[Myoclonus in children].

The article includes data on terminology, an etiology, mechanism and clinical presentation of myoclonus in children. The clinical classification, scheme of diagnostic search and feature of the differential diagnosis of different types of myoclonus is presented.

[Clinical and psychological features of children with agenesis of the vermis cerebelli].

The article considers behavioral disturbances in children with anomalies of the cerebellum found by MRI studies. Presented are literature data on the relations between pathology of the cerebellum and early autism in children. The cerebellum is involved not only in movement coordination but also in social adaptation and speech communication. Cerebellum-specific genes expressed in early age are similar to those of hippocampus. Our own study of children with agenesis of the vermis cerebelli detected by MRI and behavioral disturbances included 20 children aged 3-15 years (mean age 7,05 years, 12 male, 8 female). Some autistic features have been found.

[The structure of the diagnosis of neuromuscular diseases (the examination chart of outpatients for computer analysis)]. / Struktura diagnoza nervno-myshechnykh zabolevanii (karta obsledovaniia ambulatornykh bol'nykh dlia komp'iuternogo analiza).

To provide a prophylactic medical examination (PME) as well as to improve the quality of diagnosis and treatment in a local medical institutions special coded card case sheets for patients with neuro-muscular disease adapted for computer analyzes are proposed. Such sheets facilitated determination of the diagnosis, objective patient's state during PME and correction of the scheme of therapy. The sheets were elaborated for long-term observation of 3153 patients with different neuro-muscular diseases and syndromes. The scheme of the diagnosis included such indices as character of damage of motor unit, type of heritability, age of onset of the disease, main systems of muscular atrophies, rate of the progress of pathological process, severity of the patient's state.

[Chromosome instability in multiple sclerosis]. / Nestabil'nost' khromosom pri rasseiannom skleroze.

The results of the study have confirmed the existing data on an elevation in the spontaneous levels of sister chromatid exchange (SCE) in multiple sclerosis patients as compared to control subjects (in the patients the mean number of SCEs per cell was 10.5 +/- 0.3 whereas in the control group it was 8.2 +/- 0.25).

[Clinical systematization and diagnosis of polyneuropathies]. / O klinicheskoi sistematizatsii i diagnostike polinevropatii.

The authors proposed a working scheme for the clinical classification of polyneuropathies according to which the latter ones are divided into primary idiopathic and combined hereditary forms, and secondary ones resulting from infectious, toxic, vascular, metabolic, and physical factors. The authors also worked out the clinical and electroneuromyographic criteria for the diagnosis (including the differential one) of the following disorders: Charcot-Marie's neural amyotrophy, the Roussy-Levy syndrome, sensory polyneuropathy with acroosteolysis, toxic vegetative polyneuropathies, and secondary lesions of the peripheral nervous system in patients with diabetes mellitus and collagenous diseases.

[Problem of amyotrophy]. / K probleme amiotrfii.

Clinical and electroneuromyographic studies of 1771 patients with diseases of the nervous and neuromuscular systems revealed disruption of the trophic supply to the skeletal muscles in cases of damage to both the segmento-peripheral neuromotor apparatus and vegetative and somatic segmental and suprasegmental formations. Electroneuromyographic indicators of the amyotrophic process include a decrease in the amplitude of the maximal M-response and a reduced number of functional motor units. In hyperkinetic syndromes, a paradoxical elevation of these indices is observed, which seems to be associated with "an alleviating trophotropic effect" exerted by striatal system impairment.

[Clinicoelectroneuromyographic study of neuromuscular syndromes combined with connective tissue pathology]. / Kliniko-élektroneiromiograficheskoe issledovanie nervno-myshechnykh sindromov, sochetaiushchikhsia s patologiei soedinitel'noi tkani.

An electroneuromyographic examination of 132 patients with pseudohypertrophic and myosclerotic forms of myodystrophies, collagenoses, mucopolysaccharidoses, Marfan's disease, and Chernogubov-Ehlers-Danlos' disease was carried out. A relationship between the duration of the M-response potential and the state of the interstitial connective tissue of the skeletal muscles was revealed. This duration appeared to be longer in cases of proliferation, sclerosis, and fibrosis of the interstitial tissue, and shorter in cases of a deficiency of the collagen fibres and increase of their elasticity.

[Hereditary areflexic dysstasia (Roussy-Lévy syndrome)]. / Gereditarnaia areflektornaia distaziia (sindrom Russi--Levi).

Clinical and electroneurographic investigations of 22 cases of hereditary areflectory dysstasia (Roussy-Levy's syndrome). 20 cases of Charcot-Marie's neural amyotrophy and 8 cases of Friedreich's familial ataxia were carried out. In the patients with Roussy-Levy's syndrome, the speed of the impulse conduction along the peripheral nerves of the extremities, as well as the amplitudes of evoked potentials were lowered to the greatest extent: this was an evidence of nosological independence of the disease.