ShAYa — DRYOYDZHERA SINDROM
(G. M of Shy, amer. neuropathologist, 1919 — 1967; G. A. Drager, amer. neuropathologist, sort. in 1917) — the degenerative damage of the central nervous system of not clear etiology which is shown orthostatic arterial hypotension in a combination at different stages of a disease to symptoms of parkinsonism, disturbance of sweating (anhidrosis), disorder of function of pelvic bodies and other neurologic disturbances. The disease meets seldom.
To Sh.'s description — D. to page knew a rare disease — idiopathic orthostatic hypotension, the leading manifestation to-rogo is postural falling of the ABP of not clear genesis with heavy orthostatic circulatory disturbances. In 1960 Shai and Dreydzher described two cases of such hypotension, having paid attention to a combination it with symptoms of widespread organic lesion of c. N of page — rigidity and trembling of extremities, loss of coordination of movements, an atony of sphincters of pelvic bodies, disturbances of sweating. Since these disturbances preceded the expressed orthostatic hypotension, Shai and Dreydzher assumed that organic changes of c. N of page at this disease are primary, but are not caused by orthostatic disorders of blood supply of a brain (see. Orthostatic changes of blood circulation). However the alternating hypoxia of a brain can gain additional pathogenetic value in process of a course of a disease. In a crust, time idiopathic orthostatic hypotension and Sh. — D. villages consider as options of display of uniform pathology and both terms quite often use as synonyms.
The etiology is not known. The toxic nature of a degeneration of nervous structures is improbable though the changes observed at this disease are similar to the changes arising at hron. poisoning with nek-ry metals. There is an observation of a family form Sh. — D. of page with a dominant mode of inheritance. Development of a syndrome of Shai — Dreydzhera is described at cancer of an almond as (presumably) paraneo-plastic process (see. Para-neoplastic syndromes), what does not exclude a possible role in its origin of autoimmune disorders.
The pathogeny is studied insufficiently. Morfol. substrate of parkinsonism at III. — Of page are degenera-tivnys changes of cells of black substance. Aminoff (M. of J. Aminoff, 1973), etc. believe that their formations at Sh. — D. are the cornerstone of page preferential striatal and pallidarny defeats. However the pathogeny of parkinsonism at Sh. — D. remains to page not clear and differs from Parkinson, that at a disease (see. Trembling paralysis). Cholinolytic substances (see) do not weaken displays of parkinsonism at this syndrome, and L-DOFA in some cases even worsens somatic nevrol. frustration.
ShAYa - DREYDZHERA the SYNDROME 371
is Rather clear genesis of orthostatic hypotension, communication a cut with loss of sympathetic regulation of a hemodynamics is proved not only pathoanatomical data, but also svedeniyami*o to concentration of noradrenaline in blood of patients. At healthy faces upon transition from horizontal position to the vertical maintenance of noradrenaline increases in blood on average almost in 1V2 of time and it is caused by orthostatic activation of a tone of sympathetic nerves. At patients with Sh. — D. the page of such gain of concentration of noradrenaline in an ortostaza is not observed (it even decreases) while at horizontal position of a body it is close to normal (only in nek-ry cases decrease in concentration of noradrenaline in blood and cerebrospinal liquid is found). The hemodynamics and tone of peripheral vessels in horizontal position at patients significantly do not differ from norm, but in vertical position there is no increase (the so-called fixed speed) observed at healthy serdtsebiyeniye and are sharply reduced or there are no vascular reactions, the most dependent on sympathetic regulation (see. Orthostatic changes of blood circulation). It leads to the decrease in cordial emission which is not compensated by growth of peripheric vascular resistance therefore at patients with Shai's syndrome — Dreydzhera upon transition from horizontal position in vertical abruptly falls both systolic, and diastolic the ABP inflow of blood to a brain decreases and owing to his diffusion ischemia the syncope develops.
Decrease in a tone of sympathetic nerves is shown by hypofunction of sweat glands, weakening of sweating at temperature increase, and also substantial increase of sensitivity and - adrenore-tseptorov blood vessels to noradrenaline. The last is shown by heavy hypertensive reactions even at very slow intravenous administration by the patient of noradrenaline (with a speed only 0,5 or even 0,1 mkg! kg in 1 min.). As L-DOFA at patients with Sh. — D. the page has no central hypotensive effect, quite often raising the ABP (peripheral action), it is possible to draw a conclusion
on dissociation of sympathetic bonds between a brain and the periphery.
