What is Rasmussen Syndrome?
Rasmussen syndrome is an autoimmune disorder that causes one hemisphere of the brain to become inflamed, and eventually deteriorate. It is usually first seen when the patient reaches the age of 14 months to 14 years; however, most severe problems do not begin until one to three years after the onset of the seizures. Although treatments of Rasmussen syndrome do not always work, the disorder is rarely fatal.
One of the first symptoms associated with Rasmussen syndrome is seizures. Usually the seizures are considered partial motor seizures; however, nearly 20 percent of the children affected by the disorder suffer from a grand mal or tonic-clonic seizures. Other symptoms include a slight weakness in a leg or an arm. As mentioned above, other neurologic issues, such as speech disorders, weakness on one side of the body, or mental incapacity do not occur until one to three years has passed.
Treatment of Rasmussen syndrome does not always cure the disorder. Steroids are the most effective form of non-surgical treatment; however, additional research is needed before they become widely recommended for long-term use. In addition, therapy associated with immunological disorders is sometimes useful, such as shots of gamma globulin or plasmapheresis. Generally, the medicines used to treat or prevent seizures are not helpful with Rasmussen syndrome – unlike with epilepsy. For children with a severe form of the disease, a surgical operation called a functional hemispherectomy may be the most useful, where half of the brain is operated on and removed.
Even though Rasmussen syndrome is not generally fatal, it significantly affects both those with the disorder and their families. When the seizures occur, they are unrelenting. Unfortunately, the seizures usually lead to mental retardation and severe weakness on one side of the body. In fact, MRI scan of a patient’s brain will actually show a loss of brain matter. As mentioned above, a hemispherectomy may be the best option for those with the disorder. After the surgery, most children can run and walk and language skills can remain intact.
Among the difficulties for people dealing with Rasmussen syndrome is that the patients had a normal, healthy existence prior to the onset of the disorder. Discovering the problem early is key to ensuring the well-being of the patient. When it is discovered in its early stages, the brain is less deteriorated than in later stages of the disorder. Additional research and a better understanding of the causes and potential treatments of Rasmussen syndrome are essential for the disease.
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