The soul is separated from the body, and people's real feelings are gradually frozen

Pete frates, an athlete suffering from "progressive frostbite" in 2014, aims to make more people around the world pay attention to and help the "progressive frostbite" patients through the "ice bucket challenge" On December 10, 2019, this admirable character, after all, failed to bear the suffering of the disease and died Why don't we look back on the significance behind the ice bucket challenge while we remember this "great athlete" and "hero"? Photo source: Southern Metropolis News The rule of "ice bucket challenge" is simple: the person who receives the challenge should pour a bucket of ice water on himself within 24 hours (to feel the real situation of numbness and weakness of patients with "progressive frostbite"), record the challenge process into a video and upload it to social network, otherwise donate $100 to ALS charity; after the challenge is completed, other people can be named It is such a simple and interesting activity that makes "progressive frostbite" really come into people's vision China's 200000 patients with progressive frostbite are no longer "watching their own death", but more people are willing to help them and more pharmaceutical companies are willing to develop new drugs for them What is "Involution" and "Involution" what is a disease? The full name of "progressive frostbite" is amyotrophic lateral sclerosis (ALS), which is a neurodegenerative disease characterized by the loss of motor neurons in the spinal cord, brain stem and motor cortex The main clinical manifestations are progressive skeletal muscle weakness, atrophy, fascicular fibrillation, bulbar palsy and pyramidal tract sign [1] It is also because of its gradual muscle atrophy and weakness, and even paralysis, it seems that the body is gradually frozen in general, so it is commonly known as "gradually frozen man" Throughout the course of the disease, he has always maintained a clear consciousness and good sense of skin Some patients said frankly: since the onset of the disease, the biggest feeling is that gradually lost the control of the body, as if the soul gradually pulled away from the body in general, want to struggle, but not even strength! This reminds me of the state of "Naruto" after the big snake pill was sealed, how similar! Photo source: photo web - Hawking's life is only the last few years since he became ill And most people, even after they have a preliminary understanding of "ice bucket challenge", seem to stay on the concept of "nothing but being unable to move", but what they don't know is that in general, it's only 3-5 years from the onset of symptoms to the death of respiratory paralysis Mapping: the pathogenesis of yaozhiwang and sun Liying's "progressive frostbite" (ALS) in patients with "progressive frostbite", the family patients account for about 5% ~ 10% and the sporadic patients account for about 90% ~ 95% [2] At present, the pathogenesis of ALS is still unclear, and different theories have been put forward in various fields of basic medicine There are mainly the following points: gene mutation: in recent years, there are about 20 kinds of ALS related genes have been found, among which SOD1, tartdp, Fus, ang and OPTN can cause typical clinical manifestations after mutation; Neuroexcitotoxicity: glutamate is the main excitatory neurotransmitter in the central nervous system Normally, glutamate combines with receptor to cause postsynaptic membrane excitation, but its overexcitation will produce excitotoxicity to postsynaptic neurons and surrounding tissues Mitochondrial abnormalities: in ALS lesions, there are abnormalities of mitochondrial structure and function of motoneurons Oxidative stress: oxidative stress is easy to cause damage to the central nervous system, on the one hand, it is due to the high oxygen consumption of neuron cell membrane, on the other hand, it contains relatively insufficient antioxidants [3]; immune response imbalance: the imbalance of human immune system may attack the normal cells, which may lead to the death of nerve cells [4] But there is no one who has been widely recognized, that is, why the disease is still unclear! At present, there are only two drugs in patients with progressive frostbite, but the effect is limited The mechanism of riluzole (Rilutek) - riluzole is not clear Its effect may be related to the inhibition of glutamate release, the stabilization of voltage-dependent sodium channel inactivation, the interference of neurotransmitter and excitatory amino acid receptor binding intracellular events The most common side effects of riluzole were asthenia, nausea and liver dysfunction Edaravone (radicava) - FDA approved edaravone for ALS in 2017 and registered with the National Drug Administration (nmpa) on July 31, 2019 The mechanism of edaravone to slow down the decline of body function in ALS patients is not clear Edaravone is a kind of free radical and peroxynitrite scavenger, which can prevent the oxidative damage of cell membrane and may help to inhibit the progress of ALS Radicava was administered intravenously The most common adverse reactions were contusion, gait instability, and headache But these two drugs are not only expensive in price, but also unsatisfactory in effect To get rid of the disease by drugs really requires not only the early use of drugs, but also the number of people who are cured is very small It seems that in addition to the incidence rate below the pale color of cancer, no matter the symptoms, survival cycle or happiness, it is more terrible than "cancer" Nowadays, "progressive frostbite" is still a rare disease that can't be cured In addition to early diagnosis and treatment as soon as possible, and then through the use of drugs to delay the development of the disease, giving patients good nutritional support, respiratory support and psychotherapy, more treatment aims are just to better improve the quality of life of patients and prolong their lives Deposit period 【1】 Electromyography and clinical electrophysiology group of Neurology branch of Chinese Medical Association, neuromuscular disease group of Neurology branch of Chinese Medical Association Diagnosis and treatment guidelines of amyotrophic lateral sclerosis in China [J] Chinese Journal of Neurology, 2012,45 (7): 531-533 [2] kiernamc, vucics, cheahbc, et al Amyotrophic neurosclerosis [J] Lancet, 2011377 (9769): 942-955 [3] d'amicoe, Factor litvakp, santellarm, et al Clinicalperspective oncoxidative ressporadicamyophilalclerosis [J] Freeradicalbiomed, 2013,65:509-527 [4] Guo Yongqiang, Li Sen, Zhang Haihong, Wu Yamin Research progress of pathogenesis of ALS [J] Rehabilitation Theory and practice in China, 2017, 23 (06): 685-689 Responsible editor | penicillin statement: This article is the original article of Yaozhi online, the viewpoint only represents the author, not the position of Yaozhi online, welcome to exchange and supplement in the message area; if you need to reprint, please be sure to indicate the author and source of the article.