Celebrities and symptoms of ALS
The full name of ALS is amyotrophic lateral sclerosis (ALS), which is a degenerative disease of autonomic motor neurons. Here Note the difference from Parkinson's disease. The neurons affected by Parkinson's disease are dopamine neurons in the internal brain areas and do not directly cause muscle atrophy. However, once ALS is diagnosed, the patient's survival time is basically only 2-4 years. During this period, the condition deteriorates rapidly and the symptoms are very obvious. The word "ALS" vividly describes some of the symptoms of ALS. The main symptoms after diagnosis are muscle spasms, muscle atrophy and loss of strength. The early outward manifestation is weakness in the hands and feet, and symptoms of weakness increase appreciably month by month. From being unable to run, it gradually progressed to difficulty walking, and then to falling easily when walking; unable to make a sound when speaking, and then difficulty swallowing when eating. In the middle and late stages, some patients will have symptoms of neuralgia, and some will develop cognitive or behavioral disorders. Finally, as the muscle strength disappeared, breathing became impossible, and the weight dropped severely, the patient also reached the end of his life. However, it is worth noting that patients with pure ALS generally have normal bowel function, their bladder is still under patient control, and their eye muscles can still move.
The most well-known ALS patient at present is the famous physicist Stephen William Hawking, author of "A Brief History of Time". Hawking was diagnosed with ALS in 1963, when he was only 21 years old. Ah, it is the beginning of the golden time of life, which is undoubtedly a blow to the young Hawking. Fortunately, Hawking was not intimidated by the disease. He struggled with ALS in a wheelchair for 55 years. This was the longest case of fighting this terrible disease since there was a clear record of ALS. What is even more admirable is that during this period, Hawking wrote popular physics works such as "A Brief History of Time" and "The Universe in a Nutshell". He also made considerable achievements in the study of quantum cosmology and black holes. Hawking is not suffering from early-onset learning and cognitive dysfunction, but a motor function disease. After the illness, Hawking's mind can continue to benefit mankind, which can be said to be a blessing among misfortunes. Hawking passed away in 2018 at the age of 76. It has to be said that he is a legend.
Coincidentally, another ALS patient named Stephen Hillenburg also died of ALS in 2018. Many people may not have heard of his name, but his work "SpongeBob SquarePants" can be said to be popular all over the world. The versatile Hillenburg is a marine biologist and animator, and is also a successful businessman. Unfortunately, Unfortunately, he was diagnosed with ALS at the age of 56 and died suddenly a year later.
Discovery and naming of ALS
A British doctor, Charles Bell, described in detail in 1824 a disease that caused muscle atrophy and resulted in death of the patient. Judging from the symptoms recorded at that time, it can be determined that it was ALS later. However, the disease was not accurately named and defined at that time. 45 years later, the famous French neuroscientist Jean-Martin Charcot (Charcot for short) discovered ALS and its related pathological anatomical symptoms in his research, and after a series of modifications, he finally analyzed this This disease is different from many other motor nerve diseases, and it was officially named amyotrophic lateral sclerosis in 1874. Although this disease is fatal and the symptoms are very severe, the incidence rate among the entire population is not very high. Therefore, except for patients and their families, society is very unfamiliar with this disease.
Until 1939, the popular star of the famous American baseball team, the New York Yankees (although baseball is not a popular sport in China, New York Yankees baseball caps with NY on them are very popular), was nicknamed "Iron Horse" Lou Gehrig delivers his farewell speech as he leaves the court where he set countless records. In fact, 6 years before he retired, Glick began to feel the loss of muscle strength, and his condition worsened year by year. Until the year of retirement, he was completely unable to persist. However, even after retiring, the disease did not let go of Glick. The legendary star died of ALS in 1941 at the age of 39. Flags in New York City flew at half-mast following his death, and the following year a documentary film about Glick, "Yankee Lights," was released. This shows the status of this athlete in the hearts of Americans, especially the people of New York.
Therefore, for a long time, even with the official name of ALS, the disease has been called "Glick's disease" in the United States.
