PROGRESSIVE HEMIFACIAL ATROPHY WITH AGENESIS OF THE HEAD OF THE CAUDATE-NUCLEUS

We describe a woman with right hemifacial atrophy, a high palate, part ial left motor seizures, and mild atrophy of the left arm. CT scan sho wed asymmetrical lateral ventricles and MRI (magnetic resonance imagin g) showed atrophy of the right cerebral hemisphere and agenesis of the head of the right caudate nucleus. To our knowledge, this is the firs t report of Parry-Romberg syndrome associated with structural abnormal ities of the basal nuclei documented by MRI. We suggest that a neurova scular aetiology can explain the spectrum of segmental defects associa ted with hemifacial atrophy.