В отечественной литературе недостаточно внимания уделено анатомическим вариантам иннервации конечностей. Тем не менее практикующему неврологу и нейрофизиологу регулярно приходится сталкиваться с нетипичной клинико-нейрофизиологической картиной, выявляемой как случайно, так и при патологии. В настоящей статье представлена информация о наиболее часто встречающихся вариантах межневральных анастомозов длинных нервов рук и ног. Особое внимание уделено нейрофизиологической картине данных межневральных коммуникаций. Настороженность в отношении возможной атипичной невральной иннервации поможет сократить ошибки в интерпретации данных, получаемых в ходе электронейромиографического исследования, улучшит понимание клинической картины при повреждении периферических нервов, участвующих в образовании анастомоза.

Diabetes mellitus is a widespread disease often affecting peripheral nervous system. This include diabetic autonomous neuropathy that can endanger the patient's life. Timely detection of complications of diabetes mellitus as well as its adequate therapy can improve prognosis of the disease. The possibilities of Milgamma and Tiogamma for pathogenic therapy in patients with diabetic polyneuropathy are considered in this paper. Gabagamma can be effectively relieve neuropathic pain and used together with other drugs that normalize nerve tissue metabolism.

Background. In the recent years interest towards nerve sonography has largely increased, specifically in terms of differentiating types of hereditary motor and sensory neuropathy (HMSN). The diagnostic possibilities of high-resolution ultrasound (HRUS) compared to standard neurophysiological tools in the peripheral nerve disorders is still a matter of debate.

Objectives. Analysis of quantitative and qualitative ultrasound changes of limb nerves in patients with HMSN type 1 and its comparison with anthropometric and nerve conduction study data.

Materials and methods. 44 HMSN patients were analyzed: 16 men, mean age 35,9 ± 6,8 years; 16 (37 %) with autosomal dominant type 1А, 11 (25 %) – with 1В type and 17 (38 %) with Х-linked inheritance. Control group included 44 subjects, 16 male; mean age 35,9 ± 6,8 years. HRUS parameters were analyzed bilaterally on the selected levels: cross-sectional area (CSA), visual cross sectional and longitudinal patterns of the median and ulnar nerves, C5, C6, C7 spinal nerves, tibial, peroneal and sciatic nerves. HRUS parameters were compared to standard anthropometric data, nerve conduction velocity and CMAP amplitude.

Results. In all HMSN cases CSA was enlarged compared to healthy controls. Greater changes were found in patients with autosomal dominant inheritance. CSA enlargement in С5, С6, С7 spinal nerves was found in patients with HMSN 1A, С6, С7 – in HMSN 1В, С6 – in HMSN 1X, confirming the necessity to include those nerves in the sonographic protocol in patients with HMSN. Three qualitative cross sectional and longitudinal patterns of the investigated arm nerves were identified, distinct for each of the HMSN type. Absence of significant differences in CSA of the upper limb nerves among analyzed types of HMSN makes it unreliable as the differential parameter, opposite to the defined sonographic patterns. Methodological issues and absence of significant quantitative and qualitative data from the lower limb nerves makes it possible to exclude those from HRUS protocol in HMSN.

Conclusions. Nerve sonography of the upper limbs could be a useful additional tool in the differential diagnosis of HMSN type 1 when applying described qualitative HRUS patterns.

Background. Management of the myasthenic crisis remains one of the issues in clinical neurology.

Objective. Analysis of the timeframes of the myasthenic exacerbation since the disease onset, sex distribution, age predominance, specific clinical features, precipitating factors in order to investigate the efficacy of the management algorithm of the myasthenic crisis.

Materials and methods. Medical histories of 33 female and 19 male patients with myasthenia for the period of 2000 to 2003 were analyzed.