Epidemiological study of ALS
ALS is the most common motor neuron disease (Parkinson’s disease damages the brain, but the motor neurons are not damaged), and it is also a neurodegenerative disease. Parkinson’s, the “third eldest brother” and “second brother” among degenerative diseases, has been introduced before (you can click here to review), but the eldest brother “Alzheimer” has not yet appeared. The incidence rate (the proportion of new patients per year) of ALS is 1.9/100,000, while the prevalence (the proportion of existing patients in the total population) is 4.5/100,000. The disease has a higher incidence rate in countries such as Europe and the United States, which is 2.6/100,000, and the prevalence rate has increased to about 7-9/100,000. Like Parkinson's disease, it is more common in men than in women. At the same time, the incidence rate among white people is also higher than that among other people of color, and among white people, the incidence rate among Anglo-Saxon and Germanic people is significantly higher than that among Latin people. In comparison, the incidence rate in East Asian yellow people is only 0.8/100,000, and in South Asia it is as low as 0.7/100,000. It is precisely because of this that Europe and the United States have invested heavily in ALS research, accounting for more than 80% of the world's research projects. Most of the above data come from Europe, North America, East Asia and South Asia, and data from other regions are relatively scarce. Some studies have found that the incidence rate of ALS in Guam, Kii Peninsula, and West New Guinea is 50-100 times the world average incidence rate. I would like to guess whether it is because the islands are relatively closed, the customs are relatively backward, and incest is caused by intermarriage?
Methods to treat ALS
There are currently only two drugs on the market for ALS. One is Riluzole, which can extend patient survival by an average of 2-3 months and has a better effect on bulbar ALS. However, its mechanism has not been fully elucidated, and current research can only temporarily attribute the effect to the inhibitory effect of riluzole on presynaptic glutamate. However, the drug is three-part toxic and inhibits excitatory neurotransmitters. The consequence is that it will cause severe mental and motor weakness in the patient. Moreover, this drug is also more effective in the early stage. Because complications are complex, many medications are needed to control their occurrence.
The other is Edaravone from Japan. This drug can reduce the disability of various body functions in ALS, but it is only effective in a small proportion of early-onset ALS. The mechanism may be to protect neurons by inhibiting excessive oxidative damage to cells. But it also has serious side effects, and the cost reaches 140,000 US dollars a year, which is much more expensive than billuzole.
The remaining drugs are drugs aimed at relieving the pain of ALS symptoms. Their main functions include alleviating neuralgia, relieving fatigue, relaxing muscles, anti-spasmodic, and suppressing external symptoms such as saliva and phlegm. There are also antidepressant, anti-anxiety and other psychotropic drugs; as well as some drugs that can help cognitive function. I won’t give examples one by one here.
Although there are not many drugs to treat ALS, there are some auxiliary methods that can alleviate ALS. Among them, non-invasive ventilation (NIV) is an auxiliary method for ALS patients in the late stage who have lost the ability to breathe on their own. The principle is very simple. It uses a certain frequency to pump air through a mask that covers the mouth and nose, and then presses it into the patient's lungs from the mouth and nose to assist breathing. This method is usually performed when the patient is sleeping, because late-stage patients who are unconscious are more likely to die from suffocation due to inability to actively breathe. However, this method requires frequent monitoring of the patient's respiratory filling and adjustment of ventilation intervals and intensity. Some studies have shown that the use of NIV alone has a better effect on extending the life of patients than riluzole, and the evaluation results of patients' quality of life are also better. However, about 25-30% of patients with severe cognitive impairment and severe impairment of bulbar function cannot receive this treatment.
Another non-drug treatment method is what we commonly call "intubation", which requires cutting off the trachea and directly inserting an external gas delivery device. It is suitable for patients with weaker respiratory systems for whom NIV is ineffective. Similarly, this method can only keep the patient "alive", and the quality of life will be greatly reduced, and this method is extremely expensive. Different countries, different cultures and different values ??have different acceptance of this therapy. In Japan, 30% of ALS patients can accept "intubation", while in Europe and the United States, only 5% of patients are willing to live longer in this way. moon.