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Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis.

Abstract

OBJECTIVE

Hereditary sensory and autonomic neuropathy type IV (congenital insensitivity to pain with anhidrosis) is a rare disorder. In this study, we investigated the oral and dental manifestations associated with hereditary sensory and autonomic neuropathy type IV.

STUDY DESIGN

Eighteen patients with hereditary sensory and autonomic neuropathy type IV whose ages ranged from 1 year 0 months to 22 years 3 months were examined for oral signs and symptoms of tooth abnormalities, malocclusions, soft tissue disorders, tongue papilla atrophy, and morphologic abnormalities of hands and fingers.

RESULTS

All 18 patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe biting injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa), were found in most patients. In infant patients the condition was typically characterized by decubital ulcers on the ventral surface of the tongue, resulting from trauma of the incisal edge of erupting mandibular primary incisors during sucking or nursing. These ulcers led to several local and systemic problems, such as tongue bleeding, infection, malnutrition, and halitosis. A large number of missing teeth and a high incidence of dental caries were additional characteristic findings. Such oral self-mutilations were found to decrease with age and with the intellectual, social, and/or emotional development of the patients. However, not all of the mutilations were completely eliminated. Two patients had partial dentures to replace missing teeth.

CONCLUSIONS

Our study suggests that early diagnosis and specific dental management for patients with hereditary sensory and autonomic neuropathy type IV are important for prevention of the characteristic oral and dental problems accompanying this disorder.

Authors+Show Affiliations

Division of Special Care Dentistry, Osaka University Faculty of Dentistry, Japan.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

9798226

Citation

Amano, A, et al. "Oral Manifestations of Hereditary Sensory and Autonomic Neuropathy Type IV. Congenital Insensitivity to Pain With Anhidrosis." Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, vol. 86, no. 4, 1998, pp. 425-31.
Amano A, Akiyama S, Ikeda M, et al. Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86(4):425-31.
Amano, A., Akiyama, S., Ikeda, M., & Morisaki, I. (1998). Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics, 86(4), pp. 425-31.
Amano A, et al. Oral Manifestations of Hereditary Sensory and Autonomic Neuropathy Type IV. Congenital Insensitivity to Pain With Anhidrosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;86(4):425-31. PubMed PMID: 9798226.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Oral manifestations of hereditary sensory and autonomic neuropathy type IV. Congenital insensitivity to pain with anhidrosis. AU - Amano,A, AU - Akiyama,S, AU - Ikeda,M, AU - Morisaki,I, PY - 1998/11/3/pubmed PY - 1998/11/3/medline PY - 1998/11/3/entrez SP - 425 EP - 31 JF - Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics JO - Oral Surg Oral Med Oral Pathol Oral Radiol Endod VL - 86 IS - 4 N2 - OBJECTIVE: Hereditary sensory and autonomic neuropathy type IV (congenital insensitivity to pain with anhidrosis) is a rare disorder. In this study, we investigated the oral and dental manifestations associated with hereditary sensory and autonomic neuropathy type IV. STUDY DESIGN: Eighteen patients with hereditary sensory and autonomic neuropathy type IV whose ages ranged from 1 year 0 months to 22 years 3 months were examined for oral signs and symptoms of tooth abnormalities, malocclusions, soft tissue disorders, tongue papilla atrophy, and morphologic abnormalities of hands and fingers. RESULTS: All 18 patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe biting injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa), were found in most patients. In infant patients the condition was typically characterized by decubital ulcers on the ventral surface of the tongue, resulting from trauma of the incisal edge of erupting mandibular primary incisors during sucking or nursing. These ulcers led to several local and systemic problems, such as tongue bleeding, infection, malnutrition, and halitosis. A large number of missing teeth and a high incidence of dental caries were additional characteristic findings. Such oral self-mutilations were found to decrease with age and with the intellectual, social, and/or emotional development of the patients. However, not all of the mutilations were completely eliminated. Two patients had partial dentures to replace missing teeth. CONCLUSIONS: Our study suggests that early diagnosis and specific dental management for patients with hereditary sensory and autonomic neuropathy type IV are important for prevention of the characteristic oral and dental problems accompanying this disorder. SN - 1079-2104 UR - https://www.unboundmedicine.com/medline/citation/9798226/Oral_manifestations_of_hereditary_sensory_and_autonomic_neuropathy_type_IV__Congenital_insensitivity_to_pain_with_anhidrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-2104(98)90368-7 DB - PRIME DP - Unbound Medicine ER -