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Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report

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 add...

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Autores principales: AZADVARI, Mohaddeseh, EMAMI RAZAVI, Seyedeh Zahra, KAZEMI, Shahrbanoo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Shahid Beheshti University of Medical Sciences 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4885159/
https://www.ncbi.nlm.nih.gov/pubmed/27247588
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author AZADVARI, Mohaddeseh
EMAMI RAZAVI, Seyedeh Zahra
KAZEMI, Shahrbanoo
author_facet AZADVARI, Mohaddeseh
EMAMI RAZAVI, Seyedeh Zahra
KAZEMI, Shahrbanoo
author_sort AZADVARI, Mohaddeseh
collection PubMed
description 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.
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spelling pubmed-48851592016-06-01 Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report AZADVARI, Mohaddeseh EMAMI RAZAVI, Seyedeh Zahra KAZEMI, Shahrbanoo Iran J Child Neurol Case Report 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. Shahid Beheshti University of Medical Sciences 2016 /pmc/articles/PMC4885159/ /pubmed/27247588 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
AZADVARI, Mohaddeseh
EMAMI RAZAVI, Seyedeh Zahra
KAZEMI, Shahrbanoo
Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title_full Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title_fullStr Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title_full_unstemmed Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title_short Hereditary Sensory and Autonomic Neuropathy Type IV in 9 Year Old Boy: A Case Report
title_sort hereditary sensory and autonomic neuropathy type iv in 9 year old boy: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4885159/
https://www.ncbi.nlm.nih.gov/pubmed/27247588
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