Neuromuscular diseases. Amyotrophic lateral sclerosis
Myasthenia is a disease, caused by lesion of neuromuscular synapse and ismanifested by pathologic muscle fatigue and muscular weakness. Prevalence: 4cases per 100 thousand populations.
Etiology and pathogenesis
. Myasthenia is an autoimmune disease with theformation of antibodies to the postsynaptic membrane of neuromuscularsynapse, which leads to the disturbance of conductivity in the Ger. In 60% of patients with myasthenia is revealed hyperplasia of thymus, in 10% of patients -its tumor (thymoma).Myastenic conduction syndromes are observed in bronchogenic cancer (Lambert-Eaton syndrome), with the use of penicillamine, aminoglucosides (neomycin,gentamicin, Kanamycin and other), streptomycin, polypeptide antibiotics.
Characteristic manifestations of disease are weakness of muscles and their pathologic fatigue. In many cases the disease begins from theoculomotor disturbances, caused by the lesion of ocular muscles and manifestedby doubling and by lowering eyelid (ocular form of myasthenia). In the morning,disturbances can be absent, and considerably increase to evening and with load(prolonged reading ). Less frequently, disease begins from the disturbances of ingestion (dysphagia), speech (dysarthria) and voice (dysphonia) or weakness inthe extremities (peripheral paresis). In some (15%) of patients, manifestation of myasthenia is only [ ostayuts] local, while the rest, it gradually progress, up tothe generalize form of disease. With [raspro] of [straneiii] of weakness to themuscles of extremities, as a rule more greatly suffer the muscles of proximalpart of final fingers, they are frequently scalene muscle of neck. One of to moresevere symptoms of myasthenias is weakness of respiratory muscles,accompanied by suffocation.In myasthenia, rapid worsening state can be observed - myastenic crisisgeneralize by muscular weakness, expressed by dysphagia, dysphonia,dysarthria and respiratory disturbances. Similar to clinical manifestations of cholinergic crisis state as it appears with the overdose of being adapted [dl] of the treatment of anticholinesterase preparations and usually [sonro][vozhdaetsya] by hypersalivation, by [oznobopodobnym] tremor, increasedperspiration, diarrhea, pallor, narrow pupils, bradycardia.
of myasthenia is based on the clinical data and is confirmed by testswith the motor loads, which make it possible to provoke pathologic muscularfatigue. Great diagnostic value has the prozerine test - introduction of S/Cly 1-3ml 0,05% of proserin solution (in the combination with 0,5 ml 0,1%- GO of thesolution of atropine), after which after 20-40 min usually decrease muscularweakness and fatigue during 2-3 h. With electroneuromyography is notedreduction in the amplitude of muscular potentials with the stimulation of nervewith a frequency of 3-10 in second in 75% of patients. Electromyography with astudy of the potentials of separate muscular fibers also possesses highinformativeness. Serological studies make it possible to reveal antibodies to thesynaptic choline receptors in 70% of patients with the ocular form of myastheniaand in 80- 90% of patients with the generalized form. The complete inspection,which includes serological studies, to electroneuromyography andelectromyography, ensures 100% diagnostics. In all patients with myastheniais,X-ray and CT of chest showed the development thymomae or hyperplasias of thymus gland.