BDC #25 - Goldflam disease: Too tired to open your eyes?

Have you ever met somebody how can open their eyelids properly, even if they want and work hard to? Or somebody who can't really control  his/her facial movement? Drooping jaw, shabby talking? Well, that person might be a patient of Goldflam disease.

It is formally called as Myasthenia Gravis (MG). Based on medical dictionary, it is a disorder of neuromuscular transmission marked by fluctuating weakness and fatigue of certain voluntary muscles, including those innervated by brainstem motor nuclei; caused by a marked reduction in the number of acetylcholine receptors in the postsynaptic membrane of the neuromuscular junvction, resulting from an autoimmune mechanism.

OH!! This definition is definitely NOT FUN!! It ends up my whole post in ONE-FULL-CONFUSING-Sentence. SO, I will explain it again, in more sane-shorter-sentences. Let's restart it ^^

Myasthenia Gravis or Goldflam disease is an autoimmune disease on our neural and muscular system (OR neuromuscular system). It is general caused by abnormal antibodies in the body, either blocking or destroying the receptor/receiver of acetylcholine, one of neural signals from our nerves to our muscles. It is practically cutting the communication between our brain with our conscious muscles. It leads to fluctuating muscles weakness and heavy fatigue, and in a progressive phase losing the control over the conscious muscles. Normally, our unconscious muscles like heart or digestive track (except the throat, since we swallow food 'consciously') will be spared. Means, the patient's heart might keep beating thought the patient cannot move his/her body properly anymore. 

The early symptoms can be listed as:

• Heavy fatigue on patient
• Progressive weakening of muscles during periods of activity
• Eye muscles - including ptosis (drooping of one or both eyelids), diplopia (double vision) and blurred vision.
• Facial muscles - involve the weakening of face and throat muscles, leads to:
• Dysarthria - difficulty in speaking, depends on which muscles are effected; speech may become soft or nasal
• dysphagia - difficulty in swallowing
• difficulty in chewing 
• difficulty in controlling facial expression
•  Weakening of limbs mucles   

There are some classifications for MG, but the most widely used is the one by Myasthenia Gravis Foundation of America Clinical Classification, which are:

• Class I: Any eye muscle weakness, possible ptosis, no oter evidence of muscles weakness .. yet
• Class II: Any severity of eye muscles weakness, with mild weakness of other mucles
• Class IIa: predominantly on limbs muscles 
• Class IIb: predominantly on respiratory muscles
• Class III: Any severity of eye muscles weakness, with moderate weakness of other muscles
• Class IIIa: predominantly on limbs muscles 
• Class IIIb: predominantly on respiratory muscles
• Class IV: Any severity of eye muscles weakness, with severe weakness of other muscles
• Class IVa: predominantly on limbs muscles 
• Class IVb: predominantly on respiratory muscles
• Class V: intubation on the throat needed for airway 

Up to now, there is no specific cure for MG. Luckily, most of the treatments are very effective in controlling or delaying the symptoms. The currently available treatments are:

• Cholinesterase inhibitors: helps the muscles to contract properly and maintain good strength. It is effective for mild symptoms, though some side effects like nausea or stomach cramps might occur.
• Steroids or Immunosuppressants: may alter the body's immune system and lower the production of antibodies that cause the MG.
• Removal of the thymus gland. About 15% of MG patients have a tumor in their thymus gland - thus the surgery might be needed. However, it is unclear whether the risks of removing the thymus gland could outweigh the benefits for MG patients.  
• Plasmaphoresis: is a procedure of depleting the body of blood plasma without depleting the body of its blood cells. To put it short, the blood is taken put of the patient, the abnormal antibodies are removed, and the blood is placed back to the patient. OK...It DOES sound scary!!!
• Intravenous immunoglobin therapy: is a procedure of injecting normal antibodies that alter the way the of the immune system. However, the last 2 treatments only give short effect lasted only weeks for the patient. 

In the end...be grateful of your full control over your body !!! ^^