The etiology of West syndrome (WS) with special reference to prenatal factors was investigated in 180 patients admitted between 1969 and 1990 to the Department of Child Neurology, Okayama University Medical School, Japan. The most common cause was prenatal, accounting for 77 cases (43%). The frequency of perinatal factors was 14%, and postnatal 7%. Only 18 cases (10%) were idiopathic; in 48 the etiology was uncertain. Among prenatal factors, tuberous sclerosis was most frequent, occurring in 23 (30%) of prenatal and 13% of the total cases. Chromosome abnormalities were found in 10 (trisomy 21 in 6), and cerebral dysgenesis in 10. Seizure remission (47% of cases) and intellectual development (IQ<25 in 65%) were significantly lower in the prenatal group compared to idiopathic cases. Pyridoxal phosphate (100-400 mg daily, orally) tried in 170 cases was followed by seizure remission in 12% of the total, 12% of prenatal cases, and 28% of idiopathic cases. ACTH (0.25 mg/d/im for 26 days) was effective in 89% of 114 cases treated, including 40 (85%) prenatal cases; seizure remission with ACTH was similar in all etiological groups. [1]

COMMENT. In this issue of Epilepsia, several papers refer to West syndrome (WS), 4 from the Neuropaediatric Dept, Hopital Saint-Vincent-De-Paul, Paris: 1) Cusmai R et al, reviewing 32 patients with perinatal insults, found a better prognosis in children with periventricular leukomalacia or localized porencephalic cysts than in those with diffuse cerebral lesions or extensive cysts; 2) van Bogaert P, Dulac O et al report that the MRI in 46 patients with WS of unknown etiology was more informative than CT, demonstrating one case of delayed myelination and 4 with focal lesions; 3) Jambaque I et al found that visual inattention, an early manifestation of WS, was frequently associated with long-term cognitive defects and parieto-occipital abnormalities in SPECT studies; and 4) Plouin P et al, using 24-hour ambulatory EEG monitoring, recorded partial seizures in 31 patients with an unfavorable outcome.

Additional articles on WS in Epilepsia July/Aug 1993;34: include: 5) persisting hypsarrhythmia during a cluster of infantile spasms correlated with a favorable outcome, in a report from the Bambino Gesu Children’s Hospital, Rome, Italy (Fusco L, Vigevano F); 6) the term modified hypsarrhythmia should be discarded, and unusual features of interictal EEGs in infantile spasms should be specified, according to Watanabe K et al, Dept of Pediatrics, Nagoya University, Japan; and 7) infantile spasms, with hypsarrhythmia, retardation and a poor prognosis, occurring in one member of a family with benign familial neonatal convulsions, is a unique case report from Tokushima University, Japan (Mori K, Yano I, Hashimoto T).