HYPERKINESIAS (grech, hyper-+ kinesis the movement) — the excess involuntary movements which more often are shown reduction of face muscles, a trunk or extremities are more rare — reduction of muscles of a throat, a soft palate, language, outside muscles of eyes. Happen various depending on an etiology, a pathogeny, character and localization patol, process.
G. arises often at encephalitis: epidemic (parkinsonism, the torsion dystonia, athetosis, etc.), tick-borne (kozhevnikovsky epilepsy), rheumatic (hysterical chorea), leukoencephalitis and other forms of encephalitis. Etiol, factors at G. are also vascular damages of a brain, a craniocereberal injury, tumors of a brain, intoxication, hereditary diseases (trembling paralysis, a myoclonus epilepsy, a chorea of Gentington, etc.).
G. arise more often at defeat of extrapyramidal system: a striate body (striatum), a pale sphere (pallidum) usually in combination with black substance of a mesencephalon (pallido-nigralny system), a thalamus and its bonds, a subthalamic kernel, gear kernel of a cerebellum, a red kernel and their bonds (olivo-dentato-rubralnoy systems). Disturbance of functions of system of a feed-back between bark and a subcortex matters.
Value of a cerebral cortex in G.'s origin comes to light at the local convulsive twitchings arising at irritation of a motive zone (field 4) a tumor, a posttraumatic cyst, etc., and during the developing of local cortical spasms at kozhevnikovsky epilepsy. At disturbance of an afferent cortical innervation of one hemisphere (defeat of talamo-cortical bonds) there is a hemochorea. In G.'s pathogeny it is necessary to consider formation of a dominant in subcrustal departments of a brain, impact on which is exerted by the arriving afferent impulses, and also switching off of the regulating role of bark. The dream and rest exert the braking impact on G., emotions strengthen G. Talamus plays a large role along with cortical and subcortical mechanisms in emergence and G.'s strengthening at nervousness, emotions, a stress. The reticular formation of a brain trunk can promote development and G.'s strengthening (tremorogenny influence), and also their braking.
Extrapyramidal G. are combined with change of a tone of muscles and vegetative dysfunctions. Localization patol, process is not unconditional for G.'s manifestation: at the same localization (a thalamus, a striate body) there can be various manifestations of excess movements. Extrapyramidal G. happen difficult and sometimes include components of several forms, napr, the choreoathetosis can be combined with elements of the torsion spasm. Not only localization of defeat, but also extent of defeat, quantity of the involved cells and their bonds matters. In G.'s pathogeny factors matter biochemical: disturbance of exchange of catecholamines, in particular change of content of dopamine in a striate body and acetylcholine which is in interaction with it.
A clinical picture
Hyperkinesias of an extrapyramidal origin differ various a wedge, manifestations, frequent in pretentiousness, affectation, a combination to change of a muscle tone (decrease or increase on plastic type).
The tremor (trembling) takes all body or its separate parts. It is shown variously: rhythmical or spasmodic, constant or paroxysmal, static (at parkinsonism, essential trembling, etc.), postural (at multiple sclerosis, alcoholism, etc.), intentsionny (at essential trembling, multiple sclerosis, a leukoencephalitis, etc.), localized (in muscles of a hand, a neck, a leg) or widespread in groups of muscles (a hand, a neck, a trunk, a leg). The tremor is observed at postencephalitic, atherosclerotic, posttraumatic parkinsonism; trembling paralysis, at intoxications (an alcoholic poisoning, mercury, cocaine, aminazine, etc., at a hyperthyroidism, multiple sclerosis, collagenoses, at vascular damages of a brain, after a contusion, at a general paralysis, etc., at senile trembling; besides, essential trembling, or a hereditary and family tremor, sometimes in form of motion of the head («yes», «is not present»), the emotsiogenny tremor arising at disorders and after a mental injury are possible. Trembling at parkinsonism in the form of «driving of pills». At hepatocerebral dystrophy trembling happens large amplitude, reminds the movement of a wing of a bird at take-off. Peculiar G. are observed at Pelitseus's disease — Mertsbakhera, Gallervorden's leukodystrophy — Shpattsa.
