Congenital insensitivity to pain (or congenital analgia) is a rare condition where a person cannot feel (and has never felt) physical pain. The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... “Hurting” redirects here. ...

Presentation

Cognition and sensation is otherwise normal; for instance they can still feel discriminative touch (though not always temperature[1]), and there is no detectable physical abnormality. Look up Cognition in Wiktionary, the free dictionary. ... It has been suggested that this article or section be merged with Sensation and perception psychology. ...

Children with this condition often suffer oral cavity damage both in and around the oral cavity (such as having bitten off the tip of their tongue) or fractures to bones. Unnoticed infections and corneal damage due to foreign objects in the eye are also seen. Because the child can not feel pain, they may not respond to problems, thus being at a higher risk of more severe diseases or otherwise. The mouth, also known as the buccal cavity or the oral cavity, is the opening through which an animal or human takes in food. ... This article includes a list of works cited or a list of external links, but its sources remain unclear because it lacks in-text citations. ... An infection is the detrimental colonization of a host organism by a foreign species. ... The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber, providing most of an eyes optical power [1]. Together with the lens, the cornea refracts light and, as a result, helps the eye to focus. ...

In some people with this disorder, there may be slight mental retardation, as well as an impaired corneal reflex. Corneal reflex This is an automated involuntary blinking of the eyelids (See : Reflex) elicited by stimulation (such as touching or a foreign body) of the eyeballs cornea. ...

Causes

There are some cases where the condition is caused by increased production of endorphins in the brain, in which case naloxone may be used as treatment. This treatment does not always work.^[1] Runners high redirects here. ... In animals, the brain or encephalon (Greek for in the head), is the control center of the central nervous system, responsible for behaviour. ... Naloxone is a drug used to counter the effects of opioid overdose, for example heroin and morphine overdose. ...

In some cases, this disorder can be caused by mutations in the voltage-gated sodium channel SCN9A (Na_V1.7). Patients with such mutations are congenitally insensitive to pain and lack other neuropathies. There are three mutations in SCN9A: W897X, located in the P-loop of domain 2; I767X, located in the S2 segment of domain 2; and S459X, located in the linker region between domains 1 and 2. This results in a truncated non-functional protein. Na_V1.7 channels are expressed at high levels in nociceptive neurons of the dorsal root ganglia. As these channels are likely involved in the formation and propagation of action potentials in such neurons, it is expected that a loss of function mutation in SCN9A will lead to abolished nociceptive pain propagation.^[2] Sodium channels (also known as voltage-gated sodium channels) are integral membrane proteins that are localized in and conduct sodium ions (Na+) through a cells plasma membrane. ... SCN9A is a gene which codes a sodium ion channel. ... This is a dorsal root ganglion (DRG) from a chicken embryo (around stage of day 7) after incubation overnight in NGF growth medium stained with anti-neurofilament antibody. ...

One of the side effects of Hansen's Disease is the progressive destruction of the nerves; this can be passed on to offspring, remaining dormant except for nerve insensitivity. Father Damien was a Roman Catholic missionary who helped lepers on Hawaii and also died of the disease. ...

Types of congenital pain indifference

There are generally two types of non-response exhibited.

• Insensitivity to pain means that the painful stimulus is not even perceived: a patient cannot describe the intensity or type of pain.
• Indifference to pain means that the patient can perceive the stimulus, but lacks an appropriate response: they will not flinch or withdraw when exposed to pain.

See also

• Congenital insensitivity to pain with anhidrosis
• Familial dysautonomia

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare inherited disorder of the nervous system which prevents the sensation of pain, heat, and cold. ... Familial dysautonomia, or FD, is a disorder of the autonomic nervous system which affects the development and survival of sensory, sympathetic and some parasympathetic neurons in the autonomic and sensory nervous system resulting in variable symptoms including: insensitivity to pain, inability to produce tears, poor growth, and labile blood pressure...

References

1. ^ Manfredi M, Bini G, Cruccu G, Accornero N, Berardelli A, Medolago L (1981). "Congenital absence of pain.". Arch Neurol 38 (8): 507-11. PMID 6166287. 
2. ^ Cox JJ, Reimann F, Nicholas AK, Thornton G, Roberts E, Springell K, Karbani G, Jafri H, Mannan J, Raashid Y, Al-Gazali L, Hamamy H, Valente EM, Gorman S, Williams R, McHale DP, Wood JN, Gribble FM, Woods CG (2006). "An SCN9A channelopathy causes congenital inability to experience pain". Nature 444 (7121): 894-8. PMID 17167479. 

External links

• iii_1/p/PAIN_CONGENITAL_INDIFFERENCE_TO article at GE's Medcyclopaedia
• A child with congenital insensitivity to pain on the Oprah Winfrey show.