Congenital 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. The purpose of this report is to present the case of a 10-year-old boy with congenital insensitivity to pain (hereditary sensory autonomic neuropathy [HSAN] type V) with dental implications. 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.
Singla
Shilpy
S
Department of Pedodontics and Preventive Dentistry, Government Dental College, Rohtak, Haryana, India. shilpy1_singla@yahoo.co.in
Marwah
Nikhil
N
Dutta
Samir
S
D002648:Child; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis*; D006801:Humans; D008046:Lip / Q000293:injuries; D008297:Male; D009061:Mouth Mucosa / Q000293:injuries; D000699:Pain Insensitivity, Congenital / Q000175:diagnosis*; D016728:Self-Injurious Behavior / Q000175:diagnosis* / Q000517:prevention & control; D014071:Tooth Abnormalities / Q000175:diagnosis