Atetoz – the separate form of a subcrustal giperkinez which is characterized by the involuntary slowed-down movements in disteel departments of extremities with change of a muscular tone. Can be a component of clinic of perinatal, hypoxemic, hereditary and degenerate damages of a brain. Atetoz is diagnosed clinically, the basic disease is specified on the basis of a research of the neurologic status, brain blood circulation, cerebral morphology, biochemical indicators of blood. In treatment neuroleptics, benzodiazepines, medicines of a levodopa, holinolitika are used. At severe forms resort to stereotaksichesky interventions.
The term "atetoz" came from the Greek word meaning "moving". The name reflects the characteristic worm-shaped involuntary movements. The synonymous name of a disease – atetoidny appears in literature on neurology. Giperkinezami is called by not purposeful motive acts happening without volition of the patient. As is followed by the slowed-down movements without transition to a tonic spasm, in group of giperkinez it is intermediate between horey with its bystry motive acts and the torsion dystonia which is characterized by spastic hardening in a certain pose. There are boundary forms resistant to an unambiguous differentiation which designate as , atetoidny dystonia. The disease occurs among children and adults, prevalence does not depend on a sex of patients.
Reasons of an atetoz
As Etiofaktorami the hereditary and acquired defeats subcrustal act. At adults the genetic and vascular reasons prevail, children have a perinatal defeat of TsNS. Distinguish from major factors:
- Hereditary diseases. Atetoz is typical for Wilson's disease, Lesh-Nikhana's syndrome, Machado-Joseph's disease, Gentington.
- Violations of cerebral blood circulation. Sharp and chronic ischemia of a brain causes hypoxemic defeat, including the basal kernels entering extrapyramidal system.
- Patrimonial trauma of the newborn. The complicated childbirth, a narrow basin, a pathological current of the period of exile of natural childbirth can become the reason of an intra cranial patrimonial trauma, intranatalny asphyxia with defeat of extrapyramidal structures.
- Fruit hypoxia. Oxygen starvation can be provoked by fetoplatsentarny insufficiency, pre-natal infections, a hemolytic disease. The cerebral structures responsible for regulation of the motive sphere are more sensitive to a hypoxia. Atetoz of hypoxemic genesis enters a clinical picture of cerebral palsy.
- Prematurity. Emergence of violations in the motive sphere is connected with an underdevelopment of the relevant cerebral structures.
- Encephalitis. Atetoz against the background of inflammatory damage of a brain is marked out mainly at children. Among adults it is observed at a rare form of infectious encephalopathy – Kreyttsfeldta-Jacob's disease.
Influences of etiofaktor result from violation in the extrapyramidal system distributing a tone, duration and force of reductions between various muscular groups. The exact pathogenesis is not established. Presumable localization of pathological changes – having a tail and chechevitseobrazny subcrustal kernels. The excessive uncoordinated stimulating impulsation going to muscles on nervous trunks is a consequence of extrapyramidal dysfunction. Under the influence of nervous impulses there are serial muscular contractions which are externally expressed violent motive acts. At the same time remains to unknown that causes emergence of atetoidny form of a giperkinez.
Depending on localization and the nature of movements allocate several clinical forms of pathology:
- Gemiatetoz – unilateral , covering muscles of one half of a body and the person. Happens left-and right-hand.
- Generalized – the bilateral atetoidny movements extending to all muscles. Difficulty of speech function is typical.
- Horeoatetoz – the hyperkinetic syndrome combining signs of an atetoz and trochees. The movements, specific to an atetoz, have peculiar a trochee high speed.
- Atetoidny dystonia – typical is periodically combined with lengthening of the phase of tonic muscular contraction which is expressed in fixing of a pose.
Symptoms of an atetoz
The fundamentals of clinical symptomatology are made by the gradual slow change of a tone of various muscular groups which is externally shown the chaotic creeping motive acts. The movements arise spontaneously, are out of conscious control of the patient. Atetoz is provoked and amplifies at purposeful actions, psychoemotional pressure, regresses at rest, disappears in the period of a dream. Patients specify the certain pose (often lying on a stomach) allowing to reduce the movements.
