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Congenital Insensitivity to Pain (Hereditary Sensory and Autonomic Neuropathy Type V): A Rare Case Report

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Congenital insensitivity (HSAN) to pain is a rare disorder, which affects the body's pain-protective 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.
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Keywords: HEREDITARY SENSORY AUTONOMIC NEUROPATHY; INSENSITIVITY TO PAIN; PERIPHERAL SENSORY NEUROPATHY; SELF-MUTILATION

Document Type: Case Report

Affiliations: Department of Pedodontics and Preventive Dentistry, Government Dental College, Rohtak, Haryana, India

Publication date: May 1, 2008

More about this publication?
  • Acquired after the merger between the American Society of Dentistry for Children and the American Academy of Pediatric Dentistry in 2002, the Journal of Dentistry for Children (JDC) is an internationally renowned journal whose publishing dates back to 1934. Published three times a year, JDC promotes the practice, education and research specifically related to the specialty of pediatric dentistry. It covers a wide range of topics related to the clinical care of children, from clinical techniques of daily importance to the practitioner, to studies on child behavior and growth and development. JDC also provides information on the physical, psychological and emotional conditions of children as they relate to and affect their dental health.
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