Syndrome of the sluggish child
Contents:
Description:
The syndrome of the sluggish child ("floppy baby", floppy infant) is observed at various diseases which main display is sharply expressed hypomyotonia and lag in motive development. A number of authors identify this syndrome with a so-called amyotonia of Oppengeym now.
Reasons of a syndrome of the sluggish child:
The reason causing a syndrome of "the sluggish child", more than in 60% of cases is the early form of a disease of Verdniga - Goffmanna. In considerable number of cases benign inborn hypotonia, and also not crimson, miotubulyarny or mitochondrial myopathy takes place. Quite often the syndrome of "the sluggish child" is caused by an atonic form of cerebral palsy and a severe form of rickets. It can be observed at a Down syndrome, Marfan's syndrome, at an injury of a spinal cord. Cases of various metabolic disturbances which are followed by sharply expressed hypotonia, for example, pathology of exchange of amino acids, a galactosemia, a generalized form of a glycogenosis are described. At some forms of metabolic disturbances perhaps rational treatment (correction of exchange with the help of special diets at a fenilketonuriya, a galactosemia), use of biotin at leucine amino acid exchange disturbance
Symptoms of a syndrome of the sluggish child:
At a syndrome of "the sluggish child" of a muscle at a palpation the quiet, passive movements in joints excessively free, "the frog pose" with the taken-away hips and rotirovanny feet of a knaruzha is characteristic.
At an atonic form of cerebral palsy the atony results from defeat not only big hemispheres, but also a cerebellum. Such children have an expressed disturbance of intelligence and unlike the spinal or muscular level of defeat it is possible to cause tendon jerks. At observation of patients in dynamics they have signs of a hyper tone and extrapyramidal hyperkinesias.
The inborn form of spinal amyotrophy of Verdniga - Goffmanna is characterized by almost total absence of spontaneous movements, respiratory muscles suffer early. Inborn muscular dystrophy is followed by accurate increase in enzymes in blood serum, especially kreatinfosfokinaza. Rare options of an inborn myasthenia are characterized by diffusion muscular weakness without rough hypotonia, and also participation of bulbar and facial muscles. The beneficial effect of antikholinesterazny drugs is especially characteristic, as gives the chance to specify the diagnosis. Inborn benign hypotonia differs from other forms in existence of spontaneous movements and tendon jerks and usually lack of bulbar frustration. EMG and definition of activity of enzymes in blood serum do not reveal aberrations.
Treatment of a syndrome of the sluggish child:
Treatment and the forecast at a syndrome of "the sluggish child" depend on a specific nosological form. Persistent search of the correct diagnosis with use of electrophysiologic, thin biochemical and pathomorphologic methods is necessary.
