Trigeminal nerve involvement in congenital insensitivity of pain with anhidrosis

Abdullah Kürşat Cingü,Seyhmus Arı,Yasin Çınar,İhsan Çaça,Alparslan Şahin,Muhammed Şahin

doi:10.5799/ahinjs.01.2013.04.0335

Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare cause of corneal anesthesia. Here, we report a case of CIPA with clinically prominent features of severe dry eye symptoms with painless corneal epithelial defect, early loss of primary teeth and a painless fissure on her upper lip in association with trigeminal anesthesia, and painless wounds on extremities.

Highlights

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare cause of corneal anesthesia
Despite a large central corneal epithelial defect, she seemed very comfortable and there was no sign of foreign body sensation
Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare autosomal-recessive disease of the nervous system which is one of the hereditary

Summary

Introduction

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare cause of corneal anesthesia. She had multiple painless lesions on her extremities as well as a previously healed painless fracture on her right arm. The family was familiar to this event because of their 10 years old son who experienced early loss of deciduous teeth and fractures. In her right eye approximately 2x4 mm fluorescent stained central corneal epithelial defect, superficial punctate keratopathy (SPK), and diminished tear break-up time were found.

Results

Conclusion