Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study

Developmental and degenerative patterns associated with cognitive, behavioural and motor difficulties in the Sanfilippo syndrome: an epidemiological study

Nidiffer F D; Kelly T E

Journal of Mental Deficiency Research

1983

Parents; Family; Male; Child; Humans; Adult; Adolescent; Child Development; Stress Psychological; Female; Child Preschool; Motor Skills; Infant; Child Behavior Disorders; Cognition; Mucopolysaccharidoses; Mucopolysaccharidosis III; behavioral problems; feeding difficulties; urinary incontinence; MPSII; MPSIII; trajectory; characteristics; hyperactivity; sleep disturbance; sleep problems; physical aggression; aggression

The results of a survey given to parents with Sanfilippo syndrome (MPS III) children (n = 30) are described with an emphasis in several areas. Developmental and degenerative patterns are outlined in detail and contrasted with developmental milestones of normal children. An essentially uniform pattern of behaviour problems was discovered and is discussed along with the efficacy of pharmacological and non-pharmacological approach to remediation. Finally, practical suggestions are offered for physicians and parents which are encountered by the daily management problems of children with this disorder.

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