[Rett syndrome: individual differences in developmental and behavioral markers and psychosocial stress].
OBJECTIVE
Rett Syndrome is a severe neurological developmental disorder caused by a genetic mutation on the long arm of the X chromosome (Xq28). This disorder is characterized by a loss of purposeful hand use, verbal language, and specific behaviors.


METHODS
Data were obtained from a survey of the parents of 83 girls on pre-verbal skills (Pre-verbal Communication Schedule), behavioral characteristics and emotional expression (Rett Syndrome Behaviour Questionnaire) and on maternal psychosocial stress (Handicap-related Problems for Parents Inventory).


RESULTS
There is considerable individual variability in the developmental and behavioral characteristics which is not explained as a function of age. Maternal stress correlates with the daughters' mood, anxious reactions and night-time behaviors.


CONCLUSIONS
Some implications for diagnosis and family counseling are discussed.
Sarimski
Klaus
K
Kinderzentrum München.
D000203:Activities of Daily Living / Q000523:psychology; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis / Q000235:genetics / Q000523:psychology*; D002675:Child, Preschool; D017281:Cost of Illness; D005260:Female; D006801:Humans; D007206:Individuality; D007805:Language Development Disorders / Q000175:diagnosis / Q000235:genetics / Q000523:psychology; D009035:Mothers / Q000523:psychology*; D009633:Nonverbal Communication; D010552:Personality Assessment; D010641:Phenotype; D015518:Rett Syndrome / Q000175:diagnosis / Q000235:genetics / Q000523:psychology*; D013315:Stress, Psychological / Q000150:complications*