a trunk are followed by feeling of tightening, pressure, a banding on the certain sites corresponding to the level of segmented defeat of certain roots of spinal nerves. Tabic pains define as shooting, «knife-like», tearing. They begin suddenly, usually last 1 — 2 sec., but several hours and even sometimes proceed days. Pains more often happen in legs, however their prevalence and localization are various and connected with localization of defeat of roots of spinal nerves and cranial nerves. Quite often megalgias have the nature of visceral crises, to-rye note approximately at 15% of sick S. of page. They are followed by pains in epigastriß area, sometimes with vomiting (so-called gastric crises; meet in 8% of cases of S. of page), or colicy pains on the course of intestines tenesmus and a diarrhea (intestinal, rectal crises). Also other crises are possible: guttural (convulsive cough), pulmonary (suffocation), cordial, uterine, vulval, etc. Crises can repeat within several days. Intensity of visceral crises and their character vary. Displays of crises can be sometimes taken for attacks of stenocardia, hepatic or renal colic, etc.
In nek-ry cases of S. of page also hypothalamic crises (usually sympathoadrenal type), with fervescence are observed (see. Hypothalamic syndrome ). At frequent visceral crises, especially gastric and intestinal, at nek-ry patients quickly there occurs sharp weight loss (a marantic forkhma of S. of page), in a pathogeny to-rogo also disturbance of the central regulation of fatty and carbohydrate metabolism plays a role.
At nevrol. a research in an initial stage of S. of page reveal decrease in tactile and painful sensitivity on a trunk in dermatomas of Thm — ThVII (a zone of Gittsiga), and also on the elbow surface of forearms and on an outer surface of shins. Disturbances of sensitivity on a face are more often than a polimorfna and a zone, in to-rykh they come to light, have no certain borders. Quite often the prick at a research of sensitivity is followed by an unpleasant burning sensation with irradiation around a site of application of irritation. In an initial stage of S. of page vibration sensitivity (see), and also a deep joint myesthesia decreases (see. Sensitivity, frustration ), especially in toes. In parallel with disorder of deep sensitivity other classical symptom of S. of page — an ataxia, an ataxy begins to develop (see). It is shown by instability during the walking in the dark and blindly, and also at a research of a symptom of Romberg (see Romberg a symptom). Tendon jerks (see) at first can be raised, but then quickly begin to die away and disappear absolutely.
Almost constant symptom in an initial stage of S. of page is the disorder of function of pelvic bodies which is expressed a delay of an urination and persistent locks.
From cranial nerves most often are surprised visual, oculomotor, is more rare acoustical (before - door and cochlear) nerves. In 10 — 20% of observations at classical S. of page simple or primary (gray) atrophy of optic nerves is found (see. An optic nerve) with the progressing bilateral decrease in sight.
The pupillary syndrome of Argayll Robertson belongs to characteristic manifestations of S. of page (see Argayll Robertson a syndrome). Besides, at S. of page pupils often have irregular shape, neravnokhmerna in size (anisocoria), are narrowed. The full reflex immovability of pupils is sometimes observed. Treat rather early and frequent symptoms ptosis (see), squint (see), caused by defeat of third cranial nerves. Defeat before - a door and cochlear nerve leads to decrease in hearing.
In a stage of the expressed S. of page in connection with the progressing degeneration of conductors of deep sensitivity and disturbance of a segmented reflex arc the ataxy accrues (especially in legs), disappear knee (see Vestfal symptoms) and akhill the reflex (see), develops a hypomyotonia; gait changes — patients go, widely placing and highly throwing out legs forward, «stamping» heels. Hypotonia of muscles of a sgibately shin leads to the fact that in a standing position there is overextension in knee joints (genu recurvatum). Along with an ataxy and hypotonia the tabic analgiya goes deep (see). Its brightest manifestations — painlessness at a prelum of an elbow nerve (sgshpty Bernatsky), absence of pain at a prelum of an Achilles tendon (sikhmpty Abadi), a prelum of a small egg, with a pressure upon an eyeglobe — Genel's symptom. Normal painful sensitivity of an eyeglobe to pressure is shown at load of it in 150 — 350 g. If pain arises only at bigger loading, Genel's symptom is considered positive. Existence of a symptom of Genel without other pages of signs, characteristic of S., gives the grounds to suspect S. of page with high (cervical level) localization of process of a spinal cord.
In this stage of a disease dysfunction of pelvic bodies is characterized by an incontience of urine (see) and calla; the impotence (see) arising in the presence of other symptoms of S. of page is regarded as tabic.
