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Congenital insensitivity to pain (CIP), also known as congenital analgesia, is one or more rare conditions where a person cannot feel (and has never felt) physical pain. The conditions described here are separate from the HSAN group of disorders, which have more specific signs and etiology.
What is CIP:
Congenital insensitivity to pain with anhidrosis (CIPA) has two characteristic features: the inability to feel pain and temperature, and decreased or absent sweating (anhidrosis). This condition is also known as hereditary sensory and autonomic neuropathy type IV. The signs and symptoms of CIPA appear early, usually at birth or during infancy, but with careful medical attention, affected individuals can live into adulthood.
An inability to feel pain and temperature often leads to repeated severe injuries. Unintentional self-injury is common in people with CIPA, typically by biting the tongue, lips, or fingers, which may lead to spontaneous amputation of the affected area. In addition, people with CIPA heal slowly from skin and bone injuries. Repeated trauma can lead to chronic bone infections (osteomyelitis) or a condition called Charcot joints, in which the bones and tissue surrounding joints are destroyed.
Normally, sweating helps cool the body temperature. However, in people with CIPA, anhidrosis often causes recurrent, extremely high fevers (hyperpyrexia) and seizures brought on by high temperature (febrile seizures).
In addition to the characteristic features, there are other signs and symptoms of CIPA. Many affected individuals have thick, leathery skin (lichenification) on the palms of their hands or misshapen fingernails or toenails. They can also have patches on their scalp where hair does not grow (hypotrichosis). About half of people with CIPA show signs of hyperactivity or emotional instability, and many affected individuals have intellectual disability. Some people with CIPA have weak muscle tone (hypotonia) when they are young, but muscle strength and tone become more normal as they get older.
What causes CIP:
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. In some cases, this disorder can be in the voltage-gated sodium channel SCN9A (NaV1.7). 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. NaV1.7 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.
Types:
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.
Classification:
Autophagia (eating one's own body) is not classified as a mental disorder or a symptom of a mental disorder in the Diagnostic and Statistical Manual of Mental Disorders (DSM), the diagnostic manual used in the United States.
However, Autophagia could be classified under the DSM's Impulse-Control Disorders Not Elsewhere Classified.
Impulse-Control Disorders involve failing to resist an impulse, drive, or temptation to perform an act that is harmful to the person or to others.
It is sometimes seen with schizophrenia,psychosis and Lesch–Nyhan syndrome.
Symptoms:
Frequent physical injuries
Lack of pain sensation
Lack of awareness of physical danger
Oral damage
Fractured bones
Beahvious:
For patients with this disorder, cognition and sensation are otherwise normal; for instance patients can still feel discriminative touch(though not always temperature), and there are no detectable physical abnormalities.
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 cannot feel pain, they may not respond to problems, thus being at a higher risk of more severe diseases.
Read more:
Congenital analgesia: The agony of feeling no pain
Φοβάμαι μήπως χαθεί η ιδεολογία στη γη και γίνουμε ανθρωπάκια που θα θέλουμε να καλοπεράσουμε, που θα θέλουμε να κάνουμε καταναλωτική ζωή. Κι εμείς οι Έλληνες ακόμα, να χάσουμε αυτό που λέγεται αξιοπρέπεια, αυτό που λέγεται αγωνιστικότητα. Ναι, αυτό φοβάμαι περισσότερο από όλα.
