Oral and maxillofacial manifestations of hereditary sensory neuropathy.
Hereditary sensory neuropathies are a rare group of neurological disorders manifested from early childhood by diminished or absent sensibility to pain, touch and temperature. A Kashmiri family with four members affected by congenital sensory neuropathy and its oral manifestations is described. Pain and temperature sensation was lost in various parts of the body including the orofacial region resulting in mutilating acropathy, particularly of the limbs and face. Orofacial motor function was normal. Three of the four members had corneal opacification due to scarring from keratitis. To prevent any further mutilation, any corrective surgery is best delayed until the patient is old enough. A discussion of the oral manifestations of this condition with a review of the literature is presented.
Maxillofacial Unit, St Luke's Hospital, Bradford, UK.
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Narayanan
D000328:Adult; D002648:Child; D003241:Consanguinity; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D009059:Mouth Diseases / Q000209:etiology*; D009410:Nerve Degeneration; D019226:Oral Ulcer / Q000209:etiology; D000699:Pain Insensitivity, Congenital / Q000209:etiology; D010375:Pedigree; D012652:Self Mutilation / Q000209:etiology*; D013697:Thermosensing; D014080:Tooth Exfoliation / Q000209:etiology