What is Alexander Disease?
Alexander Disease is an extremely rare genetic disorder which typically results in a fatal deterioration of the patient’s nervous system. In most cases, the symptoms appear in patients in their infantile years (typically starting from approximately 6 months after birth), but the disorder may be triggered at any stage of life. The disorder is usually inherited, and thus family history is one of the largest causes in this disorder. It is typically linked to the destruction of white matter in the patient’s brain, resulting in the deterioration of the nervous system. (1) The white matter, also known as the myelin, is a fatty covering which aid in the rapid transmission of nerve impulses. In other words, if the myelin deteriorates, the transmission of nerve impulses will slow down significantly, greatly impairing the patient’s nervous system. (2,3)
Background Information
Alexander Disease was first described in 1949 in Australia by Dr. W. Stewart Alexander (thus the name Alexander Disease). Since its first discovery, only approximately 500 patients have ever been prescribed with this condition. This disorder has been reported in both members of the sex, in various ethnic groups. (7)
There are 3 major different types of Alexander Disease: Infantile (birth~2 years), Juvenile (2~12 years), and Adult-onset (12 years+). The different types of Alexander Disease refers to the age when the disorder was formed, and the younger types are generally the most dangerous (the patients are given a lower life expectancy in comparison to the Adult-onset type). Unfortunately, the majority of the patients fall under the category of Infantile Alexander Disease (approx. 51%). (2)
Alexander Disease is an extremely rare genetic disorder which typically results in a fatal deterioration of the patient’s nervous system. In most cases, the symptoms appear in patients in their infantile years (typically starting from approximately 6 months after birth), but the disorder may be triggered at any stage of life. The disorder is usually inherited, and thus family history is one of the largest causes in this disorder. It is typically linked to the destruction of white matter in the patient’s brain, resulting in the deterioration of the nervous system. (1) The white matter, also known as the myelin, is a fatty covering which aid in the rapid transmission of nerve impulses. In other words, if the myelin deteriorates, the transmission of nerve impulses will slow down significantly, greatly impairing the patient’s nervous system. (2,3)
Background Information
Alexander Disease was first described in 1949 in Australia by Dr. W. Stewart Alexander (thus the name Alexander Disease). Since its first discovery, only approximately 500 patients have ever been prescribed with this condition. This disorder has been reported in both members of the sex, in various ethnic groups. (7)
There are 3 major different types of Alexander Disease: Infantile (birth~2 years), Juvenile (2~12 years), and Adult-onset (12 years+). The different types of Alexander Disease refers to the age when the disorder was formed, and the younger types are generally the most dangerous (the patients are given a lower life expectancy in comparison to the Adult-onset type). Unfortunately, the majority of the patients fall under the category of Infantile Alexander Disease (approx. 51%). (2)