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Cognitive deficits after cryptogenic infantile spasms with benign seizure evolution

Published online by Cambridge University Press:  01 October 1999

Eija Gaily
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Kati Appelqvist
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Elisa Kantola-Sorsa
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Elina Liukkonen
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Paula Kyyrönen
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Mia Sarpola
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Heli Huttunen
Affiliation:
Epilepsy Unit, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Leena Valanne
Affiliation:
Department of Radiology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
Marja-Liisa Granström
Affiliation:
Department of Neurology, Helsinki University Central Hospital, Hospital for Children and Adolescents, Finland.
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Abstract

Between 1989 and 1994, 18 children with cryptogenic infantile spasms – defined by normal development before onset of spasms, symmetrical hypsarrhythmia or multifocal spikes, and typical spasms on presentation, and no abnormal findings on aetiological studies including neuroradiology – were diagnosed and treated. To assess the risk of cognitive impairment later in life, 15 of these 18 children whose spasms completely resolved within the first year of life were studied. Age at onset of spasms varied between 4.4 and 9.8 months (mean 6.5 months). Children were effectively treated with adrenocorticotrophic hormone (10 children), pyridoxine (three), vigabatrin (one), or sodium valproate (one). Spasms lasted between 11 and 138 days (mean 50 days) and stopped between the age of 6.3 and 10.2 months (mean 8.1 months). EEGs normalized between the age of 7.1 and 13.2 months (mean 9.4 months). Early development was assessed on presentation and within a few months after spasms had stopped. A detailed neuropsychological assessment was performed between the age of 4.0 and 5.9 years. Twelve children had normal intelligence; specific cognitive deficits were found in five. Three children had mild learning disability. Abnormal developmental status at age 8 to 15 months after complete resolution of spasms and EEG abnormalities was associated with cognitive deficits at age 4 to 6 years.

Type
Original Articles
Copyright
© 1999 Mac Keith Press

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