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Journal of Child Neurology
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Familial Idiopathic West Syndrome

Edith Reiter, MD

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria

Monika Tiefenthaler, Mag

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria

Michael Freilinger, MD

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria

Günther Bemert, MD

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria

Rainer Seidl, MD

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria, rainer.seidl{at}akh-wien.ac.at

Erwin Hauser, MD

University Hospital Vienna, Department of Pediatrics, Division of Pediatric-Neurology, Vienna, Austria

Two families, each with occurrence of West syndrome in two siblings, are presented. Monozygotic twins in family 1 developed infantile spasms at the age of 4 months. Two female siblings in family 2 started to have seizures at the age of 6 months, but 2 years apart. The family history; development prior to West syndrome; clinical, electroencephalographic, and neuro-radiologic findings; diagnostic work-up; and treatment are described. The outcome in family 1 (follow-up after 2 years) showed no conspicuous findings on physical and neurologic examination, and psychomotor development appropriate to cognitive, motor, and language developmental age in both twins. In family 2 (follow-up after 3 and 5 years), the older sister only was one standard deviation below mean in intellectual developmental age. Simultaneous occurrence of infantile spasms in both siblings from these two families but with variable clinical expression suggests there is a genetic susceptibility and variable phenotypic expression. Long-term follow-up will demonstrate whether these cases may be classified as "familial idiopathic West syndrome." (J Child Neurol 2000;15:249-252).

Journal of Child Neurology, Vol. 15, No. 4, 249-252 (2000)
DOI: 10.1177/088307380001500409
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