A long-term follow-up study of 214 children with the syndrome of infantile spasms. 1982

R Riikonen

The long-term prognosis of 192 surviving children with the syndrome of infantile spasms was evaluated. The children had been admitted to three paediatric hospitals in Helsinki at the time of initial diagnosis. The aetiological factors of the syndrome were carefully studied in each case. ACTH therapy was employed in 162, usually for about six weeks. The follow-up study 3-19 (mean 10.4) years later was made at the Children's Hospital, University of Helsinki. The rate of mortality was 19.6 per cent. Normal development was seen in 12 per cent and slightly subnormal in 10.4 per cent of the surviving children. Psychiatric disorders were seen in 27.6 per cent of the survivors. Sensory defects were also common. Severe cerebral palsy was seen in 4 per cent. Other seizures after cessation of the infantile spasms were seen in 60 per cent. Serial EEG studies showed that the temporal lobe was the most common site of abnormality. Abnormalities in the temporal lobes were seen frequently in children with symptomatic neonatal hypoglycaemia as a probable cause of the spasms. Prognostically favourable factors were "idiopathic" aetiology, normal development and not other fits prior to the spasms, short treatment lag, good response to ACTH and short duration of the spasms. In this study early treatment seemed to be of great importance even with regard to mental development. The factors connected with a bad outcome were: symptomatic aetiology (especially brain malformations, early infections and tuberous sclerosis), slow development before spasms, other seizures before infantile spasms, early onset of the spasms, long treatment lag, long duration of the spasms and other later occurrence of myoclonic-astatic seizures (Lennox-Gastaut). Large doses of ACTH (120-160 units) were not associated with a better prognosis than the smaller doses (20-40 units). The benefit of long versus short treatment schedules could not be evaluated in this study. The relapse rate here was 32 per cent.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D008607 Intellectual Disability Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28) Disability, Intellectual,Idiocy,Mental Retardation,Retardation, Mental,Deficiency, Mental,Intellectual Development Disorder,Mental Deficiency,Mental Retardation, Psychosocial,Deficiencies, Mental,Development Disorder, Intellectual,Development Disorders, Intellectual,Disabilities, Intellectual,Disorder, Intellectual Development,Disorders, Intellectual Development,Intellectual Development Disorders,Intellectual Disabilities,Mental Deficiencies,Mental Retardations, Psychosocial,Psychosocial Mental Retardation,Psychosocial Mental Retardations,Retardation, Psychosocial Mental,Retardations, Psychosocial Mental
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D005260 Female Females
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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