ATAXY (grech, ataxia lack of an order, disorder; synonym inkoordination) — the disturbance of motility which is shown an ataxia. At And. the movements become inexact, awkward, disproportionate, often do not achieve the goal, walking, sometimes the speech can suffer. The reflex of a muscle on stretching changes. Coherence of action of muscular groups is broken: agonists, antagonists, synergists; at the same time the patient has no signs of defeat of pyramidal, extrapyramidal system, a peripheral motor neuron.
Depending on localization of defeat allocate sensitive and cerebellar And. In some cases And. can be the leading syndrome of some hereditary and acquired diseases.
Sensitive ataxy develops at defeat of a way of proprioceptive sensitivity (see). The proprioceptive system ceases to receive and process kinaesthetic signals from sensitive nerve terminations in muscles and in sinews. Receipt of information on extent of reduction of muscles stops. That party of motive function falls apart, edges in physiology it is designated by the term «return afferentation», in cybernetics — «feed-back». At sensitive And. deep sensitivity is broken, both coordination of movements, and muscular and joint feeling suffers.
Localization of atactic frustration varies depending on the place of damage of a proprioceptive path. At defeat of back cords at height of chest and lumbar segments only legs suffer, during the involvement of back cords above a cervical thickening — at the same time hands and legs. Hemilesion of gentle and wedge-shaped bunches leads to a gomolateralny hemiataxia, on the party of the center function of a top and bottom extremity is broken. At defeat of a medial loop above decussation the hemiataxia on the opposite side appears. At damage of a thalamus the hemiataxia on the opposite side also develops.
At expressed sensitive And. in hands performance even of the simplest household actions is complicated. The patient cannot clasp and undo a button, bring glass with water to a mouth, without having spilled water, to get precisely an index finger to a tip of a nose. At rest sometimes it is possible to see the involuntary movement by fingers of hands reminding athetosis (see). Diagnostic tests are incorrectly carried out. In attempt of the patient to get a heel of one leg into a knee another the shin describes zigzags, the heel gets above, below a knee. Also the second phase of this test is badly carried out — the patient conducts a heel of one leg on a crest of a tibial bone of other leg tolchkoobrazno, with a deviation to the right, to the left. The muscle tone in the affected extremities is lowered both in sgibatel, and in razgibatel. Standing is followed by a poshatyvaniye, especially at a smykaniye of feet and simultaneous closing of eyes (see. Romberg symptom ). Walking becomes chaotic, feet fitfully rise and with knock fall by the earth, the patient goes with the hung head, all the time looks under legs. Walking sharply worsens at switching off of visual control.
Sensitive And. meets at tabes, polyneurites (so-called peripheral pseudo-tabes).
Cerebellar ataxy develops at damage of a cerebellum and its bonds, it is characterized by motive frustration. Its pathophysiological essence comes down to disturbance of proprioceptive reflexes (see), the muscles which are becoming isolated in a cerebellum and introducing correction in tonic and phasic function. Cerebellar And. develops not only at defeat of substance of the cerebellum, but also its conduction paths, both afferent, and efferent.
On cerebellar And., unlike sensitive, closing of eyes does not exert special impact, deep sensitivity at it remains kept. Distinguish several forms cerebellar And. At dynamic And. it is broken, performance of various autokinesias by extremities. Usually reveal it a finger - nasal and pyatochnokolenny tests. This look And. meets at defeat of hemispheres of a cerebellum. In the clearest form these frustration come to light at a research of upper extremities. The hand moves with exceeding of necessary volume, is brought further a subject which should be taken, fingers are unbent more than it is required (gipermetriya). In attempt to hit a finger the mark the hand begins to shiver the stronger, than it is closer to the purpose (intentsionny trembling). The impossibility to make the bystry consecutive alternate movements, napr, pronation and supination of a brush (adiadokhokinez) is characteristic of these patients. Hypotonia of muscles develops. The speech falls apart: its smoothness is lost, it becomes slowed down, explosive, the chant is sometimes observed; the letter (fig. 1) changes.
At statiko-locomotory And. generally standing and walking falls apart. The patient costs with widely placed legs, rocks, during the walking often turns aside, gait reminds gait of drunk. Sometimes the spontaneous nystagmus is found in the patient. Coordination of autokinesias in a pose sitting or lying changes a little.
