Infantile spasms in siblings
Howitz, P.
Danish Medical Bulletin 27(6): 272-274
1980
ISSN/ISBN: 0907-8916 PMID: 7460634 Document Number: 162542
Nine families with a total of 26 children, 19 of whom had infantile spasms (IS), were investigated. Age at onset of IS, mortality, age of mother and father, EEG at admission, etiology of IS, tissue types (HLA) and the boy/girl ratio of all siblings were analyzed. As to mortality, EEG and etiology, the findings were in accordance with the literature. The tissue types suggest certain differences between the children with IS (high occurrence of HLA-A2) and healthy children. The boy/girl ratio of children with IS was equal to the ratio of total boys/total girls of the 9 families. Whether the children with IS originate from a sibling population with a boy/girl ratio different than that of the normal, healthy population of children (ratio 1/1) is discussed. The 2/1 ratio of IS-boys/girls found in this material is what is usually described in the literature. The accumulation of IS in siblings of certain families, especially the circumstances bearing on a pair of monozygote twins (both IS) and a pair of dizygote twins (only one IS), points towards a genetic background of IS. This is in accordance with a view of IS being the clinical manifestation of a genetically predisposed brain (possible immaturity of structure or enzymes) acted upon by widely different environmental insults.