[A pathogenetic model of cerebral palsy in children born to mothers with antiphospholipid syndrome].

A pathogenetic model of infantile cerebral palsy caused by maternal antiphospholipid (APS) syndrome has been elaborated. Thirty-two children with cerebral palsy born to mothers with clinical signs of APS have been studied. The basic clinical feature of cerebral palsy in children was the prevalence of the double hemiplegic form, the absence of severe cognitive disorders, global muscular hypotrophy, rapid contracture formation and a tendency to frequent respiratory diseases. A seropositive APS variant was found in 42% of mothers examined, the seronegative one--in 58%. Such factors as (1) fetoplacental insufficiency and hypoxia caused by vascular infarctions of the placenta; (2) transplacental passage of antiphospholipid antibodies from a mother to a child; (3) intracerebral hemorrhages and periventricular leucomalacia in infants play the key role in the pathogenesis of cerebral palsy in children born to mothers with APS.

[Chronic inflammatory demyelinating polyneuropathy: report of 15 cases].

15 patients with CIDP (8 men, 7 women) at the age of 39-65 have been observed. The duration of the disease varied from 1 to 9 years. The authors have used in diagnostics the WHO's criteria as well as standards of needle and stimulative electro-neuromyography (ENMG). CIDP was shown in the study to occur more frequently after 50 with male prevalence. ENMG revealed 8 patients with compromised myelin and 7 patients with combined myelin and axon abnormalities, 3 patients with nerve impulse block conduction. The following variants of CIDP were distinguished: motor-sensor--7; motor-autonomic--3; pure motor--2; multifocal mononeuropathy with conduction blocks.

[Strokes and their causes in children]. / Insul'ty u detei i ikh prichiny.

Based on the literature and their own observations of 44 children aged 5-15 years (27 girls, 17 boys) with prior strokes, the authors characterize the types of strokes and principle causes of the disease as follows: (1) intracerebral and subarachnoidal hemorrhages (key etiology--arteriovenous malformations, blood disorders, coagulopathy, thrombocytopenias, thrombocytopathy, etc.) and (2) ischemic strokes i.e. (i) thrombotic (congenital and acquired vascular aplasias, angiitis, antiphospholipoid and viral vasculopathy, blood system coagulate activation, etc.); (ii) embolic (cardiogenic, septic, placental, etc.) and (iii) hemodynamic as a consequence of severe cardiomyopathy and disrupted total hemodynamics. The definitions of metabolic stroke as a complication of mitochondrial encephalopathy, homocystinuria and non-differentiated stroke caused more often by emergence of pathologic weariness of connective tissues were suggested. To elicit the stroke causes and types in children, the authors propose a reliable algorithm for clinico-instrumental diagnostic screening in acute period of stroke.

[Some aspects of cerebral autonomic vascular dystonia in patients with mitral valve prolapse]. / Osobennosti tserebral'noi vegetativno-sosudistoi distonii u bol'nykh s prolapsom mitral'nogo klapana.

Some aspects of origination of vegetovascular dystonia (VVD) in patients with mitral valve prolapse are considered. Forms of VVD manifestation are presented. Possible causes of VVD development accompanying mitral valve prolapse are analyzed. Original investigations have been carried out designed to study the condition of cerebral bloodflow in those patients suffering from VVD as a manifestation of mitral valve with the aid of the transcranial dopplerography technique. The authors have come to the conclusion that it is necessary for us to use a differentiated approach toward choosing a corresponding policy of managing patients.

[The therapy of non-traumatic vegetative state syndrome in children]. / Vozmozhnosti terapii netravmaticheskogo apallicheskogo sindroma u detei.

