[MUSCLE WEAKNESS WITH NO SIGNS OF POLYNEUROPATHY IN PATIENTS WITH PROLONGED DISTURBANCE OF CONSCIOUSNESS. CASE REPORT].

Paper present several clinical examples of prolonged tetraplegia, which could be outcome not only of polyneuromyopathy of critical states, but also of disturbances in hierarchical relationship in CNS.

Experimental study of displays in contralateral acoustic reflex auditory stimulation.

The results of an experimental study of manifestations of the acoustic reflex with contralateral auditory stimulation at a frequency of 1 kHz are presented, and the principal possibility and informativeness of its use for diagnosing the diseases of the organ of hearing are demonstrated. The principal difference of the developed approach is the use of polyharmonic signal for measuring acoustic reflex manifestations during contralateral stimulation, which allows accelerating the examination procedure.

[Neurovegetative stabilization as a pathogenetic therapy for brain damage].

The article deals with neurovegetative stabilization as a pathogenetic therapy for brain damage. The approach is based on hypothesis that pharmacological effecting on central nervous system is able to make a passive protective medical system which can be close to passive protective systems widely represented in the nature. Complex opioid and clonidine administration provides sufficient level of neurovegetative stability on account of effecting on neuro-regulative structures the brain steam. Neurovegetative stabilization should be carry out in order of warning principle. In our opinion optimal doses are fentanyl 0.2-1.4 mkg kg(-1) per hour, clonidine 0.2-0.7 mkg kg(-1) per hour, propofol 0.5-2 mkg kg(-1) per hour, penthonal sodium 1-4 mkg kg(-1) per hour; diazepam 0.4-0.5 mkg kg(-1), and midazolam 0.05-0.2 mkg kg(-1) per hour. A criterion of the therapy sufficiency is a consistency between changes of different functional parameters. We believe the most important that new level of functioning must be maximally integrated and harmonized. It is possible if all pharmacological agents include the most reliable programs of adaptation complex human body reactions.

[Treatment outcome of chemotherapy with superselective cerebral artery catheterization in vegetative state patients].

The purpose of the study is to assess the impact of superselective neurotransmitter metabolic therapy in patients in a vegetative state. Superselective intraarterial infusion was conducted on 26 patients with relevant international criteria for the diagnosis of vegetative state. Comprehensive assessment of neurologic symptoms and severity of low metabolism on PET scan allowed to select the vascular pool, for the catheter installation. The catheter was placed either in the carotid or the vertebrobasilar pool. Infusion of neurotransmitter agents was conducted for 7 days continuously. Control of the level of metabolism of labeled glucose in the brain (PET) was performed within 2 weeks after arterial infusion. 14 out of 26 patients showed a positive trend of changes in energy metabolism of the brain. However, only 7 out of 14 patients showed further recovery of consciousness. The data confirms that the delivery path and a combination of medications play a definite role in the effectiveness of vegetative state therapy.

[Centrogenic reactions and their prognostic value during surgeries for posterior cranial fossa tumors in children].

The purpose of the study was to define the parameters of intraoperative monitoring, which allowed the prediction of complications due to surgery for posterior cranial fossa in children and the planning postoperative treatment tactics. The hypnotic propofol and a combination of the narcotic analgesic fentanyl and alpha 2-adrenoblocker clofelin were used to induce and maintain anesthesia. All patients were operated on in the sitting position. Hemodynamic parameters, arterial blood saturation, end-expiratory carbon dioxide tension, and central venous pressure were intraoperatively studied. As electrophysiological monitoring, the authors studied electroencephalograms and brainstem auditory evoked potentials by an Indeaver monitor (Nicolet). The investigators analyzed readings and data on admission to the operating suite, at the stages of anesthesia maintenance and surgery. The findings were used to define indications for anesthesia using the narcotic analgesic fentanyl and alpha 2-adrenoblocker clofelin in the postoperative period.

[The role of rivastigmine (exelon) in the treatment of consequences of cranial-brain trauma].

The use of rivastigmine in the rehabilitation of patients with cognitive disturbances after cranial-brain trauma (CBT) is reviewed. Replacement of cholinergic deficit exerts a positive effect on sanogenetic process in the brain, facilitate consolidation and learning of afferent information in the restoring brain. The authors emphasize that the administration of neuromediator preparations needs the level of organization of integrative brain activity which is sufficient for the formation of awakening reaction (opening of eyes - awakening from coma). It is suggested that neuromediator preparations, in particular cholinesterase inhibitor rivastigmine, should be used after the acutest CBT period, i.e. at least 2-3 weeks after CBT, when the main acute processes have been stabilized.

[Infectious complications after brain tumor removal: some aspects of their prevention, diagnosis, and treatment].

A hundred and fifty-five case histories of neurosurgical patients with the complicated early postoperative period were analyzed. Early postoperative regional pyoinflammatory complications were developed in 21 (4.4%) of 481 patients. There was meningitis in 17 (3.5%) cases, ventriculitis in 2 (0.4%), and wound infection (skin flap suppuration and postoperative wound fistula). Systemic pyoinflammatory complications were observed in 8% of the patients. Among them, there were pyoinflammatory complications in the respiratory system in 30 (6.2%) cases, pyonecrotic cystitis in 5 (1%), and sepsis in 2 (0.4%). Risk factors for regional and systemic complications are shown. Based on the findings, recommendations are given to prevent and treat pyoseptic complications in neurosurgical patients.

[Peculiarities of sporadic motor neuron disease associated with D90A and G12R mutations in Russian population]. / Osobennosti sporadicheskoi bolezni dvigatel'nogo neirona, assotsiirovannoi s mutatsiiami D90A i G12R, v rossiiskoi populiatsii.