Myoclonias the — the scattered, chaotic, bystry and spasmodic clonic reductions of muscles, sometimes single, but constantly renewing.
Tics — myoclonic type G. (symptomatic myoclonias) which are shown variously: twitching of mimic muscles (fig. 1), gesticulation or bystry starts and other stereotypic motive acts (smacking, prishchurivaniye of eyes etc.).
Myoclonias usually cause motive effect, but can be observed in the form of reduction of the isolated, not synergistic muscles or separate muscle bundles without movement of an extremity. Such type of myoclonias is called a paramyoclonus, or a myoclonia like Fridreykh — Unferrikhta. Mioklonusepilepsiya (Unferrikhta — Lundborga) is characterized by a combination of a generalized myoclonia to epileptic seizures. It arises at damage of a thalamus and its bonds, black substance, gear kernels of a cerebellum and rubrothalamic bonds. Myoclonias arise at an epidemic and tick-borne encephalitis, vascular damages of a cerebellum, a brain trunk, etc.
Tserebellyarnaya a myoclonic dyssynergia, or Hunt's myoclonia, happens widespread, is characterized by a combination of a myoclonia in several muscles of extremities, amplifies at autokinesias and emotions, is combined with an ataxy as a result of defeat of gear kernels of a cerebellum, at Krom regular influence on a red kernel and an olive drops out. Myoclonias in the form of widespread fibrillar twitchings are observed at Morvan's myoclonia.
The special form is made by infantile myoclonic encephalopathy, or Uest's syndrome. Myoclonias at cerebellar dyssynergias happen widespread, amplify at autokinesias and emotions. In a pathogeny of these myoclonias significance is attached to defeat of the gear kernels of a cerebellum influencing a red kernel and an olive. Myoclonic G. is observed at encephalitis (epidemic, tick-borne), Van-Bogart's leukoencephalitis, at Kreyttsfeld's disease — Jacoba, Krabbe's leukodystrophy.
Rhythmic myoclonias (a rhythmic myoclonia) represent the twitchings localized in certain muscles having wavy (undulating) character with a constant rhythm (from 6 — 10 to 80 — 100 reductions in 1 min.).
The localized myoclonias are observed in face muscles, language, a soft palate, a throat, an abdominal wall, etc. The localized myoclonias are connected with defeat of an olive of a myelencephalon, gear kernel of a cerebellum and a red kernel and their bonds. Mioritm connect preferential with defeat of the lower olive.
The spastic wryneck is a local torsion and dystonic syndrome which is shown by the tonic data of cervical muscles causing turn of the head aside (fig. 2). The spastic wryneck differs from the reflex wryneck arising in response to pain stimulations of a mastoid, a parotid gland, cervical roots, etc. The spastic wryneck results from defeat strio-padlidarny system.
The choreic hyperkinesia is shown variously depending on a form of a disease, consists of bystry: the scattered, spasmodic various movements of muscular groups of extremities, the person. Amplifies at nervousness, decreases at rest and stops in a dream. At a chorea standing, walking, the speech and phonation can be broken. Choreic G. is followed by sharp hypotonia of muscles.
Choreic G.'s etiology can be various: a hysterical chorea of Sydenham at rheumatism, a chorea of pregnant women, a trochee at encephalitis, the chronic progressing chorea of Gentington (see. Gentington chorea ), a senile atherosclerotic chorea, an electric chorea of Genokh — Berzherona (see. Chorea ), arising after a brain injury and a tick-borne encephalitis.
The polycythemic chorea in combination with gangrene of fingers is described (N. K. Bogolepov). Choreic G. quite often arises at disturbance of cerebral circulation (at the centers in the field of a mesencephalon and in the field of subcrustal nodes), quite often at the same time the hemochorea (fig. 3 and 4) is observed. At children the hemochorea is observed at a cerebral hemiplegia. Amplifies at pain stimulations and emotions.
The athetosis — is shown by the slow, worm-shaped, continuing continuously tonic movements (spasm) of fingers, brushes, feet, face muscles causing elaborate provisions of extremities. Athetosis (see) happens unilateral or bilateral.
