Incidence, etiology, development, EEG, response to ACTH, and follow-up of all cases of infantile spasms diagnosed in Iceland during a 10-year period are reported from the National University Hospital, Reykjavik, Iceland, and Columbia University, New York. In the period 1981 - 1990, 13 cases were identified and the cumulative incidence was 3 in 10,000 live births. Six were cryptogenic and seven were symptomatic in etiology. All had hypsarrhythmia, and all responded initially to ACTH or prednisolone. At follow-up, all in the cryptogenic group are seizure-free and of normal IQ. Those in the symptomatic group are mentally retarded, and 5 have persistent seizures. [1]

COMMENT. The proportion of patients with a favorable outcome is relatively high compared to some studies. The dosage of ACTH was not stated, but the interval from onset of spasms to treatment was short, generally <1 - 3 weeks.

An unusual variant of West syndrome, with focal spasms in clusters and focal delayed myelination, is reported from Nagoya University School of Medicine, Japan [2]. Seizures were controlled with ACTH, and development was normal at 3 yr 5 mos.

Of seventeen infants with visual abnormalities and occipital EEG discharges studied at Tohoku University School of Medicine, Sendai, Miyagi, Japan, two thirds developed West syndrome with hypsarrhythmia at follow-up [3]. This study confirms previous reports of visual inattention as an early manifestation of West syndrome (see Ped Neur Briefs Sept 1993).