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The expert consensus is aimed to develop an algorithm for the diagnosis and treatment of mononeuropathies for outpatient neurologists. Leading experts in the field of neurology have suggested workup options for certain types of tunnel mononeuropathies based on current data on the effectiveness and safety of various types of conservative and surgical treatment.

The article presents the work of a multidisciplinary team of experts from various fields of medicine to optimize the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology» for use in clinical practice. The survey of respondents was conducted from June 28 to September 28, 2021. As a result of this survey, by repeatedly making edits and clarifications during communication with respondents, the final version was obtained, which allows assessing the patient's subjective sensations by the nature and localization of pelvic pain, sensitivity disorders and pelvic organ function. The main objective of this Questionnaire is to differentiate patients with neurogenic pain from a huge number of patients with chronic pelvic pain. This aspect will allow a more targeted approach to the diagnosis and pathogenetically justified treatment of patients, including after appropriate instrumental examinations. The work of a multidisciplinary team implies a higher degree of objectification and terminological accuracy of the Questionnaire under discussion. The presented version of the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology» will be primarily used in coloproctological patients with pelvic pain problems and anal incontinence and obstructive defecation. Further studies will be directed to the clinical evaluation of the results of the work carried out.

Objective: To describe clinical and sonographic features of confirmed intraneural perineurioma in 5 patients., Material and Methods: We report clinical and sonographic features and retrospective follow-up data in 5 patients with intraneural perineurioma verified by biopsy., Results: In all cases, symptoms were represented by a slowly progressive painless mononeuropathy with muscle atrophy and impaired tendon reflexes. Ultrasound examination revealed locally enlarged cross-sectional area of the nerve that was confirmed by intraoperative visualization., Conclusion: Intraneural perineurioma should be obligatory suspected in case of fusiform thickening of the nerve with locally changed echogenicity. Pooled multiple-center studies with large samples are needed to determine sensitivity and specificity of the main ultrasound parameters including changes in cross-sectional area and echogenicity of the nerve.

The authors report the clinical case of a 7-year-old child with gradual development in left leg weakness considering by the neurologist as the unknown lesion of the sciatic nerve. According to the results of clinical and instrumental examinations, the diagnosis was established as a focal form of chronic inflammatory demyelinating polyneuropathy (CIDP). The dysimmune cause of the disease was confirmed by MRI of the lumbosacral plexus with contrast enhancement of neural structures and response to therapy with high-dose intravenous immunoglobulin in combination with corticosteroids.

Among many causes of compression neuropathies involving the radial nerve, compression of the posterior interosseous nerve (PIN) by the returning radial arteries is rare., Objective: To describe the clinical instrumental characteristics of cases of mononeuropathy caused by compression of the return radial arteries., Material and Methods: Two observations with neuropathy of the deep branch of the radial nerve, manifested by weakness in the corresponding muscles, inconsistent pain syndrome with previous stereotypic (recurring) physical activity are presented., Results: According to the results of ultrasound examination of posterior interosseous nerve, a local hypoechogenic increase in the cross-sectional area of nerve of different lengths was revealed. During surgical decompression of the nerve, in the first case, a tight arterial loop was found around the nerve, in the second case, expanded arterial trunks, one of which, of small diameter, covered the nerve in the 'loop', and the other, larger, pierced the nerve. After the operation, regression of motor deficiency and relief of pain were noted.

Aim: To describe the sonographic phenomenon of the focal 'hourglass-like constriction' of the peripheral nerves (FCPN)., Material and Methods: The authors described 7 patients meeting the criteria for the diagnosis of neuralgic amyotrophy with unilateral FCPN identified with ultrasound in 4 cases and detected intraoperatively in 3 cases (preliminary ultrasound was not performed). The US scanner Sonoscape Pro mode gray scale in the transverse and longitudinal scanning, linear probe 8-15 MHz and Logiq9 scanner with elastography were used., Results: FCPN was detected in the single nerve in 4 patients, in two nerves in 2 patients and in 3 nerves in one patient. Among all the nerves involved in the pathological process, the radial nerve and its branches were affected in 73% (8 nerves); the ulnar nerve was involved in 18% (2 nerves) and the musculo-cutaneous nerve in 9%. The length of the constriction of the peripheral nerve did not exceed 1.7 mm. The deformation coefficient (DC) of constriction area was 3.8 to .,9; the change in the elasticity in the form of an increase of DC to 5.9 when compared to the intact portion of the nerve and a decrease in echogenicity were observed in one patient., Conclusion: High-resolution ultrasound of the nerve can be an informative method for the diagnosis of idiopathic non-traumatic FCPN mononeuropathy.

