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An apparently normal early development was one of the initial criteria for cla ssical Rett syndrome. However, several investigators considered Rett syndrome to be a developmental disorder manifesting very soon after birth. Videos of 22 Ret t cases were assessed carefully formovements, posture, and behavior during the f irst 6 mo of life. All signs that deviated from the normal standard were recorde d meticulously. Special attention was paid to the face, the hands, and body move ments. A detailed analysis clearly demonstrated an abnormal quality of general m ovements (100%), tongue protrusion (62%), postural stiffness (58%), asymmetri c eye opening and closing (56%), abnormal finger movements (52%), hand stereot ypies (42%), bursts of abnormal facial expressions (42%), bizarre smile (32%) , tremor (28%), and stereotyped body movements (15%). Our study is the first t o apply specific standardized measures of early spontaneous movements to Rett in fants, proving conclusively that the disorder is manifested within the first mon ths of life. Although not necessarily specific, the signs that we have observed will be of value in alerting clinicians to the possibility of the diagnosis at a n early stage, when intervention is likely to be most effective.
An apparently normal early development was one of the initial criteria for classical Rett syndrome. However, several investigators considered Rett syndrome to be a developmental disorder manifesting very soon after birth. Videos of 22 Ret t cases were assessed carefully formovements, posture, and behavior During the f irst 6 mo of life. All signs that deviated from the normal standard were recorde d meticulously. Special attention was paid to the face, the hands, and body move ments. A detailed analysis quite demonstrated an abnormal quality of general m ovements (100%), tongue protrusion (62%), postural stiffness (58%), asymmetri c eye opening and closing (56%), abnormal finger movements (52%), hand stereot ypies expressions (42%), bizarre smile (32%), tremor (28%), and stereotyped body movements (15%). Our study was the first to apply specific standardized measures of early spontaneous movements to Rett in fants, proving conclusively that the diso righteous manifested within the first mon ths of life. when not necessarily specific, the signs that we have observed will be be of value in alerting clinicians to the possibility of the diagnosis at a n early stage, when intervention is likely to be most effective.