This look And. at first connected with defeat of a worm of a cerebellum, in the subsequent — from its flokkulo-nodulyarny share [Larsell, Dau, Fulton (O. Larsell, R. S. Dow, J. F. Fulton)]. Impulses from vestibular system come to this area; at its defeat the reflexes maintaining balance are broken, synergistic reductions of muscles of legs, trunks and necks fall apart. During the bending of a trunk back at the patient there does not come reflex bending of legs in a knee and it can fall owing to excessive movement of the center of gravity of a kzada (fig. 2) back.
Asynergias in reduction of muscles of a trunk and extremities can be revealed at the patient and in lying situation. The patient does not manage to sit down without hands, instead of a trunk legs up climb (a cerebellar asynergia Babinsky, fig. 3).
I. M. Irger described a special look And., arising at defeat of segments of an average worm of a cerebellum. The clinical picture is expressed by an easy poshatyvaniye during the standing and walking without true disturbance of balance of a body.
The author connects a pathogeny of these frustration with disturbance of interaction of the damaged area of a cerebellum with the visual and acoustical centers of a mesencephalon.
And. can arise also at defeat of a vestibular analyzer, in particular its proprioceptors in a labyrinth — labyrinth And. At it balance of a body falls apart, during the walking of the patient deviates towards the struck labyrinth. The rotatory vertigo is characteristic, the spontaneous horizontally ротаторный nystagmus appears. On the party of an affected labyrinth hearing is broken.
The cerebellar ataxy meets at various defeats of a nervous system (at tumors of a back cranial pole, at infektsionnoallergichesky diseases, at some intoxications, dystrophic processes).
An ataxy at hereditary and acquired diseases
Family ataxy of Fridreykh — rather rare hereditary disease which is transmitted preferential on autosomal recessively type (see. Inheritance ). It is characterized by the progressing ataxia, disturbance of deep sensitivity, nek-ry pyramidal signs. N. Friedreich in 1863 described clinic and pathological anatomy of this disease, having separated it from the progressing locomotory ataxy of Dyushenn (see. Back tabes ).
Pathoanatomical find thinning of a spinal cord, dystrophy of preferential back and side columns, Lissauer's zones, chest kernels (Clark's kernels).
An onset of the illness in 6 — 15 years. The first symptom — disturbance of gait (a poshatyvaniye, a spotykaniye). Gradually disorders of coordination extend to upper extremities, a trunk, a face, sometimes speech muscles. Quite often trembling, a choreoathetosis, a nystagmus joins. The low muscle tone, an areflexia, Romberg's symptom is noted. Some pyramidal signs (Babinsky's symptom, protective reflexes) meet. Neurologic frustration are constantly combined with anomalies of a skeleton (so-called foot of Fridreykh, a kyphoscoliosis). Often find inborn heart disease. The disease slowly progresses, becoming aggravated after intercurrent infections. Patients live up to an old age.
Diagnosis of the expressed forms of a disease usually does not represent difficulties. Sometimes Fridreykh's ataxy should be differentiated with a hereditary ataxy of Mari, with Marinesku's syndrome — Shegrena, with nek-ry forms of hereditary muscular atrophies (see. Amyotrophy , Marinesku-Shegrena syndrome , Myopathy ).
The forecast concerning life is favorable. However early enough the developing disorders of coordination limit working ability of patients.
Hereditary cerebellar ataxy of Mari — the disease which is transmitted on dominant type (see. Inheritance ). It is characterized by slowly progressing ataxia with defeat of pyramidal system.
On the basis of features of clinic and Mari's inheritance (R. of Marie) in 1893 allocated this form from group family And., described Fried by a Reich.
Pathoanatomical the hypoplasia of a cerebellum, dystrophy of cerebellar and pyramidal ways is noted. The disease begins between 20 and 45 years with disorder of gait. Gradually the ataxy in hands develops, trembling in them is frequent, often note muscular twitchings, the speech becomes slowed down, chanted. Further other symptoms (a ptosis, a strabismus, a nystagmus, sometimes Argayll Robertson's syndrome, decrease in visual acuity, an atrophy of an optic nerve) join. The muscle tone is raised, tendon jerks do not die away, and pathological reflexes amplify, found, paresis of legs is possible. Sensitivity is not upset. There are no anomalies of a skeleton. The disease slowly progresses, quite often is followed by disturbance of memory, gradual decrease in intelligence. Infections, intoxications, physical and mental an overstrain can promote aggravations.
The clinical picture often varies. The forms similar to an ataxy Fried of a Reich, meet a family paraplegia of Shtryumpell (see. Paraplegia ). Genealogical data, a clinical picture and the course of a disease shall be the main criteria of differential diagnosis. The forecast for life favorable. Working ability of patients is limited.
