[Femoral nerve repair with autografts for proximal retroperitoneal damage (case report and literature review)]. / Autoplastika bedrennogo nerva pri ego proksimal'nom zabryushinnom povrezhdenii (klinicheskoe nablyudenie i obzor literatury).

Femoral nerve damage, especially in proximal retroperitoneal space, is rare. Therefore, surgical strategy is still unclear for these patients. Various specialists discuss repair with autografts or neurotization by the obturator nerve or its muscular branch. OBJECTIVE: To demonstrate the diagnostic algorithm for proximal femoral nerve injury and favorable outcomes after repair with long autografts. MATERIAL AND METHODS: We assessed movements and sensitivity using a five-point scale, as well as ultrasound, magnetic resonance imaging and electroneuromyography data in a patient with extended iatrogenic femoral nerve damage before and after repair with long autografts (10.5 cm). RESULTS AND DISCUSSION: The patient had complete femoral nerve interruption in proximal retroperitoneal space with 10-cm defect that required repair with five autografts from two sural nerves. Postoperative ultrasound and magnetic resonance imaging revealed signs of graft survival and no neuroma within the nerve suture lines. The first signs of motor recovery occurred after 10 months. After 14 months, strength of quadriceps femoris muscle comprised 4 points, and electroneuromyography confirmed re-innervation. CONCLUSION: Femoral nerve repair with autografts for complete proximal anatomical interruption can provide sufficient restoration of movements and sensitivity. Therefore, this surgical option should be preferred instead of neurotization. Ultrasound, MRI and ENMG are valuable to clarify the diagnosis and state of the autografts.

[Age dynamic of the motor evoked potential from the tongue in healthy persons.]

Our aim was to determine parameters of motor evoked potential (MEP), elicited by the transcranial magnetic stimulation (TMS) from the tongue in healthy persons of different age. 62 neurologically healthy individuals without any speech problems (age range from 2 years old to 75 years, 34 females & 28 males) were enrolled. All underwent diagnostic TMS, single-pulse protocol, 90-sm round coil, Neiro-MS-D device. Coil was placed on Fz point, registration was performed by the surface electrode on the middle line of the tongue. Results. MEPs were of different appearance, but were registered in all cases, its average latency was 7,14±0,63 ms, average amplitude - 1,79±1,09 mV. There were no gender differences. Significant age difference (p<0,001) was registered between children (age 2-17 years) and two older groups (18-55 and 56-75 years) both on latency and on amplitude. MEPs latency was in three age groups, respectively, 6,21±0,45; 7,05±0,76, and 7,27±0,64 ms. MEPs amplitudes were 0,81±0,61; 1,88±1,01 and 1,69±0,92 mV, respectively. In healthy people aged 2-75 years MEP from the tongue may be registered in 100% of the cases; its average latency is 7,14±0,63 ms, average amplitude - 1,79±1,09 mV; there are no significant gender differences, but significant age differences. TMS of the tongue according to this protocol is relatively simple, not time-and efforts-consuming & may be widely implemented in clinical neurophysiology. There are significant differences on MEPs latencies and amplitudes in healthy persons, which may reflect age-related changes - lengthening of the cortico-lingual pathways and tongue muscle maturing in the childhood and then fibrous tissue development and other changes in nervous tissue and tongue muscle, developing with age.

[Diagnostic capabilities of spinal MR-angiography and spinal MR-tractography in Foix-Alajouanine syndrome]. / Diagnosticheskie vozmozhnosti spinal'noi MR-angiografii i spinal'noi MR-traktografii pri sindrome Fua­Alazhuanina.

The Foix-Alajouanine syndrome was originally reported by these authors in 1926, as rapidly progressive vasculitis on the background of a viral infection. The pathology was represented by the huge, more than 10 times, dilation either of the lumen, or the walls of the spinal vessels, either of the arteries, or the veins. There were no signs of thrombosis, no malformations. Massive necrosis was observed in the spinal cord. Though plenty of observations of the syndrome were reported over the past 100 years, most of them deal with arteriovenous malformations and/or thrombosis, which had not been revealed originally. We present the case of spinal viral vasculitis detected by means of spinal MR-angiography. The undoubted viral etiology of vasculitis allows us to attribute this observation to Foix-Alajouanine syndrome.

[Central motor pathways in children with multiple sclerosis].

