Long term changes in patients with hypsarrhythmia-infantile spasms: 505 patients, up to 43 years follow-up. 1997

J R Hughes, and I Rechitsky, and Y Daaboul
Epilepsy Clinic, University of Illinois Medical Center, Chicago 60612, USA.

This study involved 505 patients with the EEG pattern of hypsarrhythmia (H) and clinical attacks of infantile spasms (IS) studied over a 51 year period from 1945 until 1996. The total number of EEGs was 1300 and changes in the EEG and type of clinical seizure were followed for up to 43 yrs in a given patient. Although H and IS usually occur together in a given patient, 15% showed a disparity between the electrographic and clinical pattern, usually within a 6-12 mo period. The duration of H was usually < 1 mo but lasted as long as 7 yrs. The duration was dependent in part on the onset age and a very early onset of < 2 mo was associated with a short-lasting H and generally a good prognosis, as was an onset of 8-12 mo. Slightly more than half of these patients had an onset age of > 1 yr, likely in part related to a relatively large number studied before the introduction of the measles, mumps, and rubella vaccine. The next pattern after H was usually around 3 yrs of age, often focal discharges on the occipital or temporal areas or bilateral spike and wave complexes. The major changes in the EEG over time were the progressive increase in bilateral spike and wave complexes and temporal lobe discharges, in addition to an increase in other focal discharges at 6-9 yrs of age. Slow waves on the temporal areas and diffuse slowing became prominent in adulthood. The types of clinical attacks that became prominent in adulthood were generalized tonic-clonic and complex partial seizures. Other types of seizures most often occurred between the late teens and early 20's with absence attacks seen especially at 8-10 yrs of age.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
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
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
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013036 Spasms, Infantile An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8) Cryptogenic Infantile Spasms,Hypsarrhythmia,Infantile Spasms,Jackknife Seizures,Nodding Spasm,Salaam Seizures,Spasmus Nutans,Symptomatic Infantile Spasms,West Syndrome,Cryptogenic West Syndrome,Lightning Attacks,Salaam Attacks,Symptomatic West Syndrome,Attack, Lightning,Attacks, Lightning,Attacks, Salaam,Cryptogenic Infantile Spasm,Hypsarrhythmias,Infantile Spasm,Infantile Spasm, Cryptogenic,Infantile Spasm, Symptomatic,Infantile Spasms, Cryptogenic,Infantile Spasms, Symptomatic,Jackknife Seizure,Lightning Attack,Nodding Spasms,Seizure, Jackknife,Seizures, Jackknife,Seizures, Salaam,Spasm, Cryptogenic Infantile,Spasm, Nodding,Spasm, Symptomatic Infantile,Spasms, Cryptogenic Infantile,Spasms, Nodding,Spasms, Symptomatic Infantile,Symptomatic Infantile Spasm,Syndrome, Cryptogenic West,Syndrome, Symptomatic West,Syndrome, West,West Syndrome, Cryptogenic,West Syndrome, Symptomatic
D017668 Age of Onset The age, developmental stage, or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual. Age-at-Onset,Age at Onset,Onset Age

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