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September 1969

Massive Spasms Associated With Holoprosencephaly

Am J Dis Child. 1969;118(3):520-524. doi:10.1001/archpedi.1969.02100040522020

MASSIVE spasms, hypsarhythmia, and developmental retardation comprise a triad of clinical findings occurring only in infancy and early childhood. The triad has been associated with a wide variety of cerebral disorders.1-3 Massive spasms are an age-specific response pattern of the infant's brain. Older children and adults do not exhibit massive spasms or a hypsarhythmic pattern in the electroencephalogram under the same circumstances. Brain malformations have been associated with this triad in less than 10% of pathologically verified cases reported to date. We had an opportunity to study an infant who exhibited massive spasms, hypsarhythmia, and developmental retardation, as well as diabetes insipidus. Fractional pneumoencephalography revealed that she has lobar holoprosencephaly. The case is reported because it is the first such association to be documented

Report of a Case  The patient, a female infant, was admitted to the University of Washington Hospital at the age of 5½ months following a

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