Involvement of disteel departments of extremities (fingers, brushes, feet), language is typical. In more hard cases extends to proximal departments, cervical and mimic muscles. Forced displacements of fingers are characterized as worm-shaped, in proximal departments of extremities of the movement zmeeobrazny, damage of mimic muscles leads to grimacing. Fixing of the arising statement of fingers/extremity is absent, elaborate poses are smoothly transformed each other. Against the background of an atetoz arbitrary motive acts are complicated, in the expressed cases self-service is impossible. Involvement of face muscles and a neck leads to speech disturbance (an atetoidny dizartriya).
Usually acts as a component of a basic disease, it is combined with symptoms, typical for it. Atetoidny within hereditary pathology, crushing perinatal defeat proceeds against the background of intellectual decrease (the progressing dementia, a delay of mental development). A separate nosology is the double form of a disease demonstrating at children in 11-12 months with the previous muscular hypotonia. It is characterized by a bilateral atetoz, horeoatetozy with distribution on orofastsialny muscles, a trunk, a neck. Proceeds with disorder of swallowing, a dizartriya. In most cases mental abilities are kept.
Atetoidny is diagnosed during survey of the patient. Further researches are necessary for establishment of an etiology and diagnosis of the main disease. The following diagnostic events are held:
- Collecting anamnesis. Time of a debut of a giperkinez, its nature, development, existence of perinatal pathology (at children), a hereditary otyagoshchyonnost is considered.
- Survey of the neurologist. It is directed to a differentiation from a psevdoatetoz, identification of the symptomatology testifying to concrete basic pathology.
- Brain MRT. Visualizes organic changes, zones of ischemia, tumor, the inflammation centers, degenerate processes. In 60% of cases double is followed by existence of the hyper intensive centers in the field of a shell and forward kernels of a thalamus.
- Research of a cerebral blood-groove. It is carried out at suspicion on a vascular etiology. It is carried out by methods of duplex scanning, UZDG, MRT of brain vessels.
- Biochemical blood test. Allows to find dismetabolichesky processes, characteristic of hereditary diseases: decrease in ceruloplasmin of blood, increase in concentration of uric acid, copper in urine.
It is necessary to differentiate from a psevdoatetoz, small trochees, the torsion dystonia. Psevdoatetozny manifestations are result of defeat of the carrying-out highways of deep sensitivity. Psevdoatetoz occurs without musculotonic violations, arises when losing visual control over action, is observed together with sensitive frustration. Small a trochee differs in pristupoobrazny character of a giperkinez, a debut at ten-year age. The torsion dystonia is followed by formation of the pathological twisting poses with fixing in them.
Treatment of an atetoz
Therapy is directed to knocking over of basic pathology. Specific medicines are not developed for treatment of an atetoz. Some effect of use of holinolitik, regulators of dopamine exchange (levodopa), neuroleptics, antikonvulsant of a benzodiazepinovy row is noted. Periodic courses of reception of a pyridoxine, physiotherapy exercises are recommended. According to indications apply glutamic acid to stimulation of intellectual development.
Heavy can act as the indication to stereotaksichesky operation – destruction of a ventrolateralny kernel of the thalamus providing communication of extrapyramidal educations. Surgical intervention allows to reduce expressiveness of a giperkinez, but it is dangerous by development of a psevdobulbarny syndrome, at a double atetoz aggravates disorders of swallowing. An experimental technique of treatment is the deep stimulation of a brain representing the braking impact on extrapyramidal structures.
Forecast and prevention
The outcome depends on the main pathology. The patients who got sick in the childhood, despite the expressed motive frustration, can live to a ripe old age. The serious forecast has hereditarily caused atetoidny , proceeding with the progressing degenerate changes of TsNS. Double it is characterized by a stable current, partial compensation of motive frustration is possible with age. Prevention includes measures for the prevention of provocative factors: adequate conducting pregnancy and childbirth, timely treatment of violations of brain haemo dynamics, neuroinfections.