Ataxy teleangiectasia (Louis Bar syndrome) — early progressing cerebellar Ampere-second symmetric teleangiectasias of a conjunctiva and skin, a general disease from group of fakomatoz (see). Published the first message on this disease in 1941 Mr. D. Louis-Bar. Irrespective of it in 1957 Wells and Shai (G. E. Wells, M. of G. Shy) described the observations under the name «progressing family choreoathetosis with skin teleangiectasias». After Boder and Sedgwick's detailed work (E. Boder, R. River of Sedgwick) the disease received the name «ataxy teleangiectasia». Separate observations are published also in the USSR.
Pathoanatomical find an atrophy and a gliosis of a cerebellum, a pale sphere and black substance, multiple teleangiectasias in marrow. Find an aplasia of a thymus, an underdevelopment of an adenohypophysis, bronchiectasias. Assume that the disease is hereditary and is transferred on autosomal recessively type.
The disease begins in the early childhood progressing cerebellar And., quite often combined with the choreoathetoid movements. By 5 — 7 years symmetric teleangiectasias of a conjunctiva, face skin, slowness of movements of eyeglobes, an amimia, a scanning speech, decrease in a muscle tone and tendon jerks develop. G and EEG EM without changes. At a pneumoencephalography found an atrophy of a cerebellum. In blood deficit of immunoglobulins, preferential classes A and M is revealed. Predisposition of these children to infectious diseases, to development of a bronchietasia and malignant new growths is noted. Patients often perish at the age of 12 — 15 years. Proceeding from idea of the leading role of a thymus in formation of immunity, offer treatment of these patients with replanting of a thymus (Yu. M. Lopukhin, L. O. Badalyan and sotr.).
The forecast remains adverse. The lethal outcome is, as a rule, connected with intercurrent infections.
Cerebellar ataxy of various etiology. The ataxy acute cerebellar Leyden and — Vestfalya — a peculiar symptom complex, arises at height or after an acute infection (a sapropyra, malaria, pneumonia, flu) or intoxication (alcohol, barbiturates, Plasmocidum, insectofungicides). Pathoanatomical find dystrophy of cells of bark of a cerebellum, a gear kernel, lower olives. And. extends to the movements of extremities and a trunk, a mimicry and the speech. There are no paralyzes though occasionally found little pyramidal signs. Hypotonia of muscles is expressed. Tendon jerks, sensitivity, function of sphincters remain. And. gradually passes. The residual phenomena are rare. Forecast favorable.
There are several options cerebellar And. not clear etiology with hron, the progressing current. Their hereditary origin is not proved. Apparently, they develop because of inborn vulnerability of coordination under the influence of harmful exogenous factors.
Olivo-ponto-tserebellyarnaya the atrophy is most known in this group. It was described by Dezherin and Toma (J. Dejerine, A. Thomas) in 1900. In 1939 it was in detail studied by N. V. Konovalov.
On opening find the small sizes of a cerebellum, the bridge, olives. Histologically find dystrophy or death of pear-shaped neurocytes (Purkinye's cells) of bark of a cerebellum, own kernels of the bridge and the lower olives, an atrophy of fibers of an average cerebellar leg and olivo-tserebellyarny bunches. The gear kernel, upper legs and kernels of a cerebellum remain. In far come cases met dystrophy of cells of black substance and a lentil kernel. The disease begins gradually at advanced age (50 — 60 years) with emergence of fatigue of legs and unsteadiness of gait. Further the speech (a chant, a dysarthtia) changes, awkwardness of hands joins. An animal force and sensitivity remain kept, tendon jerks raise, separate pathological reflexes are caused. In late stages there are a gipomimiya, constraint, pseudobulbar symptoms and pelvic frustration, the mentality changes. Forecast adverse. Patients usually perish in 3 — 8 years from the joining infection.
Olivo-rubro-tserebellyarnaya the atrophy is described in 1909 by Lezhonnom and Lermitt (M. M. R. Lejonne, G. Lhermitte) on the basis of one kliniko-anatomic observation. The patient of 63 years had a right-hand alternating hemiplegia with paresis of a third cranial nerve at the left. Pathoanatomical the atrophy of bark of a cerebellum, a gear kernel, a dento-rubralny bunch and the lower olive was found. Average cerebellar legs were a little reduced in a size, but kernels of the bridge are not changed. Besides, the old center of a softening crossed a part of decussation of upper cerebellar legs. According to authors, dystrophic changes of a cerebellum did not depend on the center of a softening. At the subsequent observations it is revealed that the main clinical symptoms of a disease are the disorders of coordination which are often combined with hyperkinesias, pyramidal symptoms, changes of cranial nerves; later decrease in intelligence, disturbance of activity of pelvic bodies comes to light. Forecast adverse.
