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Neuromuscular Diseases

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No 4 (2012)
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https://doi.org/10.17650/2222-8721-2012-0-4

LECTURES AND REVIEWS

8-18 1053
Abstract

The review briefly describes the main ways of rheography development. Particular emphasis is laid on the practically forgotten and newly revived method of the harmonic analysis of rheograms. The development of electronics and computed methods of data processing could unveil the potential of this direction of rheography and consider prospects for developing new diagnostic approaches on the basis of a multicyclic harmonic analysis of bioimpedance.

19-27 1271
Abstract

The task of this paper is to familiarize practicing neurologists, neurosurgeons, traumatologists, and orthopedists with the current principles of diagnosis and treatment of different brachial plexus injuries (BPI). The anatomy of the brachial plexus is described in detail; the main mechanisms of its injuries are considered and their current classification is given. Particular emphasis is laid on the traction-based mechanism of BPI and its basic treatment in a group of patients who require (need) surgical treatment. The capabilities of various instrumental techniques, such as X-ray, neuroimaging, electrophysiological, in the diagnosis of BPI are considered. The author’s algorithm for a differential diagnostic search is proposed in this pathology.

ORIGINAL REPORTS

36-42 1227
Abstract

In the article we present the results of the retrospective clinico-electrophysiological analysis of 182 patients suffering from the idiopathic neuropathy of the facial nerve (Bell`s palsy). The comparison of the most common electromyographical (ENMG) predictors of outcomes was made. It was demonstrated that the most sensitive method in the acutest period (less then 5 days) is the level of excitability of the nerve, in the acute period (less then 14 days) – estimation of M-answer amplitude loss, and from the 21st day – the presence of denervation in muscles. The most specific electromyographical approach to estimate the therapy efficiency is an analysis of the M-answer amplitude and latency. In conclusion, neurologists have the possibility to predict the outcome and to control the therapy efficiency in any period of the disease. The correlation dynamics ÈNMG sensitivity settings – NLN on different dates can be used to determine the volume of ÈNMG – the NLN study depending on the timing for the treatment of patients.

43-52 1158
Abstract

The motor stereotype in patients with cerebral palsy (CP) is determined by the magnitude of spasticity and central paresis of muscles, the impaired mechanisms of intermuscular interaction, and the presence of consensual reactions and pathologic synkineses. There are cases of a concomitance of the suprasegmental and segmental structures damages in CP. An attempt has been made to identify and estimate the contribution of the peripheral neuromuscular system lesion in CP patients in its late residual stage. Central nervous system lesion at the central and segmental levels were detected in 12.3% of cases in CP with spastic diplegia with progressive deformities of the lower extremities joints. Needle EMG is the most sensitive technique to detect a lesion at the segmental level: increased duration and higher amplitude motor unit potentials (MUPs) and fewer recruited MUPs were registrated. A turn-amplitude analysis has indicated the shift of cloudy diagram to the left and upwards and the decreased ratio of the number of turns to their mean amplitude. The residual pattern of revealed changes is confirmed by the absence of signs of a current denervation process. The contribution of possible myelodysplasia and transneuronal degeneration of spinal motor neurons at the lumbosacral level to the clinical picture of the CP and orthopedic surgery and rehabilitation tactics are discussed.

CLINICAL DISCUSSION

53-58 1244
Abstract

A72-year old woman and 38-year old man had clinical sings of myasthenia gravis with ptosis and double vision, speech, swallowing, face and limb weakness. They was diagnosed MG after tensilon test, responding to piridostigmine and prednisolon treatment, neuromuscular
disorders and positive anti-AchR and titin antibodies. Four and 7 year ago after debut of myasthenia gravis they had clinical and electrophysiological symptoms of amyotrophic lateral sclerosis.

59-65 890
Abstract

The case of lumbosacral motor neuropathy (LSMN) in 15-yers old patient with diabetes mellitus (type I) is presented. Clinical and electromyographical patterns are considered and effectiveness of corticosteroid therapy is estimated. The differential features and taxonomic position of LSMN and chronic inflammatory demyelinating polyneuropathy (CIDP) are discussed. The necessity of some liberalization of CIDP diagnostic criteria is demonstrated.

66-73 12782
Abstract

Pompe disease is a rare inherited disease that belongs to lysosomal accumulation diseases and can be considered as cardiac glycogenosis
type II, as well as a severe neuromuscular disease or metabolic myopathy. Physicians of different specialties very rarely identify this pathology, which is due to both its rarity and clinical and genetic polymorphism. Infantile Pompe disease is the severest form. It is characterized by a progressive pattern and a fatal outcome during the first year of life. The possibility of performing enzyme replacement therapy for this disease, which can improve the prognosis and quality of life of patients, makes the early diagnosis of Pompe disease urgent. The paper describes the clinical presentation of infantile Pompe disease and current methods for its diagnosis and treatment. The authors give their experience in diagnosing and treating infantile Pompe disease, by demonstrating 3 cases of the disease. The characteristics of each infant, which confirm the clinical and genetic variety of this pathology, are discussed.

CONFERENCES, SYMPOSIUMS, MEETINGS

DISTINGUISHED RUSSIAN NEUROLOGISTS



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