The choreoathetosis arises at defeat of talamo-striatal system, at periaksialny encephalitis of Shilder, at a subacute leukoencephalitis of Van-Bogart and hyperkinetic encephalitis of Uzunov — Bozhinova, «a tetanoidny chorea» of Govers at disturbances of cerebral circulation. It is clinically characterized by a combination of atetoidny and choreic movements.
The hemiballism consists of the bystry «broskovy» rotary wide motions of a hand and leg on the party opposite to the center of defeat. It is usually combined with a hemihypotonia, change of position of a trunk and disturbance of gait. Amplifies under the influence of emotions. Arises at defeat of a subthalamic kernel (disturbance of cerebral circulation). At a hemiballism the tone of muscles is broken. Arises in proximal departments of a top and bottom extremity.
Paraballizm — the broskovy movements in both upper extremities at bilateral vascular defeat of talamo-subthalamic system.
The torsion dystonia (a lordotic dysbasia) is shown by a tonic spasm of muscles, an unnatural pose of a trunk which lead to unnatural elaborate position of a trunk (rotation around a longitudinal axis), extremities, the head. The torsion or deforming dystonia is connected with the wrong distribution of a tone of muscles, especially trunks and necks that causes a rachiocampsis and a wryneck. Amplifies during the walking, can stop at run and elaboration of compensatory receptions. Join tonic spasms clonic, and position of a trunk at the torsion dystonia (see) changes.
Giperalgichesky clonic hyperkinesias arise at damage of a thalamus and its bonds at disturbances of cerebral circulation, proceed paroksizmalno during the drawing nociceptive irritations., arising in the irritated extremities, are shown in the form of a cycle of consecutive movements (counterlateral giperalgichesky G.). In extremities opposite to the pathological center are observed in the early period of disturbance of blood circulation in talamo-striatal system.
Hyperkinesias of a cortical origin. Cortical (jacksonian) epilepsy — paroksizmalno the arising clonic twitchings of muscles on the side of a body opposite to the center of irritation of bark of motive area (a front central crinkle, the field 4 on Brodmanna). At the beginning of an attack of twitching are noted in certain departments of the person or one extremity, in the subsequent usually accept generalized character (see. Jacksonian epilepsy).
Kozhevnikovsky epilepsy, or long local cortical spasms (epilepsia partialis continua), is shown by clonic twitchings of muscular groups in the extremities opposite to the center of defeat which pass with times into a generalized epileptic seizure (see Kozhevnikovsky epilepsy).
Gemitoniya (V. M. Bekhterev) is shown by clonic reductions of paretichny extremities during recovery of motive functions after an apoplexy and is connected, apparently, with irritation of a pyramidal way (see also tab.).
Spasms and spasms of muscles can be observed as components G. or sporadic. They differ on prevalence (in narrow group synergy of the operating muscles and extended), to constancy (are shown continuously or arise at ekstero-, intero-or proprioceptive irritations, nervousness and physical. tension).
Spasms are various on a pathogeny and localization patol, process (in bark, in subcrustal educations, a diencephalon) and to manifestations. Matter also dependence on disturbance biochemical, a constant and hormonal balance (a spasm at swimmers as a result of a hypercapnia, at an alkalosis, tetanic spasms).
The Intentsionny spasm of Ryulf arises suddenly in muscles of an extremity, the cut of the patient intends to execute the unprepared movement. Beginning in narrow group of muscles, the spasm extends further to other extremities and can pass into an epileptic convulsive seizure, but without loss of consciousness. The etiology of a spasm Ryulfa is definitely not established, it was noted at neuroinfections or in the form of a family disease (see. Ryulfa intentsionny spasm ).
A tonic oculogyric spasm, at a cut are periodically looked away up and the head is thrown back back, observed at parkinsonism. In genesis of a spasm attach significance to defeat of striatal system, however observations show that an essential role is played by a reticular formation of a brain trunk and especially its mezentsefalny department (see. Look paralysis, spasm ).
