Rasmussen

’s Encephalitis Essay, Research Paper

Rasmussen’s Encephalitis The human immune system is an amazing system that is constantly on the alert protecting us fromsicknesses. Thousands of white blood cells travel in our circulatory system destroying all foreignsubstances that could cause harm to our body or to any of the millions of processes going on inside. Nowimagine a condition where this awesome system turns against the most complex organ in the human body,the brain. Deadly as it is, this condition is known as Rasmussen s encephalitis. The meaningful research on Rasmussen s encephalitis was begun (unintentionally) by Scott Rogersand Lorise Gahring, two neurologists, who were at the time measuring the distribution of glutamatereceptors in the brain. Later on when more provocative information was found they enlisted the help ofJames McNamara and Ian Andrews, epilepsy experts at Duke University Medical Center. The details on Rasmussen s encephalitis were very bleak at the time when the men began theirresearch. All that was known is that Rasmussen s encephalitis was a degenerative disease of the brainthat caused seizures, hemiparesis, and dementia normally in the first ten years of life. The seizures thatwere caused by Rasmussen s encephalitis were unstoppable by normal anti-seizure drugs usedconventionally. What the worst part of the disease was that the pathogenesis for it were not known andeven worse was how it developed. The first clue was delivered when Rogers and Gahring were trying to register the distribution of theglutamate receptors using antibodies, that tag on to the receptor itself. The proteins that make up theglutamate receptors(GluR) are only found inside the blood brain barrier(BBB). Glutamate and a fewrelated amino acids are the dominant form of excitatory neurotransmitter in the central nervous system ofmammals. If one of these GluRs happens to wander into the actual bloodstream, that is outside the BBB,it would be considered an outsider and destroyed immediately. So if these GluRs were put into the normalblood stream then the immune system would produce antibodies which could then be used in thesearching for the glutamate receptors. In order to test this theory the researchers injected the GluRs into the blood stream of a normalhealthy rabbit hoping to produce good results. At this point the experiment took a dramatic turn, afterreceiving a few doses of the protein two of the three rabbits began to twitch, as though they were sufferingthe pain of an epileptic seizure. Now the help of McNamara and Andrews was enlisted. When McNamara and Andrews examined the brain tissue of the rabbits, they saw what seemed to bea familiar inflammatory pattern, clumps of immune cells all around blood vessels. This descriptionexactly matched the description of persons suffering from Rasmussen s encephalitis, moreover somethingas this would never be found in a healthy brain. A healthy brain has its blood capillaries enclosed in theBBB membrane, so such a case as the one mentioned above would not be possible. As protective as the BBB is, it can be breached by something like a head injury. What washappening was that the antibodies which were out to get the GluR proteins were somehow finding a wayinto the brain and directing an attack towards all GluR receptor proteins in the brain itself. After some more examinations Rogers and McNamara decided that these attacks were the cause ofthe seizures that are often experienced by sufferers of Ramussen s encephalitis. Then if the case is ofantibodies in the bloodstream, than sufferers of Ramussen s encephalitis should have them in their

bloodstream and healthy normal peoples shouldn t. When this was actually tested the results werepositive that Rasmussen sufferers did have these antibodies in their bloodstreams and healthy people didnot. These were not only the right kind of antibodies but, the very antibodies that caused the seizures inpeople and rabbits. Thus when these antibodies were removed by plasma exchange(PEX) it caused atemporary relief from the seizures but soon the body starts making more antibodies of the type and theseizures start once again. After all the examinations two questions remained, why does the body mountan immune response against one of its own brain proteins, and how do these antibodies get through theBBB? What is thought right now is that people get antibodies when they are infected by a microorganismlike a bacterium or a virus that is similar in structure to the GluR. When this happens the body mounts animmune response against, and it just so happens that at this stage you suffer a blow to the head. This willopen your BBB to the antibodies and they will attack the friendly GluRs in the brain, causing seizures andfurther opening your BBB to more antibodies. Now a malicious rhythm begins: antibodies break through the BBB, inflammation is caused due tothe break in, seizures are now caused and BBB opens up further, further opening in the BBB cause moreseizures. The inflammation is caused by the autoimmune process against the GluR. All the seizuresoccur where the initial break in the BBB happened due to a blow to the head, explaining why theyseizures are confined to just one hemisphere. The only problem with this theory is that the rabbitsdeveloped seizures without ever being whacked on the head, but that also could be because a rabbit s brainis not as well insulated as a human s. Normally what happens to an individual is that after he or she is involved in this cycle the onlything that can make for relief is the recurrent plasma exchange. This will only cease the seizurestemporarily, but they will start again when the body has made more new antibodies. After this has beendone many times the hemisphere in which sufferers of Ramussen s encephalitis is present will deteriorateto the point where a hemispherectomy has to be performed. This will render the person to mentaldisintegration where he or she has no more mental capacity and generally to the point of no return, death. Rasmussen s encephalitis is a very deadly disease, but it is also a very rare disease, occurring in only48 people between 1957 and 1987. As of now there are no FDA approved drugs for the sufferers ofRamussen s encephalitis. Now the researchers are working on a drug that will block the activity of thisparticular antibody, but this could lead to further problems. If this drug is being administered and abacteria or virus of a similar structure as the GluR is present the body would disregard it and this wouldcause more health problems. After all this bad news all one can say is, “Good luck” to the ones sufferingfrom this living hell. Atkins, “Rasmussen s encephalitis: nueroimaging findings in four patients.” AJR-Am-J- Roentgenol. June 1992. Blume, “Rasmussen s chronic encephalitis in adults.” Arch-Nuerol. March 1993. Hanovar, “Rasmussen s encephalitis in surgery for epilepsy.” Dev-Med-Child-Nuerol. January 1992. Leary, “Clues Found To Rare Form of Epilepsy.” New York Times. December 5 1994, pp. A4. Whisenand, “Autoantibodies to glutamate receptor GluR3 in Rasmussen s encephalitis,” Science. July 29 1994.