Guillain Barre Syndrome

Guillain Barre Syndrome, or GBS, is one of Neurology's most mysterious diseases. Commonly noted as AIDP which stands for Acute Inflammatory Demyelinating Polyneuropathy. Classified as an autoimmune disease, it is extremely rare effecting 1-2 people per 100,000. With the advances in developing vaccinations the number of cases seem to be increasing. It is a direct result of the body's immune system attacking the peripheral nervous system. In my words on this I try to be as black and white as possible. This typically stems from an infection in the body, more commonly upper respiratory infections and infections in the digestive system that stem from such things as food poisoning, stomach bugs, etc. However we've figured out that realistically any infection can be a root to developing Guillain Barre Syndrome. I actually interviewed several GBS patients that developed it after various types of trauma such as having surgery or minor cuts. One gentleman contracted the disease after simply cutting himself with his hunting knife. So here we are wondering what could possibly happen after a simple little nick from a hunting knife or eating some undercooked shellfish? Well, GBS comes on differently for every patient, but it takes the same action on the body's nerves. Demyelinating effects is the name of the GBS game. Myelin is the insulating coating that covers the nerves. Nerves need that myelin coating to successfully transport  the signal from the brain to the right parts of the body in order to tell the muscles to active and cause movement. So if you curl your arm that means the brain sent a signal to activate the bicep muscle to contract and pull the lower arm up to a curl. That myelin is like the protective cylinder around the nerve itself much like the plastic/rubber coating around a speaker wire. So the signal from your stereo to the speaker travels through the wire inside the containment of the coating if that makes sense. But what happens if that coating gets damaged? When the coating is broke it opens a spot for the signal to escape therefor the signal weakens by the time it reaches it's destination. GBS causes the immune system to attack the myelin that coats the nerves and when the coating is damaged the signal from the brain begins to weaken as it travels through the nerve. The word "weaken" is a crucial word because as that signal weakens so does the strength of the muscles that the signal is supposed to be activating because the muscle isn't getting a full signal. You begin to get weak and this is an ascending weakness meaning your distal body parts (furthest from the brain) are typically weakened first and then the weakness grows it's way up the body. So you might, like myself, experience a weakness that starts in the feet and hands. This weakness might feel like your feet and often hands are trying to go to sleep on you. Tingly, numb feeling, and somewhat uncontrollable are very common descriptions of an on-setting case of GBS. you'll then experience this real symmetrical line of that feeling grow up your legs and sometimes arms. It will grow closer and closer to your torso and then it will work its way towards your head. So what happens when the myelin layers are completely opened up and damaged to the point that there is bare spots of nerve for substantial distances? Now that the damage is this bad we will now be experiencing paralysis. The heavier damage will allow the signal to simply just get lost before ever reaching its destination and this is because there is nothing containing it. A good example is trying to run water through a hose to a sprinkler in your garden. A good hose gets the water to the sprinkler giving it full power to spray the water over the whole garden, if we cut a few small holes in the hose the water pressure at the sprinkler will get weak because the water is actually leaking out in various spots along the way. Now if we totally damage the hose by cutting a huge piece of the hose out then the water won't get to the sprinkler at all. This my friends represents being paralyzed. -

Now that the signal from the brain is simply not going the distance to activate the movements in our body we experience total loss of function. Again this is an ascending experience so we will feel this paralysis continue to climb up the body and eventually it finds its way into the torso and this is where a GBS experience becomes even more serious than it already was. Once its made its way into the upper body we run into a whole new problem when the muscles that activate the diaphragm and whatnot to control our lung function in order to breathe quit working. This turns into respiratory failure meaning we are no longer breathing. The caring medical professional will then perform the process of intubation by installing a mechanism in the trachea to connect to a ventilator that will be doing the breathing for us at this point. After this the paralysis can continue up into the the head in some cases causing the facial muscles to quit responding and sometimes even causing the eyes to quit working.  Cases that get this bad to where intubation is required and a full body paralysis has set in are very much a flip of a coin situation. Some people do not survive this and some get lucky like myself. These cases really vary from patient to patient. The length of time it takes for the onset to hit a plateau varies so much. My case was a fast onset. From the first tingly feeling in my fingers it was a matter of 48 hours to completely paralyze me 100%. I interviewed patients that had similar onsets but then I also interviewed patients that experienced the weakness make its way to the waist and it took a few weeks to get that far. It is important to understand the differences in the cases in the event you experience a weird sensation you can at least keep track of the initial start and be able to accurately communicate with your medical provider because those little starting details can be a big help in getting right on the diagnosis of GBS.

When a patient goes into a hospital with this ascending weakness and growing paralysis timing is a sensitive thing. The faster they can make a confirmation, the faster the right method of treatment to neutralize and kill the effects can be put into play. This is why I stress to everyone I consult with that once you have a feeling where your hands or feet begin to symmetrically weaken or get that sleepy uneasy feeling write it down. Even if it turns out to be nothing, knowing when that timing starts is a huge help in developing an accurate onset rate and realistically being able to determine the speed of the onset could be a life saver. There are two main tests that work hand in hand for diagnosing GBS. The spinal tap or puncture to collect a sample of spinal fluid is a key study. The cerebral spinal fluid has a steady balance of levels. Mainly derived from blood plasma with a higher chloride content and a fairly equivalent sodium level , it is nearly protein free, but when experiencing Guillain Barre Syndrome those protein levels increase. This increase in protein is most likely caused by the associated inflammation at the nerve roots. Myelin is made of protein and fatty substances so when those roots are experience the effects the protein from the roots ends up in the spinal fluid. A second supporting study is the nerve conduction study. This study is graded by an average baseline of measurement of time it takes for an electrical signal to travel through the nerve. If the speed from point A to B is slow then that could most likely be a sign of damage to the myelin nerve coating. So increased protein levels in the CSF and a weakened response time in a conduction study are the two main factors when diagnosing GBS.