Some more options hron, dystrophies of cerebellar system are described: a pure atrophy of cerebellar bark of Thomas, an olivo-tserebellyarny atrophy of Holmes, a late atrophy of bark of a cerebellum of Mari — Fua — Alazhuanina.
The ataxy can be observed at some inherited disorders of a metabolism (hereditary diseases of exchange of amino acids, a leukodystrophy, etc.).
Treatment depends on an etiology. At hereditary forms A. radical treatment is not developed. At the infectious nature of a disease apply antibiotics, the antiinflammatory, desensibilizing and dehydrational means, vitamins. At residual states and hron, forms A. a different etiology appoint special gymnastics, at sensitive And. — gymnastics by Frenkel's method (purposeful use of sight at autokinesias). In prevention of hereditary forms A. medicogenetic recommendations have essential value.
Physiotherapy exercises at an ataxy, influencing proprioceptors in joints, ligaments, muscles and on a vestibular mechanism, promotes improvement of coordination of movements, preservation of balance during the standing. The LFK following forms are applied: morning hygienic exercises, remedial gymnastics, walking. The remedial gymnastics should be applied systematically, regularly, using special exercises for improvement of coordination of movements and exercise for development of balance under control of sight, and also at the closed eyes. Special exercises are given against the background of fortifying and breathing exercises, in initial positions lying, on all fours, sitting and standing. Preferential active exercises are used, at severe forms — exercises by means of the methodologist, and also the passive movements. Exercises with a gymnastic stick, maces, balls of various size, and also at wall bars are reasonable. Special exercises in balance are carried out on site and during the walking.
Exercises on site: 1) standing, legs are closed (fig. 4, 1—4); 2) legs on one line, one leg ahead other (fig. 4, 5—8); 3) standing on one leg (fig. 4, 9); 4) standing on tiptoe (fig. 4, 10); 5) standing on a sock of one leg (fig. 4,11). These exercises become complicated the simultaneous movement of hands, legs, trunks in various combinations.
Exercises in walking are carried out on the wide area of a support — on a floor, then with reduction of the area of a support, walking on the traces (fig. 4,12) drawn on a floor, in the subsequent — on a gymnastic bench, on its lath. Apply different types of walking, in combination with exercises to hands, legs, a trunk.
Apply exercises with simultaneous action of synergistic muscles and antagonistic muscles to development of coordination of movements. E.g., bending of one extremity and simultaneous extension of another in the joint (fig. 4,13) of the same name; bending of a hand and extension of a leg — more complex combination (fig. 4,74).
Further complication — introduction of various directions during the performance of exercises, napr, one hand is taken aside, and another — up (fig. 4, 15). In a sitting position it is possible to use for hands the direction of upstrokes, and for legs — in the parties (fig. 4,16). Change of a rhythm of movements during the walking is applied. Development of coordination is promoted by exercises with a stick (fig. 4,17), throws and catching of a ball in various ways (fig. 4,18), exercises with maces for swing movements of various amplitude (big, average and small moves and circles) with their various variations. It is necessary to use exercises with small objects, a training in the household movements (fastening of buttons, a lacing, collecting of matches, etc.).
The technique of remedial gymnastics is differentiated depending on a look And. At cerebellar And, it is necessary to alternate exercises for all joints of extremities (the movement of small amplitude) to exercise in the alternate movement (supination — pronation), at first — at slow speed, and then in bystry; to try to obtain the correct position of a trunk during the walking, to train in walking under control of sight and blindly. At sensitive And. special attention is paid to exercises in balance and on development of coordination of movements. At labyrinth And. exercises in balance are especially important.
Duration of the procedure is from 15 to 30 min. daily, on a course of treatment of 25 procedures. Walking is applied not only in procedures of remedial gymnastics, but also as addition to it with gradual increase in distance according to an athletic ability (from 500 m to 1 km). Massage of extremities is reasonable, and at dizziness — massage of a collar zone. On a course of treatment of 12 — 15 procedures.
See also Movements (pathology).
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D. K. Bogorodinsky; N. A. Belaya (to lay down. physical).