Journal of dentistry for children
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J Dent Child (Chic) · May 2008
Case ReportsCongenital insensitivity to pain (hereditary sensory and autonomic neuropathy type V): a rare case report.
Congenital insensitivity (HSAN) to pain is a rare disorder, which affects the body's painprotective mechanism and predisposes the patient to increased risk and incidence of traumatic injuries. Currently, 5 types of hereditary sensory and autonomic neuropathies have been identified, depending upon different patterns of sensory and autonomic dysfunction, peripheral neuropathy, clinical features, and genetic abnormalities. ⋯ History, clinical features, nerve conduction studies, and electron microscopy revealed no reaction to painful stimuli, a self-mutilating habit, multiple missing teeth, and an absence of small, unmyelinated fibers, thus indicating HSAN type V. Management included patient counseling and use of a mouthguard to prevent further damage and restore function.