Fifty-one blood samples of Russian patients with sporadic motor neuron disease were examined for mutations in Cu/Zn superoxide dismutase (SOD-1) gene. One female patient with amyotrophic lateral sclerosis (ALS) was heterozygous for G12R mutation. This patient suffered from ALS with cervical cord onset, pyramidal variant and fast progression. Mutation was also detected in her healthy son. Earlier, such mutation was described in 5 Italian patients with slow progressive ALS. Also, D90A SOD-1 gene associated haplotypes of the female ALS patients previously examined by the authors have been analyzed. A homozygous female patient with ALS was characterized by typical lumbar onset and extremely slow progression, as well as a female patient with heterozygous mutation and moderate progression carried so-called "Scandinavian" haplotype. To our knowledge, it is the first report on the finding of the haplotype considered as a "protective" one in the subjects heterozygous for D90A mutation with clinical symptoms of ALS. Mechanisms of "protective" influence of this haplotype on ALS course are not yet elucidated. Our finding suggests that the presence of "Scandinavian" haplotype does not completely protect from the disease development in patients exposed to other more pathogenic causative factors. This assumption is in line with modern conceptions on motor neuron disease as a complicated multifactor disorder.

[Association of homozygosity for short allele (S) of heavy neurofilament subunit gene with motor neuron disease and oxidative stress development]. / Assotsiatsiia gomozigotnosti po korotkomu alleliu (S) gena tiazhelykh neirofilamentov s bolezn'iu dvigatel'nogo neirona i razvitiem oksidantnogo stressa.

We analyzed distribution of heavy neurofilament (NF-H) gene S/L-polymorphic variants in 51 patients with idiopathic motor neuron disease (MND) vs control group and in relation to superoxide dismutases (SODs) activity and thiobarbituric acid reactive substances (TBARS) level in cerebrospinal fluid (CSF), erythrocytes and blood serum. We found that individuals with homozygosity for NF-H gene short allele (S/S-genotype carriers) in MND group predominate significantly over those in control one (p < 0.001). We revealed significant increase of oxidative markers in CSF and blood serum in MDN patients vs controls (p < 0.05), but not in patients with spondylogenic myelopathy, conforming non-specific role of oxidative stress in MND pathogenesis. There were no differences between TBARS level in CSF and serum in relation to the rate of MND progression, suggesting that oxidative stress does not influence the MND course. We showed normal SOD-1 activity in erythrocytes and CSF of MND patients that argued for the absence of these antioxidant enzymes deficiency in MND without SOD-1 gene mutations. We found significant association between homozygosity for short allele (S) and increased TBARS level in CSF (p < 0.02). These findings specify the role of NF-H with lower molecular weight in MND pathogenesis and make expedient antioxidants administration to MND patients homozygous for S-allele of NF-H gene.

[Polymorphism of glutathione S-transferase genes M1 and T1 in patients with motor neuron disease from the city of Moscow]. / Polimorfnye markery genov gliutation-S-transferazy M1 i T1 i bolezn' dvigatel'nogo neirona u patsientov iz g. Moskvy.

Comparison of the frequency distributions of alleles, genotypes, and genotype combinations of genes GSTM1 and GSTT1 did not show statistically significant differences between patients with motor neuron disease (MND) and a random sample from the Moscow population. Apparently, these genes are not involved in MND pathogenesis in these patients.

[ACE gene polymorphism in patients with ischemic brain disease]. / Polimorfizm gena angiotenzinprevrashchaiushchego fermenta u bol'nykh s ishemicheskoi bolezn'iu golovnogo mozga.

The aim of the present study was to define participation of gene angiotensine converting enzyme (ACE) in the development of atherothrombotic ischemic stroke. 153 patients with cerebrovascular disease were enrolled. The first group consisted of 50 patients with hemispheric atherothrombotic ischemic stroke and their 55 siblings. The second group consisted of 23 patients with chronic cerebrovascular disease with not less than 10 year history but with no history of strokes, including silent ones, and their 25 siblings. All the patients underwent standard clinical and laboratory study, insertion-deletion polymorphism of ACE gene was determined with polymerase chain reaction. chi 2-Test found no statistically significant differences in frequencies of I/D alleles between two groups as well as between genotypes of each group: p < 0.05. During analysis of ACE gene polymorphism in relation to level of arterial hypertension, presence of type II diabetes mellitus as risk factors no statistically significant differences in frequencies of I/I, I/D and D/D genotypes in both groups were found. We studied correlation between insertion-deletion polymorphism of ACE gene and the development of stenosis significantly affecting haemodynamics (over 70%). We found that among patients with stenosis in both groups D/D genotype significantly predominated (p < 0.05).

[Sporadic amyotrophic lateral sclerosis associated with Asp90Ala CuZn-superoxide dismutase mutations in Russia]. / Sporadicheskii bokovoi amiotroficheskii skleroz u patsientov s Asp90Ala mutatsiei med'-tsinksoderzhashchei superoksiddismutazy v Rossii.

16 blood samples from Russian patients (Moscow) with idiopathic motor neuron disease were analysed for mutations in the CuZn-superoxide dismutase gene. Two patients (12.5%) with amyotrophic lateral sclerosis (ALS) were found to have a disease related mutation. One patient appears to have autosomal recessive adult-onset ALS associated with homozygosity for Asp90Ala and presents the characteristic phenotype of very slowly ascending paresis with both lower and upper motor neuron signs. The other patient heterozygous for Asp90Ala presents ALS with lumbar onset and rapid progression. Both of cases are apparently sporadic.