AIM: To evaluate motor pathways involvement in children with multiple sclerosis. PATIENTS AND METHODS: We used transcranial magnetic stimulation method. 9 children with relapsing-remitting multiple sclerosis (mean duration 1,68 years) and 20 controls were enrolled. RESULTS: In most of the cases findings in multiple sclerosis group were abnormal. More often polyphasic changes of the motor evoked potentials (MEP) shape (78% of the cases) and elevation of MEP threshold (88%) were seen. CONCLUSIONS: Transcranial magnetic stimulation demonstrated high sensitivity in children with multiple sclerosis. Main neurophysiologic findings in multiple sclerosis in children may reflect altering membrane excitability of motor neurons and demyelinating lesions. Axonal damage in children with multiple sclerosis are less apparent.

[Diagnostic capabilities of spinal MR angiography and spinal MR tractography in a patient with motor neuron disease]. / Diagnosticheskie vozmozhnosti spinal'noi MR-angiografii i spinal'noi MRT-traktografii u bol'nogo s bolezn'yu dvigatel'nogo neirona.

Motor neuron diseases (MND) include two main forms - amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). A certain part of these diseases is hereditary, while etiology of sporadic cases remains unknown. Both entities are known to develop because of motoneurons damage. Difference between them lies in the state of the descending pyramidal pathways. The pyramidal pathways in SMA are intact, as brain pyramidal neurons are not affected, thus pathology of SMA is restricted to anterior horns of spinal cord. Meanwhile, most forms of ALS arise due to loss of both cerebral and spinal motoneurons, which, in addition to anterior horn lesion, leads to pyramidal descending pathways damage either in brain or in spinal cord. While pathological distinction between these two entities is clear and definite, the clinical difference remains obscure. We present the case of 41-year old patient with MND, in whom spinal MR tractography has revealed lateral columns to be intact that proves the utility of spinal MR tractography in differential diagnosis between ALS and SMA. Given that ischemic diseases of the spinal cord often occur with a clinical picture of MND, we also examined this patient using spinal MRI angiography, revealing a pronounced narrowing and tortuosity of the spinal arteries, complicated by occlusion of the right twelve intercostal artery.

[Molecular genetic basis of proximal spinal muscular atrophy and experience in its pharmaceutical treatment].

The review considers the original and published data on the molecular genetic basis of proximal spinal muscular atrophy (SMA), the most common monogenic neuromuscular disease. The structures of the SMN1 gene and SMN2 pseudogene, mutations distorting the SMN1 function, the structure and functions of the Smn neurotrophic protein, its role in biogenesis of small nuclear ribonucleoproteins (snRNPs), and the principles and prdblems of molecular diagnosis in SMA are described. Special consideration is given to the current approaches and prospects of gene and cell therapy of SMA, pharmacogenetic methods to correct the SMN2 function, and original results of long-term treatment of SMA patients with valproic acid drugs.

The use of micropolarization in the treatment of spinal cord lesions.

Transdermal micropolarization of the spinal cord was performed in patients suffering sequelae of spinal cord trauma and tuberculous spondylitis. Changes in clinical and electrophysiological status were monitored. These studies demonstrated that the use of local direct currents passed via skin electrodes promoted improvements in motor and autonomic functions in these patients, leading to positive changes in measures of the functional state of the spinal cord and heart activity. The possible mechanisms of the action of direct currents acting on the spinal cord are discussed, along with the potential for applying micropolarization in the treatment of spinal cord lesions.

[Clinico-electromyographic indicators in patients with ischemic myelopathy]. / Kliniko-élektromiograficheskie pokazateli u bo'lnykh s ishemicheskoi mielopatiei.

Electromyography, electroneuromyography and monosynaptic N-reflex studies were performed in 61 patients with ischemic myelopathy. These data can be used to specify localization and the extent of the process. Its differentiation with several diseases providing a phenocopy of spinal disorders (tumors, lateral amyotrophic sclerosis syringomyelia, funicular myelosis) is discussed. The results of the study are important for evaluation of the extent of motor disorders compensation states in disabled functions, and hence, the patients' working capabilities.

[Clinico-electromyographic characteristics of amyotrophic lateral sclerosis and the syndrome of amyotrophic lateral sclerosis of vertebrogenic etiology]. / Kliniko-élektromiograficheskaia kharakteristika bokovogo amiotroficheskogo skleroza i sindroma bokovogo amiotroficheskogo skleroza vertebrogennoi étiologii.

Fifteen patients with lateral amyotrophic sclerosis (LAS) and 37 patients with the LAS syndrome of vertebrogenic etiology were examined by global electromyography and recording of the H-reflex. The findings confirmed the diagnostic significance of electromyography in specifying the localization and severity of the pathological process. The method of monsynaptic testing was found to be especially valuable for diagnosing the disease at the early stage of development which helps to differentiate between LAS and the LAS syndrome of vertebrogenic etiology.

