Familial hemifacial spasm associated with arterial compression of the facial nerve. Case report

Abstract

This report of an 88-year-old woman with familial hemifacial spasm includes the first published postmortem description of hemifacial spasm with cross-compression of the seventh cranial nerve root exit zone by a redundant loop of the anterior inferior cerebellar artery and associated vascular plexus. Histological examination of the seventh and eighth cranial nerve complex suggested nerve degeneration because increased numbers of corpora amylacea were present just distal to the compression concavity. There was no evidence of demyelination or gliosis of the nerve. This case suggests that vascular compression of the nerve root exit zone is an important condition in the etiology of most of these cases. This is the third reported case of familial hemifacial spasm; to date, all such patients have had left facial involvement. The family pedigree in this case suggests a pattern of autosomal-dominant inheritance with partial penetrance. The genetic basis for familial hemifacial spasm may involve anatomical variants or anomalies of the posterior circulation, since both posterior inferior cerebellar arteries were congenitally absent in this case.