Data on a pathogeny of others nevrol. disturbances are limited to the instruction on their communication with frustration of a somatic and vegetative innervation, a degeneration of cells in different structures of c. N of page, including a dorsal kernel of a vagus nerve, and also in sympathetic gangliya.
Pathological anatomy. At a pathoanatomical research of change, specific to III. — Of page, macroscopically do not come to light. In nek-ry cases the hypoplasia of adrenal glands with a focal hyperplasia of cortical substance in the form of small adenomas is noted.
The main changes find in c. N of page. Histologically the degeneration of neurons of a shell, black substance, the lower olives, a kernel having a tail, degenerative changes in a cerebellum, the varoliyevy bridge, strionigralny, stria-topallidarny and olivopontotsere-bellyarny paths, an interstitial brain, considerable reduction of number of cells of Purkinye, patol come to light. changes of cells of a ventral column, intermediolateralny substance of a spinal cord and cells of columns of Clark, change of neurons vegetative gangliyev. Glial reaction is expressed, but signs of an inflammation are absent. With the greatest constancy reduction of cells in intramediolateralny substance of a spinal cord comes to light. According to Potye (J. Page of Potier, 1969), the degeneration in side horns of a spinal cord is more expressed, than in sympathetic gangliya.
Clinical picture. Highway — D. is observed by page at persons of both sexes, most often at the age of 50 — 70 years. The first addresses to the doctor are connected, as a rule, with emergence of orthostatic hemodynamic frustration. In the beginning patients complain of weakness, fatigue, instability of gait. In the subsequent long standing becomes impossible because of «dizzinesses», blackout, a presentiment of a loss of consciousness. Then weight of orthostatic disturbances quickly progresses, often there are orthostatic faints, to-rye become dominating and define weight of a condition of the patient irrespective of degree accompanying nevrol. disturbances. Within several months the time slice from the moment of transition of the patient from horizontal position in vertical before developing of a faint is reduced to several minutes, in the subsequent — up to 1 min. and less. In hard cases a syncope (see) can arise even in a sitting position in a bed, passive transfer of the patient in vertical position threatens with development of a deep faint with heavy ischemia of a brain.
In the anamnesis at such patients quite often reveal previous (for several months or in 1 — 3 years prior to emergence of orthostatic disturbances) other symptoms of a disease — most often decrease in a libido at women, impotence at men, vegetative frustration (a dysuria, disturbances of sweating, function of intestines). At nek-ry patients of display of a disease within several years are limited to the progressing orthostatic hemodynamic frustration described above without other deviations in nevrol. status. In the majority of cases in several months after emergence of orthostatic hemodynamic disturbances or along with their emergence (is more rare earlier) rigidity and trembling of muscles of extremities, disorders of coordination are found.
According to Shirger and Thomas (A. Schirger, J. E. Thomas, 1976), based on the analysis of 57 observations, frequency somatic nevrol. disturbances at Sh. — D. the page reaches 85%. The developed displays of parkinsonism are observed at 60% of patients and not always correspond to the akinetorigidny form of parkinsonism which was considered characteristic of ILL — D. of page. From extrapyramidal signs more than at a half of patients the amimia, muscle tension of extremities are noted and approximately 1/3 patients have a monotonous voice, trembling of extremities. Kortiko-bul-barnye disturbances (the sucking reflex, is more rare than a dysphagia) appear approximately at Vs patients. Rather often (approximately in 40% of cases) symptoms of damage of a cerebellum in the form of intentsionny trembling (see), an ataxic gate (see), dysarthtias are found (see). Are occasionally observed Bernard's syndrome — Horner (see Bernard — Horner a syndrome), an anisocoria (see), a hypesthesia (see Anesthesia). Vegetative frustration are most often shown by various disturbances of an urination (at 65% of patients), including enuresis (see the Incontience of urine), and also functions of intestines, including an atony of a sphincter of a rectum.
EEG at patients with Shai's syndrome — Dreydzhera has no specific features; usually the diffusion dizritmiya, sometimes slow activity comes to light.
In the absence of a cerebellar ataxy (see) sick often try to adapt gait and poses of a body to orthostatic changes of a hemodynamics. They move the wide, directed slightly aside ambles on the legs which are slightly bent in knees, having low inclined a trunk forward and having hung the head (a pose of the skater). To prolong time of stay in vertical position, patients accept intense, sometimes fancy poses — quite often cross legs, squeezing a strong muscle tension of a vein of shins and hips that increases venous return of blood to heart.
