A case report of congenital insensitivity to pain and anhidrosis
Keywords:
Congenital, insensitivity, pain, anhidrosis, PakistanAbstract
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disorder characterized by episodes of fever and pain insensitivity despite the fact that all other sensory modalities remain intact or minimally impaired. Other clinical features are self-mutilated behaviour, mental retardation and anhydrosis. We present a case first time from Pakistan, 9- and 4-year-old brother and sister respectively, diagnosed as CIPA clinically with the above-mentioned clinical characteristics.ÂReferences
Nagasako EM, Oaklander AL, Dworkin RH. Congenital insensitivity to pain: an update. Pain. 2003;101:213-9.
Schalka MM, Corrêa MS, Ciamponi AL. Congenital insensitivity-to-pain with anhidrosis (CIPA): a case report with 4-year follow-up. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;101:769-73.
Kouvelas N, Terzoglou C. Congenital insensitivity to pain with anhidrosis: case report. Pediatr Dent. 1989;11:47-51.
Nolano M, Crisci C, Santoro L et al. Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis. Clin Neurophysiol. 2000;111:1596-601.
Erdem TL, Ozcan I, Ilgüy D, Sirin S. Hereditary sensory and autonomic neuropathy: review and a case report with dental implications. J Oral Rehabil. 2000;27:180-3.
Neves BG, Roza RT, Castro GF. Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management. Dent Traumatol. 2009;25:545-9.
Dyck PJ. Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In: Dyck PJ, Thomas PK, Lambert EM (Editors). Peripheral Neuropathy. Philadelfia: WB Saunders; 1984. P.1600-55.
Schulman H, Tsodikow V, Einhorn M, Levy Y et al. Congenital insensitivity to pain with anhidrosis (CIPA): the spectrum of radiological findings. Pediatr Radiol. 2001;31:701-5.
Indo Y, Tsuruta M, Hayashida Y et al. Mutations in the TRKA/NGF receptor gene in patients with congenital insensitivity to pain with anhidrosis. Nat Genet. 1996;13:485-8.
Congenital insensitivity to pain with anhidrosis. Wikipedia. 13 September, 2007
Hefti FF, Rosenthal A, Walicke PA et al. Novel class of pain drugs based on antagonism of NGF. Trends Pharmacol Sci. 2006;27:85-91.
Kriel RL. Abnormalities of sensory perception. In: Swaiman KF, Wright FS, editors. Practice of Pediatric Neurology. Vol 1,St. Louis: CV Mosby; 1982. P.222-31.
Bar-On E, Weigl D, Parvari R, Katz K et al. Congenital Insensitivity to Pain. J Bone Joint Surg Br. 2002;84:252-7.
Sathiaseelan S, Rayar U. The mystery of the broken bones. CMAJ. 2003;169:1189-90.
Hafez HZ, Awad SM. Congenital insensitivity to pain with anhidrosis. Egypt Dermatol Online J. 2007;3:5.
Levy J, Monos T, Levy J et al. Intrasinus wood foreign body causing orbital cellulitis in congenital insensitivity to pain with anhidrosis. Ophthal Plast Reconstr Surg. 2004;201:81-3.
Amano S, Fukuoka S, Usui T et al. Ocular manifestations of congenital insensitivity to pain and anhidrosis. Am J Ophthalmol. 2006;141:472-7.
Rahalkar MD, Rahalkar AM, Joshi SK. Case series: congenital insensitivity to pain and anhidrosis. Indian J Radiol Imaging. 2008;18:132-4.
