Morphological Aspects of Aetiology and the Course of Infantile Spasms (West-Syndrome)¹

Abstract

The present study using the results of the postmortem examination of twenty-four children, who had infantile spasms (West-Syndrome), gives a view of the aetiology and course of the West-Syndrome. According to the time of occurrence of the lesions three groups could be established: one group of six cases with only embryofetal lesions, one group of ten cases with combined embryofetal and peri/postnatal lesions and one group of eight cases with only peri/postnatal lesions. It is significant, that the time of onset of infantile spasms depends on time of manifestation of lesions. In the groups with combined embryofetal and peri/postnatal lesions the seizures were manifested at the same time as in the cases with isolated embryofetal lesions. Even in the group with combined lesions, microdysgenesis was interpreted as being embryofetal. These embryofetal lesions, as opposed to the peri/postnatal lesions thus appear to dominate and thereby to be pathoplastic. From this correlation two thirds of the cases of infantile spasms can be regarded as fetal epilepsies. The question is open if the infantile spasms which are manifested later on and develop mostly a Lennox-Syndrome indeed should be classified as a separate group together with the isolated peri/postnatal lesions.

Key words

Neuropathology - Infantile spasms - Aetiology - Course of epilepsy - Developmental disturbances