In a final stage of a disease the ataxy and hypotonia hmyshts amplify sometimes so sharply that patients cannot go; owing to disorder of deep sensitivity idea of position of extremities in space disappears, coordination of movements is sharply broken. Trophic frustration develop: the pro-butting painless trophic ulcers of foot (malum perforans pedis), are more rare — palms, wings of a nose, a soft palate, etc. At 5 — 10% of patients arthropathies develop (see), is more often than a knee joint (see fig. 2 to St. Arthropathy ), is more rare talocrural, coxofemoral and elbow. In rare instances observe arthropathies of a backbone. Intra joint and extraarticular patol. fractures of bones at S. of page are painless; arthropathies cause sharp deformation of joints, however the passive movements and a palpation of the affected joint of pain do not cause.
In a late, final stage of S. of page the mental disturbances which are shown most often an adynamy develop (see. Asthenic syndrome ). The acrimony, fatigue, affective instability with dominance of depression are combined with hypochiondrial fixing on the state, especially in the presence of visceral crises. The asthenic syndrome sometimes is followed by decrease in memory. At S. of page there can be also so-called tabic psychoses, usually in the form of a hallucinatory-dilision syndrome (see. Paranoid syndrome). Less often delirious and amental and delirious syndromes develop (see. Amental syndrome , Delirious syndrome ). Progressing at S. of page intellectual mnesticheskikh frustration, lack of critical evaluation of the state, complacency, carelessness, euphoria, sometimes emergence of the crazy ideas of greatness give the grounds for suspicion that the general paralysis joined S. of page, i.e. developed taboparalysis (see).
Cerebrospinal liquid (see), S. which is usually changed in an early stage the page, in the subsequent becomes frequent normal. At a spinal puncture often note build-up of pressure of cerebrospinal liquid to 240 mm w.g.; liquid is colourless, transparent, contains up to 1,2 per milles (1,2 g/l) of protein; globulinovy reactions are positive; colloid reactions with gold of a pla mastic give changes in 2 — the 4th test tubes. A pleocytosis to 100 (on average 30 — 60) lymphocytes in 1 mkl liquids. Wassermann reaction in a large amount of cerebrospinal liquid is positive approximately at 80% of patients, in blood — approximately at 30 — 50%. The blood count is usually not changed.
S.'s current of page is very various. Due to the progress of the modern combined treatment of early forms of syphilis (see Syphilis, treatment), and sometimes and at the persons who were not receiving treatment concerning syphilis note changes in the classical scheme of a current of S. of page: severe forms of S. of page develop seldom; the course of a disease became slower and high-quality; often reveal atypical and monosimptomny forms of a disease. According to modern researchers, in a crust, time from classical symptoms most often note pupillary frustration (a miosis, an anisocoria), Argayll Robertson's symptom, the phenomena of an ataxy (instability in Romberg's pose), disturbance of tendon jerks and is rare (in 6 — 8% of observations) — primary atrophy of optic nerves. The progressing form when the wastage of sight comes to an end with a blindness, and stationary meets, at a cut sight falls to a certain limit and the long time remains at this level. Primary tabic atrophy of optic nerves can be the only manifestation of S. of page. Tabic arthropathies arise in a crust, time at 1 — 2% of sick S. of page, at 2 — 8% of patients happen to a tabic arthropathy patol. changes (see). The typical option a wedge, pictures with preferential damage of the lower extremities (the lower tabes) remains; selective defeat of segments of a cone of a spinal cord (anesthesia in an anogenitalny zone, dysfunction of pelvic bodies) or preferential defeat of areas of a shoulder girdle and a neck (cervical tabes) is seldom noted. Sick S. of page remain a long time able-bodied and before accession of the intensive shooting pains and visceral crises seldom ask for the help the doctor.
the Diagnosis usually comes easy in the presence of the main triad of symptoms: Argayll Robertson's symptom, lack of deep reflexes on extremities, decrease in joint muscle sense, and also such characteristic symptoms as the shooting pains, an ataxy, a dysuria. However in connection with development of atypical and oligosymptomatic forms of a disease and that fact that most of patients with S.'s symptoms of page deny the postponed syphilis at S.'s diagnosis by the village serological researches (see Wasserman reaction, Legislative Assembly — Vitebsk reaction, Cana reaction) and researches of cerebrospinal liquid are of particular importance. At the same time at sick S.' number of page can proceed with negative standard serological tests and lack of changes of cerebrospinal liquid. Therefore in S.'s diagnosis by the village reaction of an immobilization of pale treponemas has essential value (see. Nelsona — Meyer reaction ), edges happens positive more than at 95% of patients. At most of patients also reaction is positive immunofluorescence (see) with not - divorced cerebrospinal liquid.
The X-ray pattern of tabic changes in a skeleton sometimes reminds others patol. processes in joints, napr, the deforming osteoarthrosis, tubercular arthritis (see. Arthritises , Arthroses ) or osteosarcoma (cm.). The differential diagnosis should be carried out also with other neurogenic damages of a skeleton — at a myelosyringosis (see), injuries of a spinal cord and peripheral nerves.
