A new edition of The Intellectual Devotional, this time with a focus on Health, is coming to stores on October 16! (Click here to pre-order your copy.) As well as continuing to expand on posts from the General Edition, “The Devoted Intellect” blog will introduce and expand on material from the Modern Culture devotional. Today’s entry on “Angelman Syndrome” is from the “Children & Adolescents” section of the Health edition.

Angelman syndrome (AS), a neuro-genetic disorder, is characterized by cognitive and motor skill delays, absent speech, sleep disturbances, seizures, jerky movements, frequent bursts of unprovoked laughter and smiling and an unusually cheerful demeanor. It was discovered in 1965 by pediatrician Dr. Harry Angelman, who noticed that three of his patients had the same cluster of confounding symptoms that did not fit within any established diagnosis. He referred to these patients as “puppet children,” on account of the jerky gait typical of the disease. Many members of the medical community initially doubted the existence of this disorder; however, other doctors subsequently began reporting cases with the same symptoms, and affected people soon acquired the nickname “happy puppet children.” In 1982, it was suggested that the name should be changed to Angelman’s syndrome, in an effort to be sensitive to the families of those with the disease.

While AS is characterized by a host of cognitive, motor, developmental and physical symptoms, what sets this condition apart from many others is the unusually cheerful, gregarious disposition of the people affected with the disease, as well as their and their unusually youthful appearance. It is estimated that a whopping 70% of people with AS engage in frequent bursts of laughter, giggling and chortling and persistent social smiling.
Researchers have ruled out epilepsy as a cause of these fits of laughter. Interestingly, neuroimaging studies conducted on people with AS have revealed the cortical and subcortical regions of the brain that are linked to humor and laughter in normal individuals, namely:

1) The frontal lobe, which is key to cognitive processing;
2) The supplementary motor area, which controls the motor aspects of humor (laughter, smiling); and
3) The nucleus accumbens, which is linked to the experience of pleasure.

These findings suggest that the left hemisphere of the brain, combined with its subcortical and dopaminergic structures, play an integral role in the human experience of humor and laughter.

With respect to people with AS, neuroimaging studies have not revealed any specific defect in their brains that suggests a laughter-inducing abnormality. However, these studies have revealed differences in their humor-processing pathways, specifically in the regions associated with the reward mechanisms linked to humor/laughter and the motor regions that control laughing. Thus, scientists have theorized that excessive laughter and persistent smiling characteristic to AS is probably an expressive motor event; for mysterious reasons, people with AS respond to most stimuli, whether it be physical or mental, with sudden and protracted outbursts of laughter. Thus, even though people with AS are able to experience a range of emotions, it appears that their brains are mostly wired for happiness….

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