Twenty nine non-traumatic appalic syndrome (AS) cases of various etiology were observed in children aged from 3 to 14 years. The clinical picture study was carried out, along with brain structure visualization in vivo (CT and MRT), EEG and cerebral blood flow detection with transcranial ultrasonic dopplerography. All the cases were studied in dynamics in relation to influence of complex course therapy developed by the authors, including vasoactive drugs, nootropics, craniopuncture, acupuncture and electrostimulation of craniopuncture zones. Five patients (17.2%) had a distinct positive dynamics with complete consciousness rehabilitation on the background of moderate neurological deficit regress. In general, CT-data and changes of EEG and cerebral blood flow in AS were not found to have any prognostic value and to be etiologically specific. But changes of EEG and cerebral blood flow in response to the treatment appear to be positive prognostic sign. The prognosis for AS due to meningoencephalitis is worse than one for AS due to hypoxic-ischemic encephalopathy. The non-traumatic AS duration for more than 2 months corresponds to unfavorable outcome.

[Clinical instrumental and immunological studies of multiple sclerosis in children]. / Kliniko-instrumental'nye i immunologicheskie issledovaniia rasseiannogo skleroza u detei.

The results of clinical examination, data of analysis of the visual evoked potentials, computer and magneto-resonance tomography allowed to diagnose a reliable multiple sclerosis in 25 children (16 girls and 9 boys). The age of the onset of the disease was from 4 to 14 years, the duration of the follow-up--from 1 to 12 years. Monosymptomatic onset, remittent course with frequent exacerbations and successful recovery of the functions during the remissions prevailed. Purposeful treatment allowed to achieve a stable remission in 2/3 of the cases, while a gradual increase of the neurologic deficit was observed in the other patients. Phenotyping by HLA-system revealed association of demyelinating diseases in children with HLA-B12 (RR = 6.0; p < 0.025), together with the absence of HLA-B18 antigen. The authors made a conclusion that multiple sclerosis may be diagnosed in childhood and that it has some clinical and immunologic peculiarities.

[New approaches in the treatment of speech disorders in children with an organic brain lesion]. / Novye podkhody v lechenii rechevykh narushenii u detei s organicheskim porazheniem golovnogo mozga.

Speech disorders encountered in children presenting with neurological pathology make their medical and social rehabilitation a real challenge. Overall sixty children were examined. Of these, 38 had infantile cerebral paralysis, 22 that being presented with sequelae of the hypoxic affection of the brain in ante- and perinatal period, who exhibited speech disorders (alalia, dysarthria, retarded speech development). Described in the paper are features of bloodflow disorders in intracranial and vertebral arteries as evidenced by ultrasonic Doppler technique and changes in brain bioelectrical activity according to findings from electroencephalomapping. In 53 patients, a positive effect was obtained as a result of therapy conducted in accordance with the stimulation type techniques (electroscalp therapy against the background of intramuscular administration of nicotinic acid plus laser therapy, transcutaneous electrostimulation, employment of such drug preparations as cogitum, nero-force, sirdalud).

[Histocompatibility antigens in multiple sclerosis in children]. / Antigeny gistosovmestimosti pri rasseiannom skleroze u detei.

A phenotyping was done in respect of HLA system in 14 children with documented multiple sclerosis (MS), 20 children with potential MS, and 42 their near relations. HLA-B12 antigen was recordable with high frequency (RR = 6.29; P < 0.025) while HLA-B18 with low one (P < 0.01) as compared to normal subjects both among patients in the general group and in those with proved MS. The latter showed a tendency toward increase in frequency HLA-B7. Children with significant MS where there was an association in the phenotype of HLA-B12 or HLA-B7 with HLA-A3 ran predominantly an aggravating course while their association with HLA-A2 resulted in a more benign course. The results obtained in respect of HLA antigens in MS in children differ from those of other authors on MS in adults.

[The role of nuclear magnetic resonance monitoring in early clinical-instrumental diagnosis of multiple sclerosis]. / Rol' iaderno-magnitno-rezonansnogo monitorirovaniia v rannei kliniko- instrumental'noi diagnostike rasseiannogo skleroza.

