Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature.
Oral Maxillofac Surg
; 19(2): 117-23, 2015 Jun.
Article
in En
| MEDLINE
| ID: mdl-25744033
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Hereditary Sensory and Autonomic Neuropathies
Type of study:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
/
Screening_studies
Limits:
Adolescent
/
Child
/
Child, preschool
/
Humans
Language:
En
Journal:
Oral Maxillofac Surg
Journal subject:
ODONTOLOGIA
Year:
2015
Document type:
Article
Affiliation country:
India