To the main rentgenol. to signs tabic arthropathies carry patol. intra joint changes (see), to-rye usually happen many and splintered. Sometimes patol. changes are accompanied by a massive necrosis of the joint ends of bones (fig. 2, a). Disintegration of a bone tissue in the joint ends of bones leads to their mutual shift — to an incomplete dislocation and dislocation. Torn away as a result patol. an intra joint change bone fragments quite often come to light radiological as intra joint free bodies. As a result of long functioning of the affected joint in patol. conditions in it there can be a peculiar mutual otshlifovka of joint surfaces. At less expressed destruction of a bone tissue only flattening of a natural relief of joint surfaces of bones comes to light.
Importance in diagnosis of a tabic arthropathy (see) have lack of a bone anchylosis (see), patol. calcification and ossification in the joint capsule and in circumarticular fabrics. This process of a metaplastic osteogenesis radiological is shown by shapeless «limy» shadows around the deformed joint. In metaphyses of a tubular bone at considerable destruction of its joint ends periosteal imposings can be revealed. At S. of page, despite considerable destructive changes in bones, osteoporosis (see) usually is absent; often observe the expressed osteosclerosis (see). Extraarticular patol. changes at S. page are followed by productive reaction with formation of a big bone callosity (fig. 2, b), and this reaction is more expressed in a diaphysis of tubular bones and at the considerable shift of fragments. In nek-ry cases patol. the change can not be followed by noticeable periosteal reaction. Also damages of a backbone (spondylopathy) at S. differ in dominance of productive changes in page (fig. 2, c).
Differential diagnosis carry out with other forms of spinal syphilis, hron. a polyneuritis (see), such diseases as neural amyotrophy of Sharko — Mari — Here (see. Amyotrophy ), Fridreykh's ataxy (see. Ataxy ) and Russia — Levi a syndrome (see), a tumor of a horse tail, a myelosyringosis (see) at its lumbosacral form, a miyeloishemiya, a funicular myelosis (see), multiple sclerosis (see), Adee's syndrome (see. Psevdotabes ), etc.
Help to distinguish S. of page from a polyneuritis such signs as Abadi's symptom (at a polyneuritis pressure upon nerves and an Achilles tendon is sharply painful), safety of knee jerks at a polyneuritis, etc. Observations of pseudo-tabes at intoxication are described by Ergotinum («ergotinny tabes»), at Krom back cords of a spinal cord are also surprised. At Adee's syndrome the pupil very poorly reacts to light (more often on the one hand), however its reaction (narrowing) to convergence live, and after narrowing the pupil slowly extends. Adee's syndrome is not connected with syphilis, however at it in 60% of observations there are no akhillova knee and reflexes. Lack of deep reflexes standing can be inborn (a partial or total areflexia), at a myelodisplasia and the dizrafi-chesky status (see. Myelosyringosis ). At a combination of an areflexia (see) with the diseases of internals which are followed by painful attacks it is necessary to carry out the differential diagnosis with visceral tabic crises.
At identification at the patient of signs of S. of page conduct an intensive course of specific treatment according to the special instructions approved by M3 of the USSR (1976, 1981). Treatment of late forms of syphilis, in addition to introduction of massive doses of penicillin, assumes use of the drugs containing iodine and bismuth (biyokhinol, Bismoverolum). At an atrophy of optic and acoustical nerves it is necessary to show care in purpose of massive doses of penicillin and drugs of bismuth; in these cases apply a pyrotherapy (Prodigiosanum, pyrogenal, etc.) in combination with vitamins. Periodically use a symptomatic treatment. At the shooting pains appoint analgetics, and at especially sharp pain syndromes sometimes make neurosurgical intervention — tractotomy (see). At mental disturbances apply tranquilizers, nootropic drugs, neuroleptics. Visceral crises stop ganglioblokatora-m, sedative, hyposensibilizing and antispasmodics; at arthropathies appoint delagil, indometacin, physical therapy, carry sometimes out an artificial ankylosis; at an ataxy — special to lay down. gymnastics. Criteria of efficiency of protivosifilisny treatment (see. Syphilis, treatment ) data of clinical inspection and a research of cerebrospinal liquid are; results of serological researches in assessment of efficiency of therapy of S. of page play a smaller role.
Quite often a disease under the influence of treatment (sometimes and without it) is stabilized, and patients live decades with lacks of coordination and other symptoms of a disease. At insufficiently effective treatment the disease progresses, other forms of syphilitic defeat of a nervous system, in particular a general paralysis can join. The forecast becomes complicated at cardiovascular syphilis (12 — 15% of sick S. of page), a cerebral stroke with a heart attack of a brain owing to thrombosis of specifically struck vessels of a brain. The lethal outcome is possible from intercurrent diseases and other complications, in particular from an urosepsis.
Prevention consists in early detection and treatment of syphilis.
See also Syphilis .
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D. K. Bogorodineky, A. A. Skoromets; S. A. Sviridov (rents.), M. A. Tsivilno (psikhiat.).