MICROCEPHALY WITH CHORIORETINOPATHY - A REPORT OF 2 DOMINANT FAMILIESAND 3 SPORADIC CASES

This is a report of seven new cases of microcephaly with chorioretinop athy. Three cases were sporadic and four were dominant: a father and s on, and a father and daughter, Their ophthalmological, neurological, a nd systemic findings are discussed as are the genetics of the syndrome . Chorioretinopathy with characteristic punched-out lesions was observ ed in both entities, Body height emerges as a possible distinguishing feature between the dominant and recessive forms. In addition, locomot or disturbances are more frequently seen in patients with the recessiv e form.