West syndrome and pschycomotor retardation

Abstract

We have read the research titled “Autistic disorder in West syndrome” by Hançerli et al. (1) with interest. As mentioned in the introduction of the article, the three main characteristics of West syndrome include :1) infantile spasms, 2) regression and retardation in psychomotor development and 3) appearance of hypsarryhtmia on electroencephalogram. It is understood that regression and retardation was present in psychomotor development in 100% of 267 subjects who were diagnosed with West syndrome and in 90 subjects who were included in the research from this group. In approximately 95% of the subjects, seizures began in the first 12 months of life and 90 of these were examined after the age of three years. Psychomotor retardation was found in 78 subjects (87%) as a result of examination performed by the Denver developmental screening test. Thus, the rate of regression and retardation in psychomotor development which was 100% at the baseline was found to be decreased to 87% in approximately two years. Improvement of the psychomotor state with treatment in a severe condition like West syndrome is a significant success. It is known that cases with seizures assessed to be infantile spasms which are one of the three characteristics of West syndrome used to be divided into three groups (symptomatic, cryptogenic and idiopathic). Currently, however, the cases are classified mainly in two groups (symptomatic and cryptogenic) (2). The subjects in whom the cause leading to seizures can be demonstrated and/ or who have significant developmental retardation before seizures start are qualified as symptomatic subjects. The subjects in whom the cause is not known and who have normal development before seizures start are named as cryptogenic subjects (2,3). Approximately 20% of the patients are in the cryptogenic group and 80% are in the symptomatic group. The patients in the cryptogenic group have a better general well-being (4). Only 16% of the subjects with infantile seizures who had a mean followup period of 31 months had normal development in 67 studies published (3). In another article, it was reported that retardation in development was observed with a rate of 50% even in cryptogenic subjects who responded to treatment in a short time (5). It is known that medical and surgical treatment in epilepsy may affect cognition positively (6-8). In fact, it was reported that even epilepsy treatment administered in patients who had cognition disorders and EEG disorders without seizures had useful effects on cognition (9). Improvement in cognition in infantile spasm has not been mentioned much except for papers which reported improvement in cognition and behavior with vigabatrin treatment, when the cause of infantile spasm was tuberous sclerosis (10). Therefore, these results in the study of Hançerli et al. (1) are pleasing in terms of the prognosis of the disease, though the reasons were not expalined. In addition, it will be useful to correct the contradictions related with some information in the “Summary” and “Results” parts of the study. In the summary, it was reported that psychomotor retardation was found in 86% of 267 subjects with West syndrome. On the other hand, it was stated that psychomotor retardation was found in 78 (87%) of a total of 90 subjects (not 267 subjects) with the Denver developmental screening test in the“Results” part.