Nictitating spasm — the paroxysmal involuntary reduction of circular muscles of both eyes (fig. 5) which is supplemented then turn of the head in the parties and sometimes convergence of eyes (see. Nictitating spasm ). Can be initial manifestation of a paraspasm. It is observed at patients with encephalitis.
The spasm of a diaphragm is shown by a hiccups (an incidental hiccups) at patients with encephalitis as a result of disturbance of functions of a reticular formation of a brain trunk.
The front paraspasm is observed at patients with atherosclerosis, consists of bilateral toniko-clonic spasms of facial muscles (fig. 6). It is noted also at encephalitic processes in the field of subcrustal nodes. Myotonia of the person are combined with a spasm of cervical muscles.
A front hemispasm — pristupoobrazny myotonia of a half of the face on the party of defeat (see. Hemispasm ). Arises at a prelum of a facial nerve in the fallopian channel, an arachnoiditis of a back cranial pole.
The painful spasm of facial muscles arises during an attack of an epileptiform neuralgia.
Professional spasms arise during the performance of professional movements: a writer's cramp at pianists, clerks, violinists, a spasm of big and index fingers at tailors, typists, milkmaids, hairdressers, spasms of a circular muscle of an eye at watchmakers, spasms of legs at ballerinas, etc. In some cases for reduction of a writer's cramp compensatory receptions (fig. 7) are used.
Carry epileptic spasms to incidental G. (cortical, diencephalic, mezentsefalny), not -' any capture of the objects put in a brush (a symptom of involuntary capture), closing or opening of a mouth, an eye at a distantny impulsation.
is Appointed by the drugs influencing Extrapyramidal educations (Cyclodolum, artan, Parkopanum, Romparkinum, Ridinolum, Norakinum, Tropacinum, Bellazonum, Dinezinum, l-dofa), sedative (bromides, a root of a valerian, etc.), antihistaminic drugs (Dimedrol, Suprastinum, tavegil, Pipolphenum, etc.), the means influencing metabolism (glutaminic to - that, scale of l it, etc.), polyvitamins, hypnotic drugs (at sleeplessness). The psychotherapy is in certain cases shown. Surely etiol, treatment and actions for rehabilitation. According to indications make neurosurgical (stereotaxic) thalamus operations a pale sphere, an intermediate kernel of Kakhal and an upper longitudinal bunch (at a wryneck), a tractotomy, etc.
the Forecast depends on a basic disease.
Table. The Kliniko-morfologichesky characteristic of the main hyperkinesias *
- Materials of the table have the insignificant discrepancies with
the text of article caused by some distinction in approaches to classification and terminology.
- The term «symptomatic» is used for designation
of a hyperkinesia which appears at various organic processes in c. the N of page
- Should mean emergence in some cases
of a reflex spastic wryneck at osteochondrosis and cervical radiculitis which forecast is favorable.
- This group can include more rare forms
of a chorea: a postapoplectic chorea (hemochorea), the chorea caused by neuroinfections (Ekonomo's encephalitis, a leukoencephalitis, etc.), a chorea of pregnant women , etc.
Bibliography: Antonov I. P. ishan-K about G. G. Hyperkinesias at children, Minsk, 1975, bibliogr.; Badalyan L. O. Children's neurology, page 52, M., 1975; B of e x - t of e p e in and N. P., etc. Physiology and pathophysiology of deep structures of a brain of the person, L. — M, 1967; Bogolepov N. K. Disturbances of motive functions at vascular damages of a brain, page 164, M., 1953; about N e, Clinical lectures on neuropathology, page 48, M., 1971; Davidenkov G. H. Clinical lectures on nervous diseases, page 5, M., 1956; The Multivolume guide to neurology, under the editorship of N. I. Gra-shchenkov, t. 2, with: 133, M., 1962; Petelin L. S. Extrapyramidal hyperkinesias, M., 1970; P ah n S. Differential diagnosis of tremors, Med. Clin. N. Amer., v „56, p, 1363, 1972; Handbook of clinical neurology, ed. by P. I. Vinken a. G. W. Bru-yn, v. l, p. 169,279, Amsterdam — N. Y., 1969.
H. K. Bogolepov, G. H. Davidenkov; L. S. Petelin (tab).