hereditary sensory and autonomic neuropathy type iv in 9 year old boy: a case report

how to cite this article: azadvari m, emami razavi sz, kazemi sh. hereditary sensory and autonomic neuropathy type iv in 9 year old boy: a case report. iran j child neurol. spring 2016; 10(2):83-85. abstract objective the hereditary sensory and autonomic neuropathy (hsan) is a rare group of neuropathies that affects the sensory and autonomic nervous system. the patients do not have the ability of sensing different sensations such as pain and temperature, which tends to lead to different injuries. in addition, due to autonomic involvement, the patients suffer from fluctuation in body temperature periodically and lack of precipitation. hsan is divided into 5 types according to the age of onset, clinical features, and inheritance. our case was a 9-yr old boy from cousin parents. he had some developmental delay and history of recurrent fever and convulsion in the first year of his life. gradually, other symptoms added to patient’ feature such as multiple painless skin ulcers, tooth loss, destruction of toes and fingers. in electrodiagnostic study, we found decreased amplitude of sensory nerves, while the other studies were normal. laboratory test and imaging studies were also normal. all clinical and paraclinical findings were in favor of hsan type iv. there is no cure for such patients; as a result, these patients and their families need receiving appropriate education and timely rehabilitation services. references   labib s, adnaneberdai m, abourazzak s, hida m, harandou m. congenitalinsensitivity to pain with anhydrosis: report of a family case. pan afr med j 2011;9:33. amato a, dumitru d. hereditary neuropathies. in: dumitru d, amato a, zwarts m, electrodiagnostic medicine. 2nd ed. philadelphia; hanely&belfus; 2002: 911-914. yuan j, matsuura e, higuchi y, hashiguchi a, nakamura t, nozuma s, sakiyama y, yoshimura a, izumo s, takashima h. hereditary sensory and autonomic neuropathy type iid caused by an scn9a mutation. neurology 2013 apr 30;80(18):1641-9. edvardson s, cinnamon y, jalas c, et al. hereditary sensory autonomic neuropathy caused by a mutation in dystonin. ann neurol 2012;71:569–572. verhoeven k, timmerman v, mauko b, pieber tr, de jonghe p, auer-grumbachm.recent advances in hereditary sensory and autonomic neuropathies. curropinneurol 2006;19(5):474-80. axelrod fb, gold-von simson g. hereditary sensory and autonomic neuropathies: types ii, iii, and iv. orphanet j rare dis 2007;2:39. review. sany-soo lee, sung-h lee, seol-h han. terminal changes in hereditary sensory and autonomic neuropathy. a long term follow-up of a sporadic case. clinical neurology and neurosurgery 2003;105(3): 175-179. kim w, guinot a, marleix s, chapuis m, fraisse b, violas p. hereditary sensory and autonomic neuropathy type iv and orthopaedic complications. orthoptraumatolsurg res 2013;99(7):881-5. safari a, khaledi aa, &vojdani; m. congenital insensitivity to pain withanhidrosis (cipa): a case report. iran red crescent med j 2011;13(2):134-8. gharagozlou m, zandieh f, tabatabaei p,&zamanigh.; congenital sensory neuropathy as a differential diagnosis for phagocytic immunodeficiency. iran j allergy asthma 2006; 5(1): 35-37. baghdadi t ,sadeghifar a , mortazavi s.m.j, &espandar; r . indifference to pain syndrome in a twelve-year-old boy (case report). tehran univ med j 2007; 65 (4) :87-91.