After making a diagnosis GBS is typically first treated with an infusion cycle called IVIG, Intravenous immunoglobulin. Immunoglobulins are the antibodies that are naturally produced by the body's immune system that help fight infections and diseases. IVIG is immunoglobulins that have been extracted from donor blood cells that is infused into the body's blood. These antibodies are collected from thousands of people to give a very diverse and concentrated collection to have a capability of assisting with a wide range of possible infectious organisms the body might encounter. Its like the body's military, there's multiple branches of the military to handle any kind of threat and its the same with the antibodies in our body, many different types for different jobs to handle different threats. They will usually do 1 bag of IVIG per day for about 5 days. If the IVIG just isn't quite putting a stop to the advancements of the attack then another step often taken is Plasmapheresis. Plasmapheresis is known as therapeutic plasma exchange meaning the liquid portion of the blood (plasma) is separated from the blood cells and either replaced or treated or cleaned and then returned into the blood cells. I think of it as pulling your blood out and cleansing it to rid of all the negative antibodies that might be attacking the immune system. After treating it and returning it to the body it gives the immune system more of a fair undisturbed chance to actually fight the disease. Once the treatment is effective the GBS attack is then in recovery mode which means its time for the body to engage the right element to begin repairing the myelin damage which is at a rate of around a millimeter per 24 hours of so. It is a very slow process. With this repair process we also work on consistent physical rehabilitation to start conditioning the body. This is not a fast process. Sometimes months and even years in some cases of physical therapy. 

After GBS has taken its toll on the Myelin sheath around the nerves and the damage is done the recovery is extremely slow. Depending on the severity of the damage it could take months to even years to regain a healthy status of the nerves. Damaged nerves tend to repair at an average of a millimeter per 24-48 hours. If you have severe damage from your feet all the way to your head that could take a very long time to actually recover or like in my case the damages could hold a permanent status and a person could suffer from the residual effects for the rest of their life. With this damage and the length of recovery time comes another huge factor that goes beyond the damage of the nerves and that is the deterioration of the muscles and tendons. When the body isn't moving the fibers in the muscles and other soft tissues begin to shrink. This is called Atrophy which is the wasting away or degeneration of the tissue's cells. The tendons tend to harden as they shrink and the muscles seem to disappear. Joints star to seize up do to the hardening and deterioration of the tissues and it really doesn't seem to take long but stretching the tissues out and rebuilding the muscles takes quite a long time. This is why some call GBS an abbreviation for Getting Better Slowly. One strong GBS occurrence can happen throughout a timeframe of about 4 weeks until it hits its plateau causing significant damage that ends up taking years to recover from. Stretching and taking advantage of every movement as it comes is a huge factor in recovery from GBS.

What you've just read is my personal input specifically on Guillain Barre Syndrome. This is just the surface of these types of mystical autoimmune diseases. CIDP is basically the chronic, more frequent version of GBS. CIDP stands for Chronic Inflammatory Demyelinating Polyneuropathy which makes it repetitive. So imagine the symptoms of GBS but more reoccurring. Instead of plateauing and then recovering over a period of time CIDP more less peaks and you begin to recover, sometimes completely and then here it comes again. That up and down, up and down sequence is a long term term thing  with CIDP where Guillain Barre can have residual long term effects but after a typically larger main event if that makes sense. With that said though GBS can reoccur as well. I have interviewed multiple patients that have had it a more than once. Two, even three times seems to not be too uncommon. GBS effects everyone differently and there are a few different variants such as. AIDP (Acute Inflammatory Demyelinating Polyneuropathy) is a progressive weakening of the motor nerves and some sensory, about 20% of AIDP patients experience respiratory failure (like myself). AMAN (Acute Motor Axonal Neuropathy) is probably the most uncommon consisting of less then 10% and presents distal weakness, absent tendon reflexes but is considered non-inflammatory. AMSAN (Acute Motor and Sensory Axonal Neuropathy which is similar to AMAN but also shows effects in the sensory nerve response as well. Axons are specialized nerve extensions that transmit nerve impulses so instead of losing the signal to the damage of the myelin sheath that contains the signal, this is a disruption due to the neurons themselves being damaged. Miller Fisher Syndrome is the 4th recognized variant of GBS. MFS is characterized by abnormal muscle coordination, absence of tendon reflexes, and a common MFS symton is paralysis of the muscles. All these variants have rare and unique effects. Sometimes its a fast onset, sometimes a slow onset as well as some people have lighter cases and are only effected for a few days, maybe a few weeks, where some cases carry heavy damages that effect a person for months, sometimes hears, and sometimes the rest of their life. The biggest trick is knowing the signs and symptoms and having that little bit of education about it so when you do feel like you are having a weird experience you know what to bring up to your doctor, or if you are ever diagnosed with it you know what's going on and the self mystery is minimized. Never ignore the symptoms as the speed that you are diagnosed and begin treatment is a huge factor in how far the damages to the nerves make it. I figured out if you can catch it and get the progression stopped before it gets into your respiratory system it makes it is way better for the entire case. Intubation due to respiratory failure adds so much strain on the body and makes recovery more difficult. The cases that get to the point of intubation also have a much higher chance of having fatal effects. I want to tell everyone that took the time to read this page on GBS awareness thanks and I encourage everyone to share anything you've read here, you might be saving a life!