Hemifacial spasm or subcortical epilepsy?

A child with Goldenhar's syndrome presented at about one week of age with s tereotyped, repetitive paroxysmal episodes of hemifacial contraction, sugge stive of partial seizures or hemifacial spasm. Later in life he also develo ped independent, permanent abnormal eye and chin movements identical in rhy thm to those seen in myorhythmias, suggesting involvement of the dentato-ol ivary pathway. MRI demonstrated a hamartomatous lesion at the level of the pontomedullary junction. We speculate that the nature of the lesion could b e responsible for the partial seizures mimicking hemifacial spasm and that because of its location, this same lesion could also be implicated in the g enesis of myorhythmias. The presence of a hamartomatous lesion in a region affected by the abnormal development of the first and second branchial arch es is not fortuitous.