"World ALS Day" has not been long ago, but everyone's thoughts about Hawking have never stopped. Hawking was an expert in cosmology and black holes during his lifetime. Before Hawking, the universe had always been a vast and mysterious existence to humans. The emergence of Hawking helped people uncover the mystery of the universe.
After studying black holes, Hawking proposed the black hole evaporation theory and the universe model theorem about the universe, which made outstanding contributions to further human research on the universe. Therefore, he is also considered the greatest scientist of the 20th century. Genius physicist.
However, the life of a genius may be destined to be extraordinary. When he was 21 years old, Hawking was diagnosed with a rare disease, ALS . This disease had tortured him for nearly 55 years until March 14, 2018, when Hawking ended his legendary journey. Life. Hawking not only left people with his great achievements, but his ALS also attracted more attention. Today, I will introduce this disease to you in detail.
There is no cure for ALS, and death is too real.
Before Hawking, ALS was not well known, or even unheard of. ALS is one of the world's five major terminal diseases. It is a chronic, progressive neurological degenerative disease, also known as amyotrophic lateral sclerosis (ALS).
People suffering from ALS will gradually develop symptoms such as muscle atrophy, muscle tremors, , general paralysis, and speech disorders . People look as if they are frozen, which is why the name "ALS" comes from.
After Hawking suffered from this disease, as his condition continued to worsen, he could only move three fingers and needed to rely on special input methods to communicate with others.
Usually, the age of onset of ALS in clinical practice is mostly around 50-60 years old, and the survival time after the disease is usually only 2 to 5 years. People like Hawking who develop the disease at a young age and survive for a long time are very rare. According to statistics, only 10% of ALS patients in the world have survived for more than 10 years. [1]
The reason why patients with ALS have a shorter survival time after onset is that after the onset, the muscle atrophy progresses quickly and the condition progressively worsens until the patient has difficulty swallowing and breathing, and respiratory failure dies.
What factors cause ALS is not yet clear, but experts speculate that it may be related to genetic genes, , the environment, aging and other factors . As people age, their metabolism and repair abilities will also weaken. If they are affected by adverse factors, it may lead to diseases.
In addition, some experts once believed that the onset of ALS is also closely related to virus infection, excessive alcohol, chemical radiation and other factors, but this is not absolute.
Some experts have tried to explain the pathogenesis of ALS using the amino acid toxicity theory, the human immunity theory, and the abnormal neurofilament metabolism theory , but there is no unified conclusion.
Since the cause of ALS is unclear and cannot be cured, current medical treatments only focus on helping patients delay the progression of the disease and prolong life. However, patients still need to receive early treatment. Therefore, it is very necessary to understand the early symptoms of ALS so as not to miss the opportunity for treatment.
The early symptoms of ALS are easily confused, so be careful when these phenomena occur.
The symptoms of ALS in the early stages are usually not obvious, and it is very easy to be confused with other diseases. If these phenomena occur, everyone should be alert to whether it is ALS.
There are usually three signs of early onset of ALS: 1. Difficulty in fine movement of the upper limbs; 2. Difficulty in walking on one side of the leg; 3. Language impairment.
Among these three signs, difficulty in fine movement of the upper limbs is the most common one. The main manifestation is that it is difficult for the hands to complete fine movements, such as being unable to control the glyphs of writing, having trouble opening the door with keys, and being unable to pick up food while eating.As the disease progresses, muscle atrophy of the upper limbs will appear in about 2-3 months. There will also be dents in the muscles at the thumb of the hand. Then it will gradually develop to difficulty in raising the arm on one side, until later on both upper limbs will become numb. Unable to exert force.
However, it is worth noting that patients with upper limb disease are often confused with and cervical spondylosis . cervical spondylosis is caused by degenerative disease of the intervertebral disc or vertebral bone hyperplasia. The pathogenesis of between the two is completely different.
Additionally, some symptoms differ between the two. Although cervical spondylosis will also show symptoms such as limb weakness , it will not cause muscle atrophy in the hands and upper limbs. This can be found through electromyography , MRI and other multiple examinations in the hospital.
Although most patients develop symptoms from the upper limbs, there are also patients who start from the legs, but the probability of is much smaller than that of the upper limbs. Symptoms first appear on one side of the lower limb. It can be manifested as a sunken instep on the instep when walking, and it is easy to fall down when encountering some obstacles. As the disease progresses, it will become more and more difficult for the patient to walk. However, there will be no symptoms of numbness and pain in the legs during the onset.