Nucleo-magnetic resonance tomography (NMRT) facilitated considerably early diagnosis of multiple sclerosis. Yet, there appeared data indicating the existence of NMRT negative forms. These were found in 15 patients of 82 ones examined using NMRT only which failed to defect foci of demyelinization in spite of a clear-cut clinical picture. NMRT monitoring during 3 years (from 2 to 5 NMRT investigations) permitted to visualize the foci of demyelinization located primarily in the periventricular cerebral region. The conclusion was made that a presence of clear clinical symptoms as well as a remitting character of the pathologic process confirmed by visualization of multiple foci of CNS demyelinization by means of NMRT monitoring, gave full chance for a reliable diagnosis of multiple sclerosis. A lack of such confirmation may be explained by the fact, that clinical debut of the disease reflected only the phase of multiple sclerosis and forestalled forming of a focal demyelinizating process, pathognomonic for this disease.

[Magnetic resonance monitoring in the diagnosis of multiple sclerosis]. / Magnitno-rezonansnyi monitoring v diagnostike rasseiannogo skleroza.

The introduction of the method of magnetic resonance tomography (MRT) proved to be very helpful in dealing with the problem of early diagnosis of multiple sclerosis (MS) but has not finally solved it. There have appeared reports about the existence of instrumentally negative forms of MS. We attempted to objectivize with MR method an early stage of MS in 82 patients. Of these, 15 subjects who had a clear clinical picture as a result of a single MR-investigation (the unit "Bruker", Germany) did not reveal any foci of demyelinization. In the above patients, MR-monitoring was conducted over three years 2 to 5 times, as a result of which undertaking we managed to visualize demyelination foci in 12 subjects (81%) with predominant localization thereof in the periventricular area. Clear clinical symptomatology, remitting course confirmed by visualization of multiple demyelinating foci in the central nervous system with the aid of MRT permit making an accurate diagnosis of MS, which fact determines a long-continued immunomodulating therapy of this grave medical condition.

[The intensive therapy of exacerbations in disseminated sclerosis in children]. / Intensivnaia terapiia obostrenii rasseiannogo skleroza u detei.

On the basis of long clinical experience principles are substantiated and schemes are suggested of therapy of childhood disseminated sclerosis (DS), these being as follows: 1) pulse therapy with high dose intravenous corticosteroids, a switch-over to oral intake followed by synacten-depo during the phase of exacerbation; 2) long-term maintenance therapy during transition to the phase of remission. The above algorithm allowed the patients to be helped out of the exacerbation. Analysis of 3-to-11 yr catamnesis permitted assessing the efficacy and outcomes of the proposed therapy in children. Of the 21 cases, thirteen showed stabilization of the process as evidenced by continuing remission of one to 3 years in duration. Eight children showed short-time remission, with neurological deficit slowly progressing, resulting in invalidism in 7 patients. In summary, the proposed therapeutic regimen for DS exacerbation in children permits achieving quick regression of the neurologic symptomatology, returning progression of neurological deficit, which observation was recordable in two thirds of the patients. In 1/2 of children DS runs an aggressive course that does not respond even to intensive therapy; in these, long-term remission was not achievable, this resulting in progression of the neurologic symptomatology and, in the long run, disability under protracted course of the condition.

[Autonomic vascular dystonia with syncopal attacks and its correlation with epilepsy in children and adolescents]. / Vegetososudistaia distoniia s sinkopal'nymi pristupami i ee sootnoshenie s épilepsiei u detei i podrostkov.

Ninety children and adolescents with syncopal episodes were examined. Out of them a risk group was identified by the results of clinical and instrumental studies for their transformation to epilepsy. The group required anticonvulsive therapy and case follow-up along with EEG monitoring.

[Histocompatibility antigens and their role in the pathogenesis of developmental anomalies of the nervous system in children]. / Antigeny gistosovmestimosti i ikh rol' v patogeneze anomalii razvitiia nervnoi sistemy u detei.

Clinical and immunogenetic examinations of 40 patients with congenital diseases of the nervous system and of 10 couples of their parents revealed an association with HLA B12 antigen, HLA homozygosis in the majority of parents and children, and a high level of antigenic stimulation. The results may be considered as markers and criteria of a high risk of development of this disease in children.