TREMBLING PARALYSIS

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TREMBLING PARALYSIS (paralysis agitans; synonym Parkinson's disease) — slowly progressing disease of a brain which is shown trembling, extrapyramidal rigidity, an akineziya.

Dative is described as an independent disease in 1817 of the English doctor J. Parkinson. Approximately in hundred years after J. Parkinson's description the attention of researchers was drawn to this disease in connection with the works devoted epid, to encephalitis again. Hron, a form epid, encephalitis received the name of «parkinsonism» owing to similarity its wedge, pictures with clinic of Dative.

The research K was of great importance for studying of Dative. N Tretjyakova (1919), shown a role of black substance as morfol, substrate, in Krom occur the changes leading to development of a syndrome of parkinsonism. Further researches of functions of other extrapyramidal educations at Dative went deep. Results of these researches formed a theoretical basis for development of surgical treatment of Dative.

The age of the beginning of a disease fluctuates between 50 — 60 years. Men are ill more often than women.

Etiology it is unknown. There are diseases given about the hereditary nature. Allan (W. Allan, 1937), Myenes (H. Mjones, 1949), S. N. Davidenkov (1959), Pratt (J. Pratt, 1967), M. Atadzhanov (1973), etc. give family trees with presence of patients in 3 — 4 generations. The analysis indicates them a dominant mode of inheritance with low penetrance that it is shown by various expressiveness of a disease in different generations and so-called admissions of generations. S. N. Davidenkov considered that variations of penetrance and the «admissions of generation» inherent to many neurotropic genes, at Dative can depend also on a variation of age of the beginning of a disease since the patient can not live up to age when at it the disease would begin. The recessive mode of inheritance was noted in single observations. Hereditary transfer of a disease is confirmed by descriptions And. B. Weinstock (1973) monozygotic twins, concordant on Dative. However the hereditary nature of a disease admits not all. Zh. Sharko (1889), V. V. Dekhterev (1927), H. N. Anosov (1960), E. I. Kandel (1965) deny a hereditary origin of Dative, describing sporadic cases.

A pathogeny

the Main symptoms of a disease are caused by disorder of function of pallidonigroretikulyarny structures with disturbance of complex innervatsionny system of the ring bonds which are turning on the central and peripheral self-regulating mechanisms. The big part in an origin of trembling is assigned to functional disturbance of gamma system of a spinal cord. Methods of a histochemistry using a submicroscopy revealed features of distribution of biogenic amines in a brain. It is established that deficit of dopamine in subcrustal structures leads to development of extrapyramidal rigidity.

Pathological anatomy

Morfol, changes at Dative consist in slowly progressing system degeneration of ganglionic cells and myelin fibers with the subsequent growth of gliozny elements. Changes are localized in subcrustal nodes, a visual hillock and especially in black substance where, besides, find hyaline inclusions (Levi's little body) which consider as a special type of cellular pathology of involutional character. Also reticular formation and its efferent bonds with motor-neurons of front horns of a spinal cord is surprised.

The clinical picture

is shown by Dative trembling (see), the extrapyramidal rigidity resulting in the general constraint of the patient bradykinesia (see). J. Parkinson considered trembling the main symptom of a disease. However Parkinson's disease can proceed also without trembling.

Fig. 1. The oscillographic curves of a tremor observed at patients with trembling paralysis: 1 — harmonious; 2 — amplitude-modulated; 3 — the polyharmonic.
Fig. 2. A characteristic pose of «applicant» of the patient with trembling paralysis.

Trembling of hands at Dative is characterized by the small rhythmical movements reminding «rolling pills». It happens constant, is observed at rest, increases at nervousness, decreases at autokinesias and stops in a dream. Its frequency fluctuates from 2 to 8 in 1 sec. At oscillographic record of trembling distinguish the following types of curves: harmonious with a constant amplitude, amplitude-modulated with the changing amplitude, the polyharmonic with the changing form (fig. 1). Trembling begins with one extremity (a thicket upper), then passes to another, becomes symmetric, extends also to the head, a mandible, language and lips. Rigidity covers all muscular groups, both synergists, and antagonists. The bradykinesia which is shown constraint, a gipomimiya, slow rate of movements develops. The symptom of «cogwheel» — the rhythmic tolchkoobrazny resistance at passive bending and extension of extremities connected with increase in a muscle tone is characteristic. Also rare blinking, the fading speech, change of handwriting is noted (micrography), lack of consensual movements of hands during the walking (one of the earliest symptoms). Gradually the akineziya develops (see. Movements, pathology ), against the background of which there are peculiar motive phenomena in the form of propulsion, the lateropulsions and retropulsions which are shown a tendency of the patient to continue the movement in the direction given it (forward, in the parties, back). Depending on dominance of these or those motive frustration distinguish trembling, rigid or it is rigid - trembling forms of a disease. The bent pose — the pose of «applicant» (fig. 2) shuffling the tripping gait is typical. Vegetative frustration in the form of a xeroderma can be preferential. Memory and intelligence are usually kept.

