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Canavan disease is a cerebral degenerative disorder that is characterized by progressive damage and deterioration of the brain and the nerves. Canavan disease is one of the commonest degenerative cerebral diseases occuring during babyhood. The condition is a part of a set of genetic disorders, called the leuko-dystrophies.
Canavan disease occurs due to an insufficiency / absence of an enzyme called aspartoacylase. This shortage causes build up of N-acetylaspartic acid in the brain. The accumulated NAA triggers chemical imbalance that destroys the myelin. When the chemical imbalance causes the white matter in the brain to become spongy, the debilitating manifestations of Canavan's disease occur.
Canavan Disease Treatment
As yet, there is no complete cure for Canavan disease; also, there no standard Canavan disease treatment or course of management plan for the condition. However, experiments and laboratory investigations continue.
By and large, the Canavan disease treatment is supportive and symptomatic. Nonetheless, there is an investigational therapy using lithium citrate. A person diagnosed with Canavan Disease demonstrated elevated levels of N-acetyl aspartate. Lithium citrate has been used for experimentation in a rat genetic model of Canavan Disease; and results showed it appreciably reduced levels of N-acetyl aspartate. When the dug was administered to a human, the patinet reversed during the 2 week wash-out period, after the removal of lithium. Evidence suggests that a larger controlled clinical study and research of lithium is necessary to use it as supportive therapy for children having Canavan disease.
Furthermore, there are investigational clinical trials of gene therapy. In the study a normal gene is cloned to work instead of the faulty one that is known to result in Canavan disease.
Brain atrophy occurs in all cases of Canavan disease. There isnt any cure, for this. The convulsions and seizures are managed appropriately using anti convulsants.
The prognosis for Canavan disease after Canavan disease treatment is relatively poor. Death invariably occurs before 4 years, while, some children survive in to their late teenage period or early 20s.
Research and Canavan Disease Treatment
The gene that causes Canavan disease has been identified. Several laboratories proffer pre-natal screening for the disease to populations that are at risk. Researchers have devised animal models and are using them to assess possible treatment strategies. Studies to comprehend how the brain normally develops and functions and how it gets affected by genetic mutation are carried out. These studies help better understand Canavan disease; and thus offer greater likelihood to new possibilities of treatment.
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