Proposal for a standardized protocol for the systematic orofacial examination of patients with Hereditary Sensory Radicular Neuropathy.
OBJECTIVE
To apply a standardized protocol for the orofacial evaluation of two adult siblings (one male and one female) with Hereditary Sensory Radicular Neuropathy (HSRN) that presented with dental problems.


CONCLUSIONS
The systematic evaluation consisted of (a) clinical questionnaire; (b) radiographs [orthopantomography and computarized tomography (CT)]; (c) orofacial psychophysical tests (pain, thermal, mechanical and electrical sensation); and (d) histology of gingiva and pulp (optical and transmission electronic microscopy). The female patient had complete insensitivity to orofacial pain and partial facial heat sensitivity, and received dental treatment without anaesthesia or pain. She had a severe and painless jaw infection due to pulp necrosis in tooth 37. The male patient had partial insensitivity to orofacial pain and required anaesthesia for dental treatment. Histological examination of gingivae and pulpal tissue revealed an altered proportion of unmyelinated and myelinated sensory nerve fibres.


CONCLUSIONS
* Patients with HSRN may present with significant, silent dental disease. * A standard protocol is helpful when evaluating such patients. * If the opportunity arises, evaluation of pulp tissue may reveal an altered proportion of myelinated and unmyelinated nerve fibres. This may avoid the more estabilished sural nerve biopsy.
Siqueira
S R D T
SR
Division of Dentistry, Hospital das Clínicas, Orofacial Pain Clinic, Medical School, University of Sao Paulo, Brazil. silviadowgan@hotmail.com
Okada
M
M
Lino
A M M
AM
Teixeira
M J
MJ
Siqueira
J T T
JT
D000293:Adolescent; D000328:Adult; D017742:Dental Care for Chronically Ill / Q000592:standards*; D005145:Face / Q000294:innervation; D005157:Facial Pain / Q000175:diagnosis; D005260:Female; D006261:Headache / Q000175:diagnosis; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D006987:Hypesthesia / Q000175:diagnosis; D008297:Male; D008487:Medical History Taking; D009061:Mouth Mucosa / Q000294:innervation; D000699:Pain Insensitivity, Congenital / Q000175:diagnosis; D010808:Physical Examination; D012684:Sensory Thresholds / Q000502:physiology; D014070:Tooth / Q000294:innervation