Description: Adrenoleukodystrophy (ALD), or commonly known as Lorenzo's Oil, is a rare genetic disorder that affects about 1/100,000 males around the world. It is characterized by the build up of very long chain fatty acids (VLCFAs) in the brain and the adrenal glands. These fatty acids strip the myelin off nerve fibers and lead to the degeneration of the adrenal glands. ALD is one of the leukodytrophies that cause damage to the myelin sheath, the fatty covering on nerve fibers in the brain. .
There are several forms of ALD. The classic childhood form, which is the most severe, only affects boys and may occur between the ages of 4 and 10, but can appear as early as 2 years of age. In the childhood form, affected children develop normally until about the age of three or four, and it progresses quickly from there. The milder adult-onset form, it typically begins between the ages of 21 and 35. Neonatal ALD affects both male and female newborns; this form is usually quickly progressive. .
Symptoms: Some of the symptoms of the childhood form include visual loss, learning disabilities, seizures, difficulty swallowing, deafness, fatigue and vomiting. The most common symptoms are abnormal withdrawal, aggression, poor memory and poor school performance. In the milder adult-onset form symptoms may include leg stiffness, progressive spastic paraparesis (stiffness, weakness and/or paralysis). Symptoms in the neonatal form can consist of mental retardation, facial abnormalities, seizures, retinal degeneration, low muscle tone and adrenal dysfunction. .
Cause Agent: There is no cause agent for ALD; it is inherited from the mother during pregnancy. All daughters of an affected male are carriers of the disease; none of his sons will be affected. A female who is a carrier has a 50% chance of transmitting it during pregnancy. Sons who inherit it will be affected, and the daughters will be carriers but not seriously affected by the disease.
Adrenoleukodystrophy Adrenoleukodystrophy is a genetic disease that is passed on from mother to son. ALD is an X-linked disorder. That means it affects only males and is transmitted by a female carrier. This disorder is called X-linked since the genetic abnormality involves the X-chromosome. Wo...
Lorenzo's Oil Lorenzo's oil a MCA universal picture was filmed in 1992. Based on a true story with Susan Sarandon and Nick Nolte playing the parents Michalla and Augosto Odone of Lorenzo who is played by Peter Ustinov. At 5 years old Lorenzo is faced with a fatal disease called Adrenoleukodystro...
Nelson syndrome is a medical disorder which is characterized by abnormal secretion of hormones and enlargement of the pituitary gland. This condition occurs after an adrenalectomy, which causes the growth of a pituitary tumor. This is a rare disorder, thus doing research on it is a great challenge...