Results. In comparison with the literature data the number of mysthenic crisis in myasthenic patients was lower and did not exceed 25 %. Sex distribution 1,2 (male): 1 (female). In 70 % worsening of myasthenia appeared in the first two years of the disease: in 6 patients (46.2 %) at the age of 22–35 y. o.; in 5 patients (38.5 %) – at the age of 36–60 y. o. All patients suffered from generalized myasthenia, in three of them ocular and bulbar muscle weakness predominated. In 46.2 % of patients with crisis, thymus abnormalities were present (thymoma / hyperplasia). The most common precipitating factors were: infection (36.9 %), stress (26.3 %), misuse of the anticholinesterase drugs (15.8 %). In 5.2 % the worsening of myasthenia followed the delivery. The crisis triggering factor was not identified in 15.8 % of cases.

Conclusion. In order to prevent the exacerbation of myasthenia, generalized forms of myasthenia with poor response to anticholinesterase drugs require special attention towards patients in their first two years of the disease, including patient’s educational program on appropriate mode of drug administration and avoiding initiating factors, as well as thymectomy.

Herbal sedatives serve an alternative to antipsychotics and hypnotics aimed to alleviate symptoms of anxious disorders and insomnia. Valeriana officinalis L., Mentha piperita L. and Melissa officinalis are most widely used in neurology as sedatives of herbal origin. We present the results of a randomized open-label trial on efficiency and safety of Persen® and Persen® Night containing extracts of the above mentioned plants in patients with short-term insomnia. The study consisted of 60 subjects of 18–65 y.o. (mean 42.4 ± 6.9 y.o.) with short-term insomnia due to adjustment disorder or mixed anxiety-depressive disorders: 30 of them got Persen® 2 tablets a day and 30 – Persen® Night, 1 capsule 30–60 min before sleep during 4 weeks. The majority (76.5 %) of patients referred the onset of insomnia with psychosocial traumatic stressor. Persen® Night’s main action was found on superficial sleep, number of night awakenings, sleep onset rate. At the end of the therapy with this substance 39.7 % of patients fell asleep in 10–15 min, and 92.2 % – in 30 min, accordingly, while for Persen® at 17.4 and 80.3 % accordingly (р < 0.05). In the meantime Persen® decreased the bad sleep perception at awakening and day somnolence, mostly attributed to the mood improvement and decrease of anxiety. Levels of efficacy and safety for both substances were significant, allowing to regard them as potential phytotherapeutic agent in the treatment of insomnia and mixed anxiety-depressive disorders.

The clinical and genetic features of hereditary motor and sensory neuropathy (HMSN; Charcot–Marie–Tooth disease, CMT) caused by newly identified missense mutation s.65G>T (p.Pro22His) in NEFL gene located on the chromosome 8р21.2 are described. The disease was diagnosed in a large family from Ust-Dzhegutinsky district of the Karachay-Cherkess Republic with the segregation of the disease in four generations. The prevalence of the HMSN in that district was found to be 1:4340 persons, including 1:3376 among Karachays. The clinical picture of the disease was characterized by onset at the age of 11–14 years, weakness in foot muscles and steppage gait. The specific features in the majority of patients were the absence of major sensory disturbances, as well as long-term preserved distal arm muscles. Nerve conduction velocity in the median nerve varied from 30 to 42 m/s, which corresponds to values in patients with CMT2E, previously described.

Hereditary motor and sensory neuropathy (HMSN, Charcot–Marie–Tooth disease) is a group of genetically heterogeneous disorders with more than 80 genes linked to different phenotypes, including IGHMBP2 gene responsible for HMSN type 2S (OMIM 616155). Until recently, mutations in IGHMBP2 were exclusively associated with neonatal distal spinal muscular atrophy with respiratory distress (SMARD1, OMIM 604320). A case report presents a boy with infant onset decreased distal muscle tone and weakness, distal wasting and deformation in legs and hands, areflexia and decreased sensation without respiratory involvement; at age seven he had severe fixed kypho-scoliosis. EMG revealed signs distal axonal neuropathy. The exsome sequencing confirmed the allelic variant of two compound heterozygous mutations in gene IGHMBP2: known missens mutation с.1616С>Т (р.Ser539Leu) in exone 11 and a novel deletion с.2601_2602delGA in exone 13. The diagnosis of infant HMSN type 2S was confirmed. The phenotype of HMSN type 2S and its diagnostics differences between SMARD1 are discussed.