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V V Grachev

Publications and source records attributed to V V Grachev.

6 recordsLinked to original sources

[Rett's syndrome: diagnostic issues].

Rett's syndrome (RS) is a severe disease of an early childhood, affecting girls mainly and manifesting in autistic symptoms, severe mental regression and motor dysfunction. The aim of the study was to analyze both early stages of clinical symptomatology and EEG correlates of RS. 65 patients aged 2.5-13 years with classic RS were examined. It was found that affective and autistic manifestations were the main symptoms during the 1-st stage of RS in approximately 50% of the cases. Two variants of classic RS were described according to the degree of motor dysfunction and behavioral disturbances: RS with motor disorders; RS with schizophrenoform disorders. Analysis of 145 EEG of 62 patients with the III-d PS stage revealed a presence of some correlations between location of the focus of the rhythmic theta-activity and clinical manifestations as well as their prognostic value. Thus, the decrease of the index of the rhythmic theta-activity in the patients on the III-d stage of RS correlated significantly with the improvement of the attention, behavioral and social activity, while its increase correlated with both the change for the worse of the higher cortex functions and advance of the motor disorders.

Adolescent↗

[Autoantibodies to nerve growth factor and astroglial protein S-100 in Rett's syndrome].

The levels of autoantibodies (AAB) to nerve growth factor (NGF) and to S-100 protein were examined in blood of both mentally normal girls and girls with Rett's syndrome (RS). The elevated titer of anti-NGF AAB, but not of anti-S-100 AAB was determined in the blood of patients with RS. There was relative individual stability of anti-NGF AAB level which didn't depend on the stage of the disease. At the same time, a reverse correlation was found between the level of these antibodies and severity of the disease: the mild form of the disease (with relative preservation of the speech elements and locomotor functions as well as with the later age of the onset of the disease and the later development of the regressive symptoms) is characterized by the higher AAB level. Autoimmune processes role (anti-NGF AAB) in formation of RS clinical features is discussed.

Astrocytes↗

[Speech and motor disorders in Rett syndrome].

Rett syndrome (RS) is a severe genetically conditioned disorder of an early childhood with an definite clinical phenotype in girls. Motor and speech disturbances are noted as the essential part of RS clinical picture. The variability of motor dysfunction and degree of speech deterioration were noted at different stages of the illness. The aim of the present investigation was to study dynamics of both speech and motor disturbances during 2-5 years of the course of the illness and to analyze a correlation of motor and speech functions' disorders. The study was performed in 50 girls with classical RS aged from 12 months to 14 years. The data obtained show a gradual involvement of different brain cortex structures in pathological process during the course of RS (especially of frontal and temporal-parietal regions) at the early stage of the illness and subsequent spread of the pathological process with successive involvement of subcortical structures, cerebellum, brain stem and eventually spinal cord. The neurophysiological investigation showed a strong correlation of speech and motor disturbances' degree with the EEG parameters. The level of theta-activity was significantly lower and the level of alpha- and beta-activity was significantly higher in EEGs of RS patients with more preserved speech and motor functions. In discussion mechanisms of motor and speech disturbances in RS were considered.

Adolescent↗

[Quantitative EEG correlates of the human frontal lobe dysfunction].

Increased central-parietal EEG theta-2 activity (about 6.5 per sec) was found in children with cognitive disorders (in Rett's syndrome, fragile X-syndrome, infantile autism) and in elderly patients with Alzheimer-type dementia (with prevalence of neuropsychological "frontal" disorders) in the presence of suppressed alpha rhythm. This theta-activity was closely associated with cognitive deficits and possessed a specific functional topography, namely it focused in the parietal region and suppressed by both visual stimulation and motor tests. The similar EEG pattern was observed in some patients treated with neuroleptics and/or during hyperventilation. By taking into account the data available in the literature on motor, oculomotor, regional cerebral blood flow and the probability prediction in frontal lobar dysfunction, it is suggested that the theta-activity described appears in the visuomanual coordination system and is a physiological correlate of decreased functional status of frontal lobes.

Aged↗

[An elevation in the level of autoantibodies to nerve-growth factor in the blood serum of schizophrenic children].

The level of autoantibodies (AAB) to nerve growth factor (NGF) was examined in blood of mentally healthy children, children with early children schizophrenia as well as with Kanner's and Asperger's syndromes too. The elevated titer of AAB to NGF was determined in blood of children with acute, active state of disease; meanwhile the same titer wasn't changed in the state of stable remission or steady defective state as compared with control group. The correlation was found between the level of AAB to NGF and the degree of disease progression. The correlations which were revealed between AAB to NGF level and peculiarities of the clinical state of patients permitted to use this index as the objective marker of both the acuteness and the severity of the patient's state.

Acute Disease↗

Speech and motor disturbances in Rett syndrome.

Rett syndrome is a severe, genetically determined disease of early childhood which produces a defined clinical phenotype in girls. The main clinical manifestations include lesions affecting speech functions, involving both expressive and receptive speech, as well as motor functions, producing apraxia of the arms and profound abnormalities of gait in the form of ataxia-apraxia. Most investigators note that patients have variability in the severity of derangement to large motor acts and in the damage to fine hand movements and speech functions. The aims of the present work were to study disturbances of speech and motor functions over 2-5 years in 50 girls aged 12 months to 14 years with Rett syndrome and to analyze the correlations between these disturbances. The results of comparing clinical data and EEG traces supported the stepwise involvement of frontal and parietal-temporal cortical structures in the pathological process. The ability to organize speech and motor activity is affected first, with subsequent development of lesions to gnostic functions, which are in turn followed by derangement of subcortical structures and the cerebellum and later by damage to structures in the spinal cord. A clear correlation was found between the severity of lesions to motor and speech functions and neurophysiological data: the higher the level of preservation of elements of speech and motor functions, the smaller were the contributions of theta activity and the greater the contributions of alpha and beta activities to the EEG. The possible pathogenetic mechanisms underlying the motor and speech disturbances in Rett syndrome are discussed.

Adolescent↗