Vol 2, No 4 (2012)

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.

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.

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.

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.