Abstract
An electroencephalographic-video study of 14 benign neonatal familial convulsions (BNFCs) in 3 children from two families is reported from the Hopitaux Universitaires, Strasbourg, and the Institut de Biologie, Montpellier, France.
Highlights
The MRI, the increased recognition of cerebral dysgenesis as a frequent cause for infantile spasms has resulted in a lowered percentage of cryptogenic cases compared to earlier reports
Posterior fossa abnormalities in children with infantile spasms were associated with a poor developmental outcome in a study of 98 children from New England Medical Center and Children's Hospital, Boston, and Hadassah University Hospital, Jerusalem (Schiffmann R et al T Child Neurol Oct 1993;8:360-365)
Brain stem dysfunction and atrophy were remarkable in a study of 10 patients with infantile spasms, employing evoked potentials and MRI, at the University of Tokushima, Japan (Miyazaki M et al Neuropediatr Tune 1993:24:126-130)
Summary
The MRI, the increased recognition of cerebral dysgenesis as a frequent cause for infantile spasms has resulted in a lowered percentage of cryptogenic cases compared to earlier reports. The anticonvulsant efficacy and outcome of treatment with ACTH in 21 patients was related significantly to the age at the time of diagnosis and treatment; 80% of infants less than 1 year old were benefited whereas only 22% of those over 1 year showed reduction in seizures and EEG improvement. Control of seizures and hypsarrythmia was not significantly related to the etiology, but patients of borderline or normal intelligence tended to respond more frequently than did those with developmental quotients
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