flaccid baby syndrome - Congenital muscular hypotonia any genesis. The term comprises a group of diseases of different etiologies, each of which appears a decrease of muscle tone, often from birth. Also may experience frustration swallowing and breathing, convulsions and impaired consciousness. flaccid baby syndrome diagnosed by a characteristic posture of the patient, requires instrumental confirmation muscular hypotonia. In order to determine the etiology of the syndrome is conducted MRI diagnostics, laboratory examination, genetic mapping, and other studies. Treatment is symptomatic and pathogenetic apply neyrometabolicheskie means of mechanical ventilation.
Syndrome flaccid child
neurologist Greenfield, although cases of congenital muscular hypotonia were described at the beginning of the XX century. Polietiologichesky nature of the condition makes sluggish baby syndrome one of the most urgent problems of Pediatrics. Today there are more than 80 clinical entities, one symptom of which is a progressive decrease in muscle tone. The vast majority of these diseases have an unfavorable course, providing an overall high rate of mortality.
sepsis and meningitis, intrauterine infections, and hereditary chromosomal disorders, organic aciduria, and other genetic metabolic disorders. Peripheral muscular hypotension associated with myocyte damage usually system. It is a congenital muscular dystrophies and myopathies. A striking example of this group of diseases is spinal muscular atrophy Verdninga-Hoffmann.
There is a classification of the causes of sluggish baby syndrome, according to which the disease can be neurological and somatic etiology. Obviously, the first group includes all causes a neurological disease of the central nervous system and peripheral nerves. Of Somatic Diseases that cause muscle hypotonia at an early age can be identified metabolic abnormalities, intrauterine infections and sepsis of newborn, congenital heart disease, nutritional disorders, and so on. D. Thus, flaccid baby syndrome may be associated with various conditions that lead to a breach of regulation of muscle tone at any level of the nervous system.
congenital myopathies muscular hypotonia, on the contrary, is progressing. Eventually weakness observed not only in limb muscles, but also in the respiratory muscles and the muscles involved in swallowing. The child begins to experience difficulties with eating, can be shortness of breath and sleep apnea attacks.
pediatrician. The characteristic posture and appearance described above. In addition to the visual inspection conducted neurological tests such as traction of the handles in the supine position, the horizontal and vertical hanging. The baby's head while hanging, the tone of the hands and feet visually reduced. Electroneuromyography makes it possible to detect a sluggish child syndrome of peripheral origin. MRI reveals atrophy in certain brain regions. Laboratory diagnosis exclude infectious causes. Also be sure to examination of the heart. Genetic mapping is carried out to confirm the chromosomal abnormalities, which include Down's syndrome and hereditary metabolic disorders.
Specific treatment is not developed. The positive effect may have application energotonik neurometabolic and drugs, but the improvement of a minor or transitory. Most require oxygen support, use of mechanical ventilation. Most diseases presented in clinic syndrome flaccid child progress rapidly or slowly, but for usually irreversible. Therefore poor prognosis. High mortality in the first months and years of life. Lethal outcomes are often associated with weakness of the respiratory muscles, as well as the manifestations of the underlying disease - septic complications, metabolic disorders, etc.