[The use of recombinant interleukin-2 in children with encephalitis].

OBJECTIVE: To study the efficacy of the domestic recombinant interleukin-2 (IL-2) in the complex treatment of encephalitis. MATERIAL AND METHODS: Forty patients, aged from 6 months to 17 years, were treated with the drug. A comparison group involved 35 patients who did not receive the drug. RESULTS: Recombinant IL-2 reduced (p<0.05) the aggravation of symptoms to 5.4±1.3 days, neurological deficit (1.6±0.2 EDSS scores at discharge from the hospital), frequency of exacerbations (from 22.9 to 5%) and frequency of cases with the development of multiple sclerosis during 3 years (from 17.1 to 2.5%). Parameters of the drug efficacy were correlated with positive changes in immunological parameters after 3-4 days of treatment. The study of multimodal evoked potentials demonstrated the positive effect of the drug on the central afferent conduction times. The follow-up MRI examination showed the decrease in the frequency of focal changes. CONCLUSION: Recombinant IL-2 is recommended for treatment of children with encephalitis.

[Clinical and pathogenetic features of CNS damage in children with diphtheria]. / Kliniko-patogeneticheskie osobennosti porazheniia nervnoi sistemy pri difteriinoi infektsii u detei.

The paper presents clinical and neurological features of diphtheritic polyneuropathy in 110 patients aged from 6 months to 14 years. The pattern of neurological complications appeared to be quite different for nontoxic and toxic forms; electroneuromyographical criteria of preclinical diagnosis of nervous disorders were determined. The role of a vascular factor in demyelinating process' genesis, immunohomeostasis implications in severity and outcomes of diphtheritic polyneuropathies, prognostic factors of the unfavorable course of the disease were defined. Scientifically grounded differential approaches to therapy were presented including application of vasoactive neurometabolites optimizing the processes of sanogenesis.

[The clinico-electromyographic characteristics of spastic torticollis]. / Kliniko-élektromiograficheskaia kharakteristika spasticheskoi krivoshei.

The aim of the study was the investigation of central and peripheral pathogenetic mechanisms of torticollis spasmodic. It was examined 68 patients-37 with left pathology and 31-with right one. Electromyograms (EMG) were registered in m. sternocleidomastoideus at rest and under postural loadings (at sitting and lying position of patients). Statistically significant differences were found in the pattern of EMG in connection of the side of torticollis. In the case of the left side of pathology EMG changes were presumably in "participating" muscle, in the right disorders-in "participating" muscle as well as in "nonparticipating" one (this change can be compensatory reaction which direct to the correction of a torsion).

[The use micro-polarization in spinal cord lesions]. / Primenenie mikropoliarizatsii v klinike porazheniia spinnogo mozga.

Transdermal micropolarization of the spinal cord was made in patients with consequences of the spinal cord injury or tuberculous spondylitis. Changes in clinical and electrophysiologic status were evaluated. It was found that local direct current through dermal electrodes promotes an improvement of both motor and autonomic functions in such patients. This corresponded to a positive dynamics both of the spinal cord state and cardiac activity. Possible mechanisms of influence of the direct current on the spinal cord as well as perspectives of application of micropolarization in spinal cord's damage are outlined.

[Neuromyalgic syndrome in children's infections]. / Neiromialgicheskii sindrom pri infektsionnykh zabolevaniiakh u detei.

Neuromyalgic syndrome (NMS) is clinically and electroneuromyographically (ENMG) first-ever described in 50 children aged 2-13. Muscular pains and pains along peripheral nerves occur in post-infection period of acute respiratory viral infections (ARVI), intestinal and parotitoviral infections with transient (in 3-6 days), dysfunction of peripheral nervous system and muscles. NMS is characterized by predominant involvement of lower limb muscles in pathologic process and by rarely developing generalized form. There are 3 types of changes of nerve conductivity and excitability with conductive blocks absence and disturbances regression that enable to distinguish these dysfunctions from ENMG changes in polyneuropathy. Provoked by infection, transitory non-specific inflammatory muscular reaction and functional disorders of nerve conductivity and excitability may be considered as one of the possible mechanisms of NMS development. More than 20 years of clinical and research experience of in neuroinfections clinic, supports the validity of NMS distinguishing among children acute infections for further diagnostic differentiation with polyneuropathy, viral meningitis and ARVI.