Human Behavior Essay
At the beginning of the film, Lorenzo (played by Noah Banks and also Zack O’Malley Greenburg) is a bright and vibrant young boy living in the Comoros Islands, as his father Augusto (played by Nick Nolte) works for the World Bank and is stationed there. However, when his parents relocate to the United States, he begins to show neurological problems, such as loss of hearing, tantrums, etc. The boy is diagnosed as having adrenoleukodystrophy (ALD), which is fatal within two years. Failing to find a doctor capable of treating their son’s rare disease, Augusto and his wife Michaela (Susan Sarandon) set out on a mission to find a treatment to save their child. In their quest, the Odones clash with doctors, scientists, and support groups, who are skeptical that anything could be done about ALD, much less by laypeople. But they persist, setting up camp in medical libraries, reviewing animal experiments, enlisting the aid of Professor Gus Nikolais (played by Peter Ustinov), badgering researchers, questioning top doctors all over the world, and even organizing an international symposium about the disease.
Despite research dead-ends, the horror of watching their son’s health decline, and being surrounded by skeptics (including the coordinators of the support group they attend), they persist until they finally hit upon a therapy involving adding a certain kind of oil (actually containing two specific long chain fatty acids, isolated from rapeseed [canola] oil and olive oil) to their son’s diet. They contact over 100 firms around the world until they find an elderly British chemist (Don Suddaby, who plays himself in a cameo role) working for Croda International who is willing to take on the challenge of distilling the proper formula. The oil, erucic acid, proves successful in normalizing the accumulation of the very long chain fatty acids in the brain that had been causing their son’s steady decline, thereby halting the progression of the disease.
There is still a great deal of neurological damage remaining which could not be reversed unless new treatments could be found to regenerate the myelin sheath (a lipid insulator) around the nerves. The father is seen taking on the new challenge of organizing biomedical efforts to heal myelin damage in patients (see The Myelin Project). The film ends with Lorenzo at the age of 14 showing definite improvement (he could swallow for himself and answer yes or no questions by blinking) but indicating more medical research is still needed. The end credits of the film note that Lorenzo has also regained his sight and is learning to use a computer.
Adrenoleukodystrophy is a rare genetic disease characterized by a loss of myelin surrounding nerve cells in the brain and progressive adrenal gland dysfunction.
Adrenoleukodystrophy (ALD) is a member of a group of diseases, leukodystrophies, that cause damage to the myelin sheath of nerve cells. Approximately one in 100,000 people is affected by ALD. There are three basic forms of ALD: childhood, adult-onset, and neonatal. The childhood form of the disease is the classical form and is the most severe. Childhood ALD is progressive and usually leads to total disability or death. It affects only boys because the genetic defect is sex-linked (carried on the X chromosome). Onset usually occurs between ages four and ten and can include many different symptoms, not all of which appear together. The most common symptoms are behavioral problems and poor memory. Other symptoms frequently seen are loss of vision, seizures, poorly articulated speech, difficulty swallowing, deafness, problems with gait and coordination, fatigue, increased skin pigmentation, and progressive dementia.
The adult-onset form of the disease, also called adrenomyeloneuropathy, is milder, progresses slowly, is usually associated with a normal life span, and usually appears between ages 21-35. Symptoms may include progressive stiffness, weakness, or paralysis of the lower limbs and loss of coordination. Brain function deterioration may also been seen. Women who are carriers of the disease occasionally experience the same symptoms, as well as others, including ataxia, hypertonia (excessive muscle tone), mild peripheral neuropathy, and urinary problems. The neonatal form affects both male and female infants and may produce mental retardation, facial abnormalities, seizures, retinal degeneration, poor muscle tone, enlarged liver, and adrenal dysfunction. Neonatal ALD usually progresses rapidly.
Causes and symptoms
The genetic defect in ALD causes a decrease in the ability to degrade very long chain fatty acids. These build up in the adrenal glands, brain, plasma, and fibroblasts. The build-up of very long chain fatty acids interferes with the ability of the adrenal gland to convert cholesterol into steroids and causes demyelination of nerves in the white matter of the brain. Demyelinated nerve cells are unable to function properly.
Diagnosis is made based on observed symptoms, a biochemical test, and a family history. The biochemical test detects elevated levels of very long chain fatty acids in samples from amniocentesis, chorionic villi, plasma, red blood cells, or fibroblasts. A family history may indicate the likelihood of ALD because the disease is carried on the X-chromosome by the female lineage of families.
Biosynthesis (also called biogenesis or “anabolism”) is an enzyme-catalyzed process in cells of living organisms by which substrates are converted to more complex products. The biosynthesis process often consists of several enzymatic steps in which the product of one step is used as substrate in the following step. Examples for such multi-step biosynthetic pathways are those for the production of blood clots,amino acids, fatty acids, and natural products. Biosynthesis plays a major role in all cells, and many dedicated metabolic routes combined constitute general metabolism. Six organelles in the cell are involved in biosynthesis: ribosomes, chloroplasts, smooth endoplasmic reticulum, rough endoplasmic reticulum, plastids, and Golgi bodies.
 Dementia (taken from Latin, originally meaning “madness”, from de- “without” + ment, the root of mens “mind”) is a serious loss of global cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging. It may be static, the result of a unique global brain injury, or progressive, resulting in long-term decline due to damage or disease in the body. Although dementia is far more common in the geriatric population (about 5% of those over 65 are said to be involved), it can occur before the age of 65, in which case it is termed “early onset dementia”. Dementia is not a single disease, but a non-specific syndrome (i.e., set of signs and symptoms). Affected cognitive areas can be memory, attention, language, and problem solving. Normally, symptoms must be present for at least six months to support a diagnosis. Cognitive dysfunction of shorter duration is called delirium.
Dysphagia is the medical term for the symptom of difficulty in swallowing. Although classified under “symptoms and signs” in ICD-10, the term is sometimes used as a condition in its own right. Sufferers are sometimes unaware of their dysphagia. Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia; it is also referred to as an unusual “tightness”, stiffness, or “pull” of muscles. The word spasm comes from the Greek word σπασμός (spasmos), meaning “drawing, pulling.”
Clinically spasticity is defined as velocity dependent resistance to stretch, where a lack of inhibition results in excessive contraction of the muscles, ultimately leading to hyperflexia (overly flexed joints). It mostly occurs in disorders of the central nervous system (CNS) affecting the upper motor neuron in the form of a lesion, such as spastic diplegia, but it can also present in various types of multiple sclerosis, where it occurs as a symptom of the progressively-worsening attacks on myelin sheaths and is thus unrelated to the types of spasticity present in neuromuscular cerebral palsy rooted spasticity disorders. Definition
A seizure happens when there are certain types of abnormal electrical activity in the brain. During a seizure, you may:
* Stare into space
* Have convulsions (abnormal jerking of the muscles)
* Experience abnormalities of sensation or emotion
University/College: University of Arkansas System
Type of paper: Thesis/Dissertation Chapter
Date: 3 June 2017
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