Pompe disease (PD) is a rare autosomal recessive muscle lysosomal glycogenosis caused by a deficiency of acid-α-glucosidase. There are two main forms of the disease: aggressive infantile PD started within the first year of life with a severe enzyme deficiency and multiorgan involvement, and late onset PD (LOPD) with progressive signs and symptoms including predominant proximal, axial muscle weakness and respiratory insufficiency started at any time from 1 till 75 years and older. Usually due to physician's unawareness, most adults with PD are diagnosed with great delay. The typical features and early nonspecific signs in four patients, aged between 35 and 72 years, with confirmed LOPD are delineated and discussed in correspondence with the age of first signs, age development of muscle weakness, distribution and age of final diagnosis. The disorders for differential diagnosis and spectrum of conditions that expanded the possibility of PB are listed. The fluorometrically analyzed level of acid α-glucosidase from dried blood spots is considered to be the first choice diagnostic method for clinically suspected cases of LOPD.

POEMS-syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin Changes) is a rare nosological form occurred in patients with paraproteinemic hemoblastosis. Chronic progressive sensory-motor polyneuropathy is a key syndrome of the disease and it is a common reason for referral to neurologist. The paper presents data about POEMS-syndrome and own case reports with the analysis of disease features and results of examination.

During examination of 165 children aged 5 to 15 years (primarily identified during planned monitoring in Petrozavodsk children's institutions) with dysfunctional urination and encopresis without organic lesion of the central nervous system, autonomic dysfunction syndrome (ADS) was revealed. According to the results of urological examination, which was supplemented with the registration of spontaneous voiding rate and counting the radial pulse, overactive bladder syndrome and insufficient relaxation of the pelvic floor muscles during urination and defecation were detected; relationship between the number of heart rate (as a marker of sympathetic nervous system activity) and the effective volume was identified. It was revealed that the children with ADS in the presence of tachycardia show intermittent decrease of effective amounts of urination, and have residual urine. The standard course of treatment using colon hydrotherapy and biofeedback to activate cystic and obturator reflex caused a positive but short-term therapeutic effect; clinically and statistically significant increase in the effective volume of the bladder was not achieved, despite the reduction in residual urine volume. During the course of treatment using methods of biofeedback, bladder volume remained almost unchanged and tachycardia persisted, indicating the continued oppression of the sympathetic activity. The course of treatment using nootropic drug picamilon and alpha-adrenoblocker doxazosin with peripheral actions allowed to restore the reservoir and evacuation functions of the bladder, to achieve a regular bowel movement without encopresis. It was revealed that the combined dysfunction of pelvic organs occur in children with high activity of the sympathetic division of the ANS, which has a direct impact on the accumulation phase of voiding cycle and relaxation of the pelvic floor muscles.

We studied 67 children, aged 2-9 years, with cerebral palsy including 56 children with a spastic form. An electromyographic method was used for the development of optimal tactics of botulinum toxin type A injections in different clinical presentations of spasticity. The best clinical results were obtained in children with the following changes on EMG: 1) the tonic muscle activity in resting state was minimal (<10 microvolts) and had local or regional distribution; 2) the pathological synkinetic activity during voluntary movements was minimal (synergetic activity coefficient for shin muscles was less than 0.45); 3) the disturbance of interactions between synergistic and antagonistic muscles was moderate (reciprocity coefficient was not less than 0.4); 4) EMG amplitude in voluntary muscle contraction should not be less than 150 microvolts. This approach to the treatment allowed to reach higher levels on The Gross Motor Function Classification System in part of children.

Dorsalgia's is an actual medical and social problem. It gains prominent significance among railway workers, connected with railway communication. Pain syndromes among this group of patients have different complex pathogenetic mechanisms of the development. In a great number of cases it is usually seen the worsening of the condition in connection to concomitant anxiety and depression. There are also concomitant osteoporosis and autonomic trophical disturbances.

The authors present a review of literature focusing on state-of-art of surgical management of cervical spondylogenic myelopathy (CSM). Pathophysiology of CSM, evaluation and differential diagnosis are also described. Special attention is given to the value of neurophysiological aspects in pre- and postoperative examination.

Men aged over 40 years more commonly develop NK/T-cell lymphomas (NK/T-CL). The paper describes a case of NK/T-CL in a 20-year-old man. Despite the fact that the disease (nasal septum perforation, hard palate bone destruction, recurrent nasopharyngeal bleeding, considerable weight loss, and high erythrocyte sedimentation rate,) progressed rapidly for 5 months, the patient was found to be diagnosed as having Wegener' granulomatosis (WG). Repeated incisional biopsies showed massive necrotic changes with no clear histological verification of the diagnosis. The absence of lung and kidney lesions typical of WG, the lack of antineutrophil antibodies, and the detection of Epstein-Barr virus DNA in blood could presume NK/T-CL and confirm it by extended biopsy to have materials sufficient for morphological and immunomorphological studies. This observation shows that the disease may occur at a young age and rapidly progress; only early diagnosis can improve prognosis in patients with this type of lymphomas.

