Familial Dysautonomia and the Brain Behavior Enigma Essay -- Biology E

An understanding of the brain is essential to developing safe and effective treatments for disorders of the nervous system and for advancing our understanding of the human experience. Nervous system disorders cost the US more than $400 billion in medical expenses each year (1). In addition to having great clinical significance, such research offers a valuable perspective into the nature of the brain-behavior relationship. The extent to which the brain is organized in terms of overt behavior remains an open question. Because it is difficult to ethically manipulate the neural composition of humans in the laboratory setting, our understanding of the biological and neurophysiological influences on behavior is limited. In the spirit of this class, I have decided to take an alternate route to untangling the connections between brain and behavior. In this paper, I will discuss Familial Dysautonomia, a neurological disease that encapsulates the relationship between sensation, perception, emo tion, physiological response and the nervous system. Familial Dysautonomia (FD), also called Riley-Day Syndrome, is one of five hereditary sensory and autonomic neuropathies (HSANS) (2). FD is an autosomal recessive disease of the Ashkenazi, or European, Jewish population (3). As the name implies, this neurological disorder is characterized by the incomplete development of the autonomic nervous system. The behavioral phenomena observed in FD sufferers can be used as an instrument to gage the inner activities of the autonomic nervous system (ANS). We already know that the ANS is responsible for life-sustaining regulatory processes. The autonomic nerve fibers form a system that regulates the heart, blood vessels, glands, the digestive system and othe... ..., a comprehensive database of FD related information, offering links to recent press releases, online information and family support networks. http://www.familialdysautonomia.org/ 4) Memorial University of Newfoundland , Autonomic Nervous System I. http://calloso.med.mun.ca./~thoekman/autonom/ans1.htm 5) Autonomic Differential Diagnosis , a breakdown of congenital sensory neuropathologies. http://www.neuro.wustl.edu/neuromuscular/autonomic.html 6) Newton's Apple , Tears: Why do we cry? http://www.eecs.umich.edu/mathscience/funexperiments/agesubject/lessons/newton/tear.html 7) Medical College of Wisconsin , Riley-Day Syndrome, respiratory disease and the possible role of catecholamines. http://chorus.rad.mcw.edu/doc/00356.html 8) Pain and Sedation on the PICU , an outline of the neurophysiology of pain http://www.ucs.mun.ca/~skhoury/PAIN.html Familial Dysautonomia and the Brain Behavior Enigma Essay -- Biology E An understanding of the brain is essential to developing safe and effective treatments for disorders of the nervous system and for advancing our understanding of the human experience. Nervous system disorders cost the US more than $400 billion in medical expenses each year (1). In addition to having great clinical significance, such research offers a valuable perspective into the nature of the brain-behavior relationship. The extent to which the brain is organized in terms of overt behavior remains an open question. Because it is difficult to ethically manipulate the neural composition of humans in the laboratory setting, our understanding of the biological and neurophysiological influences on behavior is limited. In the spirit of this class, I have decided to take an alternate route to untangling the connections between brain and behavior. In this paper, I will discuss Familial Dysautonomia, a neurological disease that encapsulates the relationship between sensation, perception, emo tion, physiological response and the nervous system. Familial Dysautonomia (FD), also called Riley-Day Syndrome, is one of five hereditary sensory and autonomic neuropathies (HSANS) (2). FD is an autosomal recessive disease of the Ashkenazi, or European, Jewish population (3). As the name implies, this neurological disorder is characterized by the incomplete development of the autonomic nervous system. The behavioral phenomena observed in FD sufferers can be used as an instrument to gage the inner activities of the autonomic nervous system (ANS). We already know that the ANS is responsible for life-sustaining regulatory processes. The autonomic nerve fibers form a system that regulates the heart, blood vessels, glands, the digestive system and othe... ..., a comprehensive database of FD related information, offering links to recent press releases, online information and family support networks. http://www.familialdysautonomia.org/ 4) Memorial University of Newfoundland , Autonomic Nervous System I. http://calloso.med.mun.ca./~thoekman/autonom/ans1.htm 5) Autonomic Differential Diagnosis , a breakdown of congenital sensory neuropathologies. http://www.neuro.wustl.edu/neuromuscular/autonomic.html 6) Newton's Apple , Tears: Why do we cry? http://www.eecs.umich.edu/mathscience/funexperiments/agesubject/lessons/newton/tear.html 7) Medical College of Wisconsin , Riley-Day Syndrome, respiratory disease and the possible role of catecholamines. http://chorus.rad.mcw.edu/doc/00356.html 8) Pain and Sedation on the PICU , an outline of the neurophysiology of pain http://www.ucs.mun.ca/~skhoury/PAIN.html