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Familial trigeminal anesthesia.

C L Keys1, J Sugar, M F Mafee

  • 1Department of Ophthalmology, Eye and Ear Infirmary, University of Illinois, Chicago College of Medicine, IL 60612.

Archives of Ophthalmology (Chicago, Ill. : 1960)
|December 1, 1990
PubMed
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Familial congenital trigeminal anesthesia is a rare disorder causing facial numbness and self-inflicted injuries. This study identifies a new family with this condition, linked to congenital nerve hypoplasia.

Area of Science:

  • Neurology
  • Genetics
  • Ophthalmology

Background:

  • Familial congenital trigeminal anesthesia is an exceptionally rare disorder.
  • Previous reports of this condition are limited, with only one family documented.

Observation:

  • This report details a family with three individuals exhibiting congenital trigeminal anesthesia.
  • Affected members presented with facial anesthesia, corneal damage, and nasal septal injuries due to self-traumatization.
  • Magnetic resonance imaging revealed hypoplasia of the gasserian ganglia and trigeminal nerves in affected individuals.

Findings:

  • The disorder is characterized by congenital hypoplasia of the trigeminal nerves and gasserian ganglia.
  • Inheritance patterns suggest a dominant mode of transmission within the family.

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Implications:

  • This study expands the known cases of familial congenital trigeminal anesthesia.
  • Understanding the genetic basis and pathogenesis is crucial for diagnosis and potential management.
  • Highlights the link between congenital nerve abnormalities and secondary self-inflicted trauma.