The article analyses problems of diagnosis and treatment of neurogenic disorders of urination in the group of patients operated for prostatic pathology, the role of the conduction system of the corticospinal tract and conductive potential of perineal muscles in the above patients. Main standard electromyographic parameters were established by the data of examination of 7 controls--latency of the cortical and segmental evoked motor response, time of central motor conduction, amplitude of potentials of motor unities. Fifteen patients with prostatic diseases showed severe disorders of both corticospinal conduction and perineal muscular contraction. Patients with prostatic cancer or adenoma treated with different methods had denervation changes in pelvic inferior muscle in unaffected corticospinal conduction. Denervation changes after surgical treatment for prostatic cancer and delay of reinnervation, recovery of nervous control may be determined not only by the fact of operation but also by the course of the underling disease. Examination of the pelvic inferior muscles demonstrated that HIFU is a traumatic treatment of prostatic cancer because of extention of the physical impact beyond the prostate on muscular-nervous structures. These denervation muscular changes should be taken into consideration in assessment of the patient's condition and choice of treatment policy.

The aim of the study was to investigate the influence of duloxetine on clinical parameters and antinociceptive mechanisms in 46 patients with chronic migraine (CM). In addition to a clinical examination, we performed a neurophysiological investigation which included blink reflex (BR) and nociceptive flexion reflex (NFR) tests. From the beginning of the treatment with duloxetine (60 mg/d) to the third month of treatment, the number of days with headache decreased from 25,8+/-5,3 to 10,5+/-3,9 (p< or =0,001); the frequency of migraine attacks/month decreased from 11,3+/-3,8 to 6,8+/-2,5 (p< or =0,001); the amount of analgesic tablets used per month decreased from 46,6+/-14,7 to 8,5+/-10,6 (p< or =0,001). The reduction of the number of days with headache by more than 50% and more than 30% was noted in 50% and 57,5% of patients, respectively. The treatment with duloxetine resulted in the significant increase of the pain and NFR thresholds as well as in the normalization of the RIII threshold and its habituation in BR. These results confirmed the role of duloxetine in increasing of the noradrenergic and serotonergic activity of brain antinociceptive systems in patients with CM. The clinical effectiveness of duloxetine can be explained by its multilevel modulatory influence on the pathogenetic mechanisms of CM including the activity of antinociceptive systems of the brainstem and of brain nociceptive systems through the decrease of central sensitization.

The objective is to study the effect of dysport injections on the clinical and electromyographic changes in 35 patients (mean age 5,3+/-2,0 years) with spastic forms of cerebral palsy (26 with spastic diplegia, 9 with hemiparetic form) with equinus and equinovarus deformity. Depending on the clinical situation, dysport was injected in a total dose of 20-30 u per 1 kg of the body mass. Gastrocnemius muscles were injected more frequently than soleus and posterior tibial muscles. The treatment resulted in the significant reduction of spasticity on the Ashworth scale, decrease of equinus deformity, positive changes in the parameters of stepping on flat foot, independent standing and walking, the beginning of support period from the heel. During the arbitrary contraction, the amplitude of bioelectrical activity of target muscles of low extremities reduced, though not to the extent of the motor activity loss; the reciprocity coefficient decreased from 0,69+/-0,32 to 0,47+/-0,28 in patients with spastic diplegia and from 0,45+/-0,34 to 0,34+/-0,25 in patients with hemiparetic form. The effect of dysport was higher in hemiparetic form compared to spastic diplegia. The best results for spastic diplegia were revealed in patients with isolated spasticity without severe disturbances of reciprocal relations in shin muscles and pathological synkinesia.

The possibility of central and peripheral impairment of the acoustic analyser was studied in 18 patients with severe diphtheritic polyneuropathy (DP) using brainstem acoustic evoked potentials (BAEPs). The acoustic nerve impairment was found in 27.8%, the central abnormalities--in 44.4%. All the patients with CNS impairment suffered from chronic alcoholism. The data obtained have been compared to those of 26 patients with chronic alcoholism. In this group, peripheral polyneuropathy was confirmed in 76.9% cases; BAEPs revealed isolated involvement of the peripheral part of the acoustic nerve in 7.7% and CNS impairment was found in 84.6% patients. The results of the study suggest that diphtheritic toxin is not implicated in CNS lesions. Central changes found in the BAEPs analysis were related to chronic alcohol intake and did not aggravate diphtheria course.

