Preview

Neuromuscular Diseases

Advanced search
No 2 (2014)
View or download the full issue PDF (Russian)
https://doi.org/10.17650/2222-8721-2014-0-2

LECTURES AND REVIEWS

6-15 2360
Abstract

Myasthenia gravis is a classic autoimmune disease, which clinical manifestations in the form of weakness and abnormal muscle fatigue, due to the damaging effect of polyclonal antibodies to different structures of the neuromuscular synapse and muscles. The study of autoimmune substrate with myasthenia is routine in many clinics dealing with the problems of neuromuscular pathology, and the identification of high concentration of serum antibodies to a number of antigenic structures is the gold standard in diagnosis.
Determination of serum antibodies to various autoimmune targets is an important tool in clinical practice. The majority of patients shows the high concentration of antibodies to AchR that gives the opportunity to use it as an important diagnostic criterion. The specificity of changes in the concentration of AchR-antibodies due to pathogenetic treatment allows to objectify the suppression of autoimmune aggression and evaluate the reliability of remission. However, the absence of AchR-antibodies when there are clear clinical and electromyography signs of myasthenia gravis suggests an autoimmune attack against a number of other targets, the most studied of which is the MuSK. On the contrary, patients with myasthenia gravis associated with thymoma, almost always have a higher level of AchR-antibodies. The presence of thymoma is accompanied by the generation of antibodies to titin and RyR, which is also observed in persons with late-onset myasthenia without thymoma. High concentration of antibodies to these structures can be interpreted as a reliable sign of thymoma in patients younger than 60 years.

16-26 1208
Abstract

The use of MRI in myopathies dates back to more than 20 years. The first investigations were slow and only allowed segmental and limited studies. Whole-body MRI has emerged over the past twelve years and became a useful diagnostic tool in the etiological diagnosis of myopathies and muscular dystrophies. This study must always be confronted with clinical and whichever other paraclinical data without being able to replace them. Indications to perform such an investigation are getting better and better defined and the diagnostic efficacy has progressed with the increasing number of cases, communications, publications and discussions within multidisciplinary working groups. Its noninvasive nature, the radiation-free exposure and its reasonable cost also enable this technique to be easily accepted by the patient. It also provides a valuable tool for monitoring the natural disease progression or the effectiveness of therapies. The radiology team must be acquainted with the management of neuromuscular patients. Interpreting muscle whole-body MRI requires an excellent knowledge of muscle anatomy whichever body part is examined. The radiologist performing these studies is ideally a specialist of musculoskeletal disorders or a neuroradiologist well trained in muscle anatomy.

CLINICAL REPORTS

27-31 1282
Abstract

The article presents a review of the epidemiology, etiology and pathogenesis of the carpal tunnel syndrome in various rheumatic diseases: rheumatoid arthritis, gout, systemic scleroderma, polymyalgia rheumatica. We also present our own data showing the prevalence of median nerve lesions in patients with rheumatoid arthritis (15 % of 183 patients). The most characteristic clinical feature was the presence of hypoestesia in the area supplied by the affected nerve. All patients with the carpal tunnel syndrome demonstrated neuropathic pain features (numbness, tingling and burning).

32-35 1266
Abstract

Surface sensitivity disorders are observed in many diseases of the central and peripheral nervous system. Surface sensitivity thresholds were estimated in healthy individuals and patients with carpal tunnel syndrome. There was a statistically significant (p < 0.001) increase in the sensitivity threshold in the distal phalanx of the index finger in patients with carpal tunnel syndrome as compared to healthy individuals, by evaluating the surface sensitivity by Semmes–Weinstein monofilaments.

36-41 727
Abstract

Bearing in mind that the technique of surgical treatment of scoliosis and skills are high enough, iatrogenic spinal cord injury is still one of the most feared complication of scoliosis surgery. It is well known that the function of the spinal cord may be estimated by combining somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP). We have retrospectively evaluated the results of intraoperative neurophysiological monitoring (IOM) in a large population of patients underwent surgical treatment of spinal deformity. Intraoperative neuromonitoring SSEP and transcranial electrostimulation (TES) – MEP in conjunction with the assessment of the correct position of the screws was performed in 142 consecutive cases, i. e. all patients who had undergone surgical treatment of idiopathic (127 pts), congenital (10 pts) or neurogenic (5 pts) scoliosis. A neurophysiological “alarm” was defined as a decrease in amplitude (uni- or bilateral) of at least 50 % for SEPs and of 70 % for TES-MEP compared with baseline. Total intravenous anesthesia (TIVA) in 138 cases was achieved by infusion of propofol (8–16 mg / kg / h) and in 4 cases by halogenate anesthesia – sevoflurane (0.4–1.8 MAC). Seven patients (4.9 %) were reported intraoperative neurophysiological parameters significant changes that require action by the surgeons and anesthetists, with deterioration of ostoperative neurologic status in one case. Of these three cases, the amplitude drop SSEPs and TESMEPs-was due, to the pharmacological aspects of anesthetic management, in the other four cases – with surgical procedures (response halo-traction – 1 case, mechanical damage of sheath of the spinal cord by pliers Kerrison – 1case, overcorrection – 2 cases). In five cases (3.5 %) required reposting of pedicle screws (1–2 levels). Only one patient (0.7 %) had a persistent postoperative neurological disorder (neuropathic pain), respectively from a level of re-reposition of pedicle screws.

CONFERENCES, SYMPOSIUMS, MEETINGS



Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2222-8721 (Print)
ISSN 2413-0443 (Online)