The diagnosis

the Diagnosis is made on the basis of the anamnesis and characteristic by a wedge, pictures. At differential diagnosis of Dative it is necessary to distinguish from parkinsonism (see) at infectious or vascular damages of a brain. The Rapid and malignant current, existence of headaches, dizzinesses, pseudobulbar symptoms, dysmnesias is peculiar to parkinsonism of a vascular origin which is expression of diffusion cerebral atherosclerosis. Earlier beginning, hypersalivation, a hyperhidrosis, a nictitating spasm, delay and inertness of mental processes is characteristic of postencephalitic parkinsonism. The symptom complexes developing at various intoxications — manganese, carbon monoxide, aminazine are similar to parkinsonism, etc.

Treatment

Treatment is carried out for many years. A dosage of drugs, schemes of treatment and duration are defined individually in each case. Use the drugs relating to group of the central cholinolytics: Cyclodolum (artan, Romparkinum, Parkopanum) on 0,001 — 0,002 g 3 times a day, Tropacinum on 0,01 g 2 times a day, at good tolerance a daily dose increases to 0,075 — 0,1 g. Also drugs of a fenotiazinovy row with antihistaminic properties are shown: Dinezinum, isopromethazine on 0,05 g, beginning s1 and to 4 — 5 times a day. In 10 days establish a dose on 0,25 g 2 times a day. The special place in treatment of Dative belongs to the drug L-DOFA — the predecessor of dopamine capable to pass through a blood-brain barrier and having replaceable effect. Appoint it on 0,1 g inside during food 3 times a day with the subsequent gradual increase in a daily dose. The optimum daily dose is defined individually and makes from 1,5 to 6 g. At treatment of Dative Midantanum (amantadin) in a dose on 100 mg 2 — 4 times a day which, apparently, also influences exchange of dopamine has similar effect.

Use of the specified drugs demands care at stenocardia, glaucoma, diseases of a liver, kidneys, blood, a diabetes mellitus and convulsive attacks.

In the absence of effect of medicamentous therapy stereotaxic operation can be used (see. Stereotaxic neurosurgery ) — destruction of a ventrolateralny kernel of a thalamus.

Along with drug treatment are recommended to sick Dative physical. exercises and whenever possible continuation of professional activity.

The forecast

the Forecast concerning life favorable, an absolute recovery does not come. Drug treatment and stereotaxic operations usually improve a condition of patients.


Bibliography: Dekhterev V. V. Trembling paralysis (paralysis agitans), M., 1927; Davidenkov S. N. Immediate tasks in studying of hereditary diseases of a nervous system, Vestn. USSR Academy of Medical Sciences, No. 5, page 61, 1959; Kandel E. I. Parkinsonism and its surgical treatment, M., 1965, bibliogr.; The multivolume guide to neurology, under the editorship of S. N. Davi-denkov, t. 7, page 256, M., I960; Allan W. Inheritance of the shaking palsy, Arch.intern. Med., v. 60, p. 424, 1937; Barbeau A. Biochemistry of Parkinson’s disease, in book: 7-th Intern. Neurol. Gongress, v. 2, p. 925, Amsterdam, 1961; Kurland L. T. Epidemiology, incidence, geographic distribution and genetic considerations, in book: Pathogenesis and treatment of parkinsonism, ed. by W. S. Fields, p. 5, Springfield, 1958; P r a t t R. T. Page of The genetics of neurological disorders, p. 57, L. a. o., 1967, bibliogr.; Parkinson J. An essey on the shaking palsy, Arch. Neurol. (Chic.), v. 7, p. 681, 1922; Tretiakoff C.&& Contribution l' £tude de l'anatomie pathologique du locus niger de Soemmering avec quelques d6ductions relatives la pathogenie des troubles du tonus muscu-laire et de la maladie de Parkinson, P., 1919.

E. F. Davidenkova.

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