Monday, April 7, 2014

Special Guest Post by David Blocker - Lesch-Nyhan Syndrome and the Arabic Infancy Gospel

The Arabic Infancy Gospel [1] tells a story about an aggressive, demon possessed child who bit himself and others, and then struck Jesus [2].

The description of the child's biting behavior and aggression is characteristic of Lesch-Nyhan syndrome [3] [4]. This suggests that the pre-modern author of the text had seen or heard of a child who was afflicted with the disorder and attributed the abnormal behaviors to demonic possession.

David Blocker 2014-04

[1] The Arabic Infancy Gospel can be obtained from any of the sources listed below:

As The First Gospel of the Infancy of Jesus Christ in The Lost Books of the Bible, William Hone, Jeremiah Jones, William Wake. David McKay, 1901

The Arabic Infancy Gospel in the Ante-Nicene Fathers Vol. 8, Alexander Roberts, Sir James Donaldson, Arthur Cleveland Coxe - 1886 .

The Arabic Gospel of the Infancy of the Saviour at http://www.newadvent.org/fathers/0806.htm

[2] Excerpted from the “Arabic Infancy Gospel :

35. Another woman was living in the same place, whose son was tormented by Satan. He, Judas by name, as often as Satan seized him, used to bite all who came near him; and if he found no one near him, he used to bite his own hands and other limbs. The mother of this wretched creature, then, hearing the fame of the Lady Mary and her son Jesus, rose up and brought her son Judas with her to the Lady Mary. In the meantime, James and Joses had taken the child the Lord Jesus with them to play with the other children; and they had gone out of the house and sat down, and the Lord Jesus with them. And the demoniac Judas came up, and sat down at Jesus' right hand: then, being attacked by Satan in the same manner as usual, he wished to bite the Lord Jesus, but was not able; nevertheless he struck Jesus on the right side, whereupon He began to weep. And immediately Satan went forth out of that boy, fleeing like a mad dog. And this boy who struck Jesus, and out of whom Satan went forth in the shape of a dog, was Judas Iscariot, who betrayed Him to the Jews; and that same side on which Judas struck Him, the Jews transfixed with a lance. (underlining added for emphasis)

[3] Descriptions of Lesch-Nyhan syndrome:

Excerpted from Wikipedia:

Lesch Nyhan Syndrome’s Self-injuring behavior:

Persons affected are cognitively impaired and have behavioral disturbances that emerge between two and three years of age. The uncontrollable self-injury associated with LNS also usually begins at three years of age. The self-injury begins with biting of the lips and tongue; as the disease progresses, affected individuals frequently develop finger biting and head banging. The self-injury can increase during times of stress. Self-harm is a distinguishing characteristic of the disease and is apparent in 85% of affected males.

... Compulsive behaviors also occur, including aggressiveness, vomiting, spitting, and coprolalia (involuntary swearing). The development of this type of behavior is sometimes seen within the first year, or in early childhood, but others may not develop it until later in life.

[4] Excerpted from a lecture by Lars Sorensen - Prof. Stanley Vitello : IDD : 293:522 : Fall 2008 - Rutgers University - Graduate School of Education downloaded from: http://www.cs.rutgers.edu/~biglars/LNS.html

Lesch-Nyhan Syndrome

An Overview

Abstract

Lesch-Nyhan Syndrome is a disorder that strikes the sufferer with debilitating motor and cognitive problems, hyperuricemia, and the urge to do harm to yourself with acts of self-injurious behavior. Research has lead to the discovery of a genetic sequence that results in a defective enzyme, but researchers are still unsure how this leads to the neurological and behavioral problems that are the hallmark of the disorder. Treatments as simple as wearing oven mitts and as complicated as electrical wiring in the brain have been used to help LNS patients, but no cure for the syndrome seems in sight.

....

Lastly, all people with LNS display some degree of abnormal behavior, specifically, the self-injuring of one's self by biting. This behavior begins as soon as the child's teeth come in and typically results in parents’ frantically calling pediatricians asking why their children are trying to eat themselves. LNS sufferers report that they feel as if someone else is controlling them as they bite at their lips and hands until they are bloodied. The self-injurious behavior does not stop at biting though. LNS sufferers have been known to stab themselves in their eyes with sharp objects and some have bitten off their tongues (Preston, 2007). Their self-injurious behavior has been described as clever and not limited to the standard self-injurious behaviors of self-hitting and head banging. The self-injurious behavior is said to be complex, often involving devious plots (Luiselli, Matson & Singh, 1992) to become unrestrained and hurt themselves.

Their injurious behavior does not stop with themselves. LNS sufferers have been known to punch their doctors, (Nyhan, 2005) punch their friends, and even roll their wheelchairs out into the middle of traffic as they yell at the cars not to hit them because it's the LNS that's making them do it (Preston, 2007).

What adds to the mystery of all this behavior is the fact that many LNS sufferers are described as extremely kind natured. People with LNS will begin to apologize the minute they do something inappropriate to themselves or others. Matthew, the boy under Dr. Nyhan's care in 1962, would often rip the doctor's glasses from his face and hurl them across the room. Before the glasses would hit the ground Matthew would begin apologizing yelling “Sorry, I'm sorry.” A person with LNS will often apologize for acts he has not committed yet. Dr. Nyhan believes this aspect of their personality has something to do with the disorder as he knows the majority of LNS sufferers in the US (there are only a few hundred) and describes them as “great people” (Preston, 2007). A small review of pictures of children with LNS will almost always show them in seemingly good spirits as they smile through their damaged lips (Maramattom B.V., 2005). Even in the face of this strange unfortunate condition they seem happy. It's another aspect of this disorder that makes it difficult to understand.

LNS sufferers do not have an absence of pain as many believe they must in order to hurt themselves the way they do. They feel everything they do to themselves, often crying and screaming during the acts. They need to be restrained to keep from injuring themselves. They often eat with wide plastic spoons to keep from stabbing themselves. LNS caregivers tasked with restraining people will tell you that in all situations being restrained makes the LNS sufferer happy. They are assured when they know they can't hurt themselves or others and a feeling of calm comes over them.

... In every case there was a mutation found in the HPRT gene. Not all of the mutations were the same, there were different codings for different families, and in the majority of cases there was only one misspelling in the code.

... As we have seen, LNS is a terrible disorder that strikes the sufferer with debilitating motor and cognitive problems, hyperuricemia and the urge to do harm to themselves and others. While we seem to have a handle on how a damaged genetic sequence leads to a defective enzyme, we are still unsure how this leads to the neurological and behavioral problems that are the hallmark of the disorder. Treatments as simple as wearing oven mitts and as complicated as electrical wiring in the brain have been used to help the LNS sufferer but no cure for the syndrome seems in sight. ...


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