Familial trigeminal anesthesia.
Familial congenital trigeminal anesthesia as an isolated abnormality is an unusual disorder. To our knowledge, only one family has previously been reported. We report here a family with three affected members demonstrating facial anesthesia, bilateral corneal changes, and nasal septal damage secondary to self-traumatization. Magnetic resonance imaging demonstrated hypoplasia of gasserian ganglia and trigeminal nerves in the affected father of two affected sons. The pathogenesis of this disorder appears to be congenital hypoplasia of the trigeminal nerves and gasserian ganglia that is inherited in a dominant fashion.
Keys
C L
CL
Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois, Chicago College of Medicine, IL 60612.
Sugar
J
J
Mafee
M F
MF
D000328:Adult; D002648:Child; D002675:Child, Preschool; D005145:Face / Q000294:innervation; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D010375:Pedigree; D012677:Sensation; D012668:Trigeminal Ganglion / Q000002:abnormalities* / Q000473:pathology; D014276:Trigeminal Nerve / Q000002:abnormalities* / Q000473:pathology