The soul is stripped of the body, the real feeling of the frozen man.

Pete Frates, an athlete with "frozen disease" in 2014, used the Ice Bucket Challenge to get more people around the world to pay attention to and help the "frozen" populationDecember 10, 2019, the admirable figure, after all, still failed to bear the torture of the disease, left the worldWhile we remember this "wonderful athlete" and "hero", why not look back at the meaning behind the Ice Bucket Challenge?the rules of the Ice Bucket Challenge are simple: those who receive the challenge will be able to pour a bucket of ice water on themselves within 24 hours (to feel the reality of the numbness of the "frozen" patients) and video the challenge into a social network, or donate $100 to the ALS charity;is such a simple and fun activity, so that "frozen" really into people's vision, China's 200,000 people suffering from frozen disease is no longer "watching their own death", but more people are willing to help them, more pharmaceutical companies are willing to develop new drugs for themwhat iswhat is "frozen""frozen" what kind of disease is? "Frozen" is called amyotrophic lateral sclerosis (Amyotrophilateralals.ALS), a neurodegenerative disease characterized by the loss of motor neurons in the spinal cord, brain stem and motor cortex, with progressive skeletal muscle weakness, atrophy, muscle-beam fibrillation, sementalgia and cone beam signs as the main clinical manifestationsalso because of its performance of muscle gradually atrophy and weakness, to paralyze, it seems that the body is gradually frozen in general, so commonly known as "frozen man", the entire course of the disease, he always maintaina conscious and good skin feeling abilitypatients to speak up: since the disease, the biggest feeling is, gradually lost control of the body, as if the soul gradually withdraw from the body, want to struggle, but not even strength!this makes me think of the "Ninja" in the big snake pills sealed soul after the state, how similar!the onset of the disease, life is only the last few years ofand most people even through the "ice bucket challenge" after the initial understanding of frozen disease, its concept seems to just stay in "in addition to not moving, no big deal", but they do not know that, in general, from the onset of symptoms to respiratory paralysis death, only a short 3-5 yearsmapping: medicine, Sun Liying"frozen" (ALS) pathogenesis
in the "frozen" patients, familial patients about 5% to 10% and distribution about 90% to 95%At present, the pathogenesis of ALS is still unclear, different theories are put forward in various fields of basic medicine, mainly the following: gene mutation: in recent years, there have been alsa disease-related genes found, so far reported about 20 kinds, of which SOD1, TARTDP, FUS, ANG and OPTN gene mutations can cause typical clinical performance; neuroeuphoria toxicity: glutamate is the main excitatory binder in the central nervous system, normally binding to the receptor to cause synaptic after-film excitability, but its over-excitement can produce excitatory toxicity to neurons and surrounding tissues after synaptic mitochondrial abnormalities: In ALS lesions, there are abnormal mitochondrial structure and function of motor neurons oxidative stress: oxidative stress is prone to central nervous system damage, on the one hand, because of the high oxygen consumption of neuronal cell membranes, on the other hand, because of the relative lack of antioxidants in them, immune response imbalance: the imbalance of the body's immune system may attack their normal cells, which may lead to the death of nerve cells but really widely recognized but not a single, that is, why the disease is not clear! the only two drugs in patients currently suffering from defrosting, but also limited effect
Riluzole(Rilutek) - the mechanism of action of Liluazole is not clear, its effects may be inhibited glutamate release, stable voltage-dependent sodium channel inactivation state, interference with neurotransmitters and excitatory amino acid receptor binding intracellular events Liluquin is given in tablet form, and the most common adverse reactions are weakness, nausea and abnormal liver function , Radicava - The FDA approved Ida LaDea for the treatment of ALS in 2017 and was approved by the National Drug Administration (NMPA) on July 31, 2019 The mechanism by which Ida lafeng slows down the decline in bodily function in PATIENTs with ALS is not clear, and Idalaison is a free radical and peroxidase nitrite scavenger that prevents cell membrane oxidation damage and may help inhibit the process of ALS Radicava is administered intravenously, with the most common adverse reactions being bruising, gait instability, and headache and these two drugs, the price is not only expensive, but the effect is not satisfactory, really rely on drugs to get rid of the disease, not only require early onset of medication, but also the number of cures is very rare so, in addition to the incidence of less than everyone talkaboutd about the color of the "cancer", regardless of symptoms, survival cycle, or happiness in life, are more terrible than "cancer"! today"s "frozen disease" is still an incurable rare disease, in addition to early diagnosis, early treatment, and then through the use of drugs to delay the development of the disease treatment, to give patients good nutritional support, respiratory support and psychological treatment and other treatment methods, the purpose of more treatment is only hope to better improve the quality of life of patients, extend the survival of patients 1) Chinese Medical Association Neurology Credit Synome and Clinical Neuroelectrophysiology Group, Chinese Medical Association Neurology Credit Society Neuromuscular Disease Group Chinese Muscular Dystrophy Lateral Sclerosis Diagnostic and Therapeutic Guidelines 2: Kiernan MC, VucicS, CheahBC, etal Amyotrophiclateraliss Lancet, 2011, 377 (9769): 942-955 3 D'AmicoE, Factor-LitvakP, Santella RM, etal Clinicalperspective onononvestinsporadicamiamtrotroprthalalals FreeRadicalBioMed, 2013, 65:509-527 4) Guo Yongqiang, Li Sen, Zhang Haihong, Wu Yamin Research Progress on the pathogenesis of amyotrophic lateral sclerosis.