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01-06-2015 | Review Article

Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature

Authors: D. P. Ashwin, G. D. Chandan, Handa Kaur Jasleen, G. C. Rajkumar, K. B. Rudresh, R. Prashanth

Published in: Oral and Maxillofacial Surgery | Issue 2/2015

Abstract

Hereditary sensory and autonomic neuropathy (HSAN) IV is a rare autosomal recessive disorder which is characterized by a decrease in the number of myelinated and non-myelinated nerve fibers of peripheral nerves which causes diminished or absent pain sensation leading to increase in self-mutilative habits. A retrospective study of eight cases ranging from age group of 4 to 17 years for oral and digital signs and symptoms is presented. All the patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe bite injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa) were found in most patients. Our study suggests that early diagnosis and specific treatment plan are important for prevention of characteristic of the oral as well as digital trauma associated with this disorder.

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Title: Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature
Authors: D. P. Ashwin
G. D. Chandan
Handa Kaur Jasleen
G. C. Rajkumar
K. B. Rudresh
R. Prashanth
Publication date: 01-06-2015
Publisher: Springer Berlin Heidelberg
Published in: Oral and Maxillofacial Surgery / Issue 2/2015
Print ISSN: 1865-1550
Electronic ISSN: 1865-1569
DOI: https://doi.org/10.1007/s10006-015-0486-5

ACC 2024 Congress

Springer Medicine

Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature

Abstract

ACC 2024 Congress

Springer Medicine

Abstract

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