Cerebral atrophy in microcephalic cousins.

Microcephaly is one of the commonest findings in low-grade mental defectives (idiots and imbeciles). It is associated with different forms of encephalopathy. Rarely, it occurs in families; Brandon, Kirman and Williams (pending) found that 114 unselected cases of microcephaly had 166 siblings of whom nine were also microcephalic. However, its occurrence in siblings or other relatives does not necessarily imply the same cause, though, when the nature of the lesions is similar, the possibility of a single disease being involved becomes likely. This was so in the following two children, who presented at necropsy granular and cortical atrophy (ulegyria) of a pattern often thought to follow cerebral birth trauma. However, this could be excluded in one child and was not definitely established in the other. The findings are reported since similar cases with familial association have not been recorded in the literature. Family History The two children, both boys, were first cousins, and gentiles. The father of one (Case 1) and the mother of the other (Case 2) were twins. Enquiry into the family tree (Fig. 1) failed to reveal evidence of similar illnesses in other members. Nine years after the birth of Case 1, a healthy sister