A complex study of pathophysiological characteristics of corticospinal systems was performed in patients with amyotrophic sclerosis (ALS) by transcranial magnetic resonance stimulation (TMRS) and estimation of the levels of mediatory amino acids in the cerebrospinal fluid (CSF). There was a time course of changes in the excitability of cortical motor neurons (MNs) and in the functional status of the pyramidal tract as ALS progressed. The large cortical MNs were found to be afflicted just at early stages by further involving their axonal systems into the pathological process. As a result, mainly fine slowly conducting axons of MNs functioned at the later stages of the disease. Neurochemical changes correlated with the duration of the disease and were supposed to play an important role in the pathogenesis of ALS. Besides, a significant correlation revealed by TMRS between the pathology of the corticospinal tract and the cerebrospinal fluid levels of glutamate confirmed the role of glutamate as a the main neurotransmitter of this motor system. The results of the study also confirm the significance of excitotoxic mechanisms in central motor disorders in ALS.

A clinicomorphological study was conducted in 11 patients with severe forms of Guillain-Barré syndrome (GBS) at different periods of the disease. Five postmortem cases of GBS were investigated. In all the cases there was a multifocal loss of myelin in the peripheral nervous system with axon degeneration of various degree. Macrophages always took part in demyelination sometimes followed by lymphocytic infiltration. Ultrastructural investigation of nerve biopsies from 6 patients with GBS showed macrophage-associated demyelination with little or no lymphocytic infiltration. It is likely that axonal damage revealed in the biopsies and at the autopsies occurs as secondary consequence of demyelination.

The paper reports three cases of myositis. The findings at detailed electroneuromyographic, morphologic and ultrastructural tests were indicative of characteristic vacuole inclusions in the muscular fibers. Two patients had associated neuritic disorders diagnosed neurophysiologically and morphohistochemically. The neuritic component proved aggravating in the course of the disease. Diagnostic myographic and morphological criteria are analyzed which can distinguish myositis with inclusions from other muscular inflammatory disease.

Changes of the activity potentials of motor units and muscular fibres registered with the aid of needle electrodes are compared with the results of morphological examinations of biopsy specimens of 1,096 muscles taken from 137 patients with myasthenia, combinations of myasthenia with thymoma and polymyositis, as well as from patients with terminal polyneuropathy with the myasthenic syndrome. It has been shown that characteristic of myasthenia are differentiated changes of the motor unit activity potentials: in half of the muscles examined the parameters of those potentials were found to be normal, no polyphasic or protracted potentials were observed. In the patient with combinations of myasthenia with thymoma, polymyositis, and in terminal polyneuropathy with the myasthenic syndrome pronounced changes of all the activity potentials of the motor units were observed in the form of both a sharp shortening and a prolongation of their duration; a great number of polyphasic potentials was registered and spontaneous activity of various degree was observed. Morphological examinations of 55 biopsy specimens of the muscles of the same patients revealed a correlation between the degree of the dystrophic and atrophic changes of the muscular fibres and depth of the affection as determined on the basis of electromyographic findings.

The clinical data and findings of thorough electrophysiological and morphohistochemical examinations of the muscles of 13 patients with the myasthenic syndrome associated with terminal polyneuropathy are presented. Correlation of the clinical and electrophysiological data to the clinical manifestations of the disease and the magnitude of the block of the neuromuscular transmission disclosed a number of characteristic features of the clinical course of the disease (a predominantly oculodistal type of damage, a diminution or the absence of tendon and periosteal reflexes, the presence of atrophies of distal muscular groups) and of the EMG pattern (the presence of enlarged PDDE, an increase in residual latency, the inverse ratio of the decrement of electric and mechanical responses upon indirect stimulation of the muscle at a frequency of 3/sec) and in the histochemical picture (the presence of groupings of type I and II muscular fibrils, which are not characteristic of myasthenia). On the basis of the evidence obtained, a conclusion is drawn that the described syndrome is an independent clinical form rather than a myasthenia variant.

Patients with mitochondrial myopathy have been subjected to clinical, electromyographic, mechanographic, morphological and ultrastructural examination. The main syndromes involving the neuromuscular system in these patients have been considered. The authors draw attention to a generalized character of changes in clinically local lesions. The diagnostic value of electromyographic and morphological examinations has been compared. An analysis of the clinical picture and the results of electromyographic and morphological studies have suggested heterogeneity of the given clinical group of patients.

The density of muscle fibers in a motor unit (MU) was studied electromyographically with the aid of a microelectrode. Muscle biopsy specimens withdrawn from patients with different neuromuscular diseases were subjected to morphological and histochemical studies. During denervation-reinnervation processes, the motoneuron actively reconstitutes the structure of an innervated MU. Apart from branching in the central part, the motoneuron excludes from the innervation zone the muscle fibers located in the most remote areas in respect of the MU center.