Patients with lower limb disease are often confused with lumbar disc herniation. In clinical examination, the diagnosis can also be confirmed through comprehensive factors such as MRI and electromyography.
There are also signs of language barriers in and . To put it simply, it means difficulty in speaking. Because the muscle atrophy in ALS is systemic and affects the muscles of the head, patients may be unable to control facial muscles and may have difficulty speaking.
And this onset phenomenon is easily confused with stroke . However, stroke usually occurs suddenly and can be recovered after treatment. However, ALS is persistent, and symptoms such as dysphagia and other symptoms may appear in the later stages of language disorder.
In addition, many patients have the phenomenon of muscle beating in daily life, so they wonder whether they have ALS. In fact, this kind of worry is completely unnecessary. There are many reasons for muscle twitching, such as mental stress, overwork, poor sleep quality, etc. If there is no muscle atrophy and weakness, ALS is usually not considered. .
Although the early onset of ALS is hidden, with the advancement of science and technology, suffering from ALS does not mean death. Because the current medical level can delay the patient's condition, especially the mature of eye movement input technology can also solve the interpersonal communication problems of patients.
Medical exploration will never end, and patients with ALS will have more hope.
The universe is boundless, and human exploration is endless. Although terminal diseases cannot be completely defeated, humans have never succumbed to them. For example, although ALS is rare, good progress has been made in the fight against it.
As of today, there are many medical treatments available to delay the progression of patients' disease, such as the drugs approved by the US FDA for the treatment of ALS: riluzole and edaravone.
Riluzole is more targeted at patients in the middle and early stages. It can help patients prolong their survival time without the need for intubation or tracheotomy. However, the effect is not good for patients in the advanced stage. Edaravone is a brain protective agent. The main function of is to prevent the progression of cerebral edema and cerebral infarction . It can relieve neurological symptoms. inhibits delayed neuronal death. However, some studies have shown that the drug can cause acute renal failure , abnormal liver function and other adverse reactions .
In addition, according to expert research, high doses of methylcobalamin can delay the progression of ALS , and this drug may also be launched in my country in the future. There is also sodium phenylbutyrate, ursitating cholic acid, which also shows positive results in delaying the disease of ALS, , but there is a lack of clinical trials and it is not yet available on the market.
In addition to drug therapy, another important measure is supportive therapy .For example, patients can be given drugs to relieve salivation and muscle spasms , and the patient's respiratory function can be monitored regularly. If breathing difficulties occur, non-invasive ventilators can be used. If the patient has difficulty swallowing, liquid food can be chosen to supplement the patient's health. Nutrition, if the swallowing situation is serious, the patient should undergo gastrostomy surgery or nasogastric feeding diet method as soon as possible.
can also perform rehabilitation therapy for patients. An effective rehabilitation program and careful care are very important to improve the condition of patients with ALS. Research shows that continuous rehabilitation training can effectively delay muscle atrophy, avoid diseases such as aseptic arthritis and pneumonia, and prolong the life of patients.
Finally, even if you suffer from ALS, you should try to maintain a positive and optimistic attitude and receive treatment. A negative attitude will not improve the condition, but will make it progress faster. Hawking suffered from ALS at the age of 21. The reason why he has been able to fight against it for more than 50 years is inseparable from his positive and optimistic attitude.
After falling ill, in addition to active treatment, Mr. Hawking also completed the amazing work "A Brief History of Time: From the Big Bang to the Black Hole" even though he could only use three fingers, which shows his strong and optimistic attitude. . In addition, he also wrote children's physics books with his daughter, participated in performances, and experienced weightless flight, etc., providing spiritual strength to many ALS patients.
Based on the above, although ALS is rare, everyone should be reminded to take precautions in life. From living environment to diet and daily life, everything is closely related to health. A good physical foundation is the best barrier against disease.
References
[1] Wei Xing. ALS.[J] Chinese Scientific and Technological Terminology. 2018, 20(2)
[2] Xiong Jian. Analysis and diagnostic value of electromyographic manifestations of amyotrophic lateral sclerosis.[J] ] Contemporary Medicine. 2019, 25 (25)
[3] He Yunhan. Research progress in the treatment of amyotrophic lateral sclerosis. [J] Modern Journal of Integrated Traditional Chinese and Western Medicine. 2020, 29 (6)
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I am Dr. Li Ning. Thank you for reading patiently in your busy life. I hope my sharing can help more people prevent and stay away from diseases. If you have any confusion about the article, you are welcome to leave a message and I will actively answer your questions.
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