Facial expression of affect in 28 children with intractable infantile spasms was studied longitudinally for 1.8 years after epilepsy surgery, at the Department of Psychiatry, Mental Retardation Research Center, UCLA, Los Angeles. The mean age at onset of spasms was 2.8 mos, surgery was performed at a mean age of 18 mos, and age at last follow-up was 40 mos. Surgery consisted of hemispherectomy in 11 and multilobar resection in 17. The Maximally Discriminate Movement Coding System (MAX) and Early Social Communication Scale (ESCS) were used for measuring discrete facial movement changes related to emotion. Epilepsy surgery was associated with a significant increase in the use of positive affect (surprise, astonishment, joy), irrespective of seizure, AE drug-related, and surgical variables. Children with a right hemispherectomy did not express more positive or negative affect than those with left hemispherectomy. At base-line and 1.8 yrs after surgery, a low rate of negative affect (sadness, anger, discomfort) was expressed. The lateralization (right hemisphere dominance) theory of emotional expression, and the valence theory (both hemispheres involved- left subserving positive emotion and right controlling negative affect) were not supported.

Intractable infantile spasms are associated with reduction in facial expression of positive affect and impaired use of positive emotion during social communication. [1]

COMMENT. In addition to the well known association of autistic behavior with infantile spasms, children with intractable spasms have a reduction in positive affect during social communication. The use of positive affect may be increased following epilepsy surgery, but the relation of this effect to the localization of brain pathology and functional plasticity of facial expression of affect requires further study.

The origin of hypsarrhythmia and tonic spasms in West syndrome is discussed in relation to the report of a 3-year-old girl with porencephaly and hydrocephalus with focal hypsarrhythmia from Tohoku University, Sendai, Japan [2]. The left memisphere was completely defective, and hypsarrhythmia was seen over the residual right frontal cortex. Despite focal EEG findings, tonic spasms were symmetrical, and an intact brainstem appeared to be essential for the occurrence of spasms. An ictal SPECT showed hyperperfusion of the brainstem and cerebellum.