At inspection of patients in horizontal position of essential changes of activity of internals (except connected with associated diseases, complications or caused by disorders of nervous control) it is not noted: The ABP normal, is sometimes raised or moderately reduced; its instability at hourly and daily measurement is characteristic. During the conducting orthostatic tests (see) reaction of the ABP and pulse corresponds to asimpatiko-tonic type of disturbances of orthostatic regulation — both systolic, and diastolic the ABP in the absence of changes of pulse rate or his insignificant increase quickly falls, arises and quickly «alabaster» pallor of head skin progresses, persons, necks, an upper half of a trunk, the look of the patient becomes unstable, there comes the syncope and in the absence of assistance of the patient can fall. In position of the patient lying consciousness gradually is returned, and within several hours after that the patient notes the expressed weakness, fatigue, drowsiness.
The complications observed at Sh. — D. of page, are most often connected with orthostatic hemodynamic frustration:
injuries owing to falling at a faint are possible, frustration of mentality and intelligence as a result of the alternating ischemia of a cerebral cortex, at elderly people ischemic strokes sometimes develop. Dysfunctions of bodies of uric system often are complicated by cystitis, a pyelitis.
The diagnosis at a prematurity of the orthostatic haemo dynamic frustration caused by deficit of sympathetic activity does not cause difficulties. Sometimes there is a need for an exception of organic occlusion of an internal carotid artery (see. Carotid arteries) and carotid sinus syndrome. In cases when orthostatic arterial hypotension develops already against the background of the expressed somatic neurologic disturbances, early diagnosis is difficult.
The differential diagnosis depending on a syndrome nevrol. frustration carry out with a disease of Par^ of a kinson, effects of encephalitis, secondary degenerative changes of c. N of page at various diseases and intoxications. The vegetative frustration (an anhidrosis, enuresis, an atony of a sphincter of a rectum) developing at parkinsonism arise later, than at Sh. — D. pages also are considerably less expressed. Delay and inertness of mental processes are characteristic of postencephalitic parkinsonism hypersalivation (see Salivation), a hyperhidrosis (see), a nictitating spasm (see). The differential diagnosis with degenerative changes of c. the N of page of toxic genesis is based on careful a toksi-stake. the anamnesis and identification of signs of defeat of peripheral nerves, a cut it is not peculiar to Shai's syndrome — Dreydzhera.
Treatment consists in correction of the heaviest displays of a disease — orthostatic frustration of a hemodynamics and parkinsonism. Apply hard bandaging of extremities, a pelvic girdle and a stomach to restriction of speed of development of orthostatic arterial hypotension. From pharmakol. means is more often than others synthetic fluorinated corticosteroids, the mechanism of action are effective to-rykh it is not clear (the effect does not match increase in volume of blood and a delay of salts), and digidri-rovanny alkaloids of an ergot (di hydroergotamine or dihydroergotoxin in or in the form of intramuscular injections). Use and - adre-nomimetikov is less effective and accompanied by developing of arterial hypertension in horizontal position of the patient and danger of development of complications. In some cases improve the portability of vertical position MAO inhibitors, tyramine enriched with salt a diet a little; the positive take of use of indometacin is described. A. J. Moss, etc. (1980) reported about considerable improvement of a condition of the patient after implantation of the pacemaker setting the frequency of reductions of auricles 100 in 1 min.
Parkinsonism at Sh. — D. of page rezistenten to use of L-DOFA and hardly gives in to correction. There are messages on efficiency of the combined use of Cyclodolum and a dibenzipin.
Forecast. Usually the disease quickly progresses. Relative stabilization of manifestations of Sh. — D. by page for 3 — 5 years is in rare instances observed. The lethal outcome at most of patients can come in
7 — 8 years after emergence of the first symptoms.
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page 119, 1978; Beds a nickname about va S. M. and A. S's Adam's apples. To morphology of a syndrome of Shai — Dreydzhera, Arkh. patol., t. 41, century 10, page 43, 1979; A m i n about f f M. J. and. lake of Levodopa therapy for parkinsonism in the Shy — Drager syndrome, J. Neurol., Neurosurg. Psychiat., v. 36, p.
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