Trigeminal nerve involvement in congenital insensitivity of pain with anhidrosis

Öz

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. J Clin Exp Invest 2013; 4 (4): 509-511

Anahtar Kelimeler

• Congenital pain insensitivity, trigeminal involvement, painless corneal epithelial defect

Doğumsal ağrı duyarsızlığı ve trigeminal sinir tutulumu

Öz

Konjenital ağrı duyarsızlığı ve anhidrosis birlikteliği (CIPA) kornea anestezisinin nadir görülen bir nedenidir. Burada, trigeminal anestezi ile ilişkili olarak ağrısız kornea epitel defekti, ciddi kuru göz belirtileri, süt dişlerinin erken kaybı ve üst dudakta ağrısız fissür ve periferik ağrı duyarsızlığı ile ilişkili olarak ekstremitelerde ağrısız yaraları bulunan bir CIPA olgusunu sunmayı amaçladık.

Anahtar Kelimeler

• Doğumsal ağrı duyarsızlığı, trigeminal sinir tutulumu, ağrısız kornea epitel defekti

Kaynakça

1. Sztriha L, Lestringant GG, Hertecant J, Frossard PM, Masouye I. Congenital insensitivity to pain with anhi- drosis. Pediatr Neurol 2001;25:63-66.
2. Nagasako EM, Oaklander AL, Dworkin RH. Congenital insensitivity to pain: an update. Pain 2003;101:213- 219.
3. Amano S, Fukuoka S, Usui T, et al. Ocular manifesta- tions of congenital insensitivity to pain with anhidrosis. Am J Ophthalmol 2006;141:472-477.
4. Yagev R, Levy J, Shorer Z, Lifshitz T. Congenital in- sensitivity to pain with anhidrosis: ocular and systemic manifestations. Am J Ophthalmol 1999;127:322-326.
5. Theodorou SD, Klimentopoulou AE, Papalouka E. Con- genital insensitivity to pain with anhidrosis. Report of a case and review of the literature. Acta Orthop Belg 2000;66:137-145.
6. Ramaesh K, Stokes J, Henry E, Dutton GN, Dhillon B. Congenital corneal anesthesia. Surv Ophthalmol 2007;52:50-60.
7. Mathen MM, Vishnu S, Prajna NV, Vijayalakshmi P, Srinivasan M. Congenital corneal anesthesia: a series of four case reports. Cornea 2001;20:194-196.
8. Schalka MM, Correa MS, Ciamponi AL. Congenital insensitivity-to-pain with anhidrosis (CIPA): a case re- port with 4-year follow-up. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:769-773.

9. Suriu C, Khayat M, Weiler M, et al. Skoura - a genetic island for congenital insensitivity to pain and anhidro- sis among Moroccan Jews, as determined by a novel mutation in the NTRK1 gene. Clin Genet 2009;75:230- 236.