"Frozen human disease" is a common name for a group of motor neuron diseases. Because the motor neurons in the patient's brain, brain stem and spinal cord are attacked, the patient's muscles gradually shrink and weaken, and even the whole body is paralyzed, so it is commonly known as "gradually frozen person". Because the sensory nerves are not invaded, this disease does not affect the intelligence, memory and feeling of patients. Hawking, a famous master of theoretical physics and a great scientist, is a "frozen man". "Frozen people" is characterized by progressive degeneration of motor nerve cells (neurons) in the brain and spinal cord. Because motor neurons control the activities of muscles that enable us to move, speak, swallow and breathe, without nerve stimulation, muscles will gradually atrophy and degenerate, showing muscle weakness and paralysis, and the functions of speaking, swallowing and breathing will decline until respiratory failure and death. Because the sensory nerve is not invaded, it does not affect the intelligence, memory or feeling of the patient. The development of diseases is generally rapid and ruthless. From the onset of symptoms, the average life expectancy is between 2 and 5 years. The "frozen man" is also called Lou Gehrig's disease, because the famous American baseball star Roger Herrich died of this disease. Related classification Clinical symptoms can be roughly divided into two types: limb invasion (1), starting with limb invasion: first, the muscles of the limbs begin to atrophy and weaken, then spread to other places, and finally respiratory failure occurs. Medullary palsy (2) begins with Medullary palsy: when the limbs move well, it is difficult to swallow and speak, which quickly progresses to respiratory failure. The medical profession is not quite clear about the cause of this disease. Five percent of the cases may be related to genetic and genetic defects, some of which are environmental factors, such as heavy metal poisoning and lead poisoning, but 90 percent of the cases are primary, that is, the reasons are unknown. It is believed that the main reason of motor nerve atrophy is that glutamate in cells can not be metabolized, and it accumulates in motor neuron cells to produce toxicity, which leads to nerve cell atrophy. The process of gradual freezing is 1. Symptoms begin: at the beginning of the disease, you may not be able to hold chopsticks with both hands, or you may walk and fall for no reason; Some began to have hoarseness without any obvious symptoms. At this time, electromyography, nerve conduction velocity, magnetic resonance vibration and other necessary examinations should be carried out by neuromuscular doctors to make a clear diagnosis. 2. Hard work period: the body is obviously weak or even shrinking, and life can take care of itself, but obstacles have appeared in the workplace. At this time, you need a moderate rest to avoid getting worse. Assessed by rehabilitation doctors to provide necessary rehabilitation, and assisted by social workers for psychological adjustment and social resources. 3. Difficulties in life: the course of the disease enters the middle stage, and there are serious obstacles in hands or feet, or both hands and feet, so they can't take care of themselves, and they may not be able to walk, dress, hold chopsticks, and have unclear speech. 4. Dysphagia: In the middle and late stage, the limbs are almost completely weak and the speech is seriously damaged. Eating is easy to choke, and some need tube feeding, otherwise it will easily lead to aspiration pneumonia. 5. Dyspnea: The respiratory muscles are affected and it is difficult to breathe. Patients may stay in bed and need a respirator. Some patients will live in a respiratory treatment center or choose home care, while others will be served by a hospice care team. Clinical manifestations 1) According to the severity of the lesion, there are two symptoms: 1. Symptoms of superior neurons include: poor fineness, muscle weakness, spasm, pathological reflex, bending spasm and false marrow sign. 2. Symptoms of lower neurons include muscle weakness, muscle atrophy, decreased reflexes, muscle tremor, and easy cramping. At first, it may only be common symptoms such as limb weakness, muscle twitching and fatigue, which will gradually progress to muscle atrophy, dysphagia and finally lead to respiratory failure. 2) According to the different initial symptoms, it can be divided into two disease development modes: 1, which begins with limb invasion: limb muscles atrophy and weaken from one place, then spread to other parts, and finally respiratory failure occurs. 2. It begins with bulbar palsy: when the limbs are still in good motion, symptoms of swallowing and speech difficulties have appeared, and then they will soon progress to respiratory failure. Without effective treatment, success is likely to come from the so-called "cocktail" therapy, that is, comprehensive treatment for multiple possible causes. A variety of therapeutic combinations for different aspects of ALS diseases include: anti-glutamate drugs, neuroprotective agents, neurotrophic factors, symptomatic treatment and stem cell gene therapy. Only riluzole (rilutek) is approved for the treatment of ALS. Clinical trials have confirmed that riluzole can prolong the life of als patients for 3 to 6 months, and its main function is to delay the deterioration of the disease, rather than radically cure ALS. In addition, some experiments show that vitamin E can delay the deterioration of the disease, but it will not prolong the life of patients. Many other potential drugs, including epilepsy drugs, COX-2 selective inhibitors and adjuvant drugs, are in clinical trials. 1, and anti-glutamic drugs include 1) riluzole (daily 100mg), which is the only confirmed effective drug at present. Although the effect is limited, it is the beginning of effective drug treatment. ; 2) lamotrigine; 3) Branched amino acids, including L- leucine, L- isoleucine and L- valine, mainly enhance the oxidation of glutamic acid; 4) gabapentin; 5)D- methoxymethyl morphine. The latter four require more clinical trials to evaluate the efficacy. 2. Neuroprotective agent includes 1) vitamin E; (2) Si Laijilan; (3)N- acetylcysteine; (4)DHA dehydroepiandrosterone. The neuroprotective effect of neuroprotective agents has not been confirmed. 3. Nerve growth and nutritional factors: insulin-like growth factor-1. Symptomatic treatment includes noninvasive biphasic positive pressure ventilation (BIPAP), percutaneous endoscopic gastrostomy (PEG), mucolytic agent, antidepressant treatment, sleeping pills and daily life care. Physical therapy maintains good muscle strength and athletic ability, and maintains the independence of daily life function under the condition of physical and mental comfort, thus improving the quality of life and highlighting the brilliance of life. According to the pain situation, physiotherapists can use various hyperthermia and electrotherapy, such as hot compress, wax therapy, short wave, laser, ultrasound, transcutaneous nerve stimulation and so on. , or help him massage and exercise with omnipotent hands, all have the function of relieving, especially caring massage. Although the intensity is light, all muscle, bone and joint pains caused by hypoxia, spasm and tension can be relieved immediately through the tactile judgment of the therapist. Convenient and practical. Muscle weakness gradually leads to the gradual decline of sports ability, which is another common and troublesome problem. This can't be helped by passively waiting for a massage. You must take action on your own. Don't take a wheelchair if you can walk. Don't lie in bed if you can sit up. Just follow the therapist's instructions, don't be forced, don't be too tired, do a full-body stretching exercise every day to keep your muscles flexible and strong. You can do it yesterday. If some functions have been lost, under the principle of maintaining independence as far as possible, we can also consider using assistive devices to compensate and help. For example, if you can't walk, you can use crutches or even wheelchairs to walk. Only under what circumstances, the professional judgment of the therapist is needed to avoid using AIDS too early or excessively, hindering the self-function, or using them too late to worsen the physical condition. Whole body hydrotherapy is a rehabilitation exercise therapy in water. The deeper the water, the greater the pressure, which is very helpful for blood and lymphatic reflux. When the whole body is immersed in water, warm water can reduce abnormal muscle tension, and water provides good support and protection for the body. Water sports are safe and effective, and the buoyancy of water greatly reduces the weight. Although it is not suitable for walking on land, it can easily move and walk in water, and induce limb movement with the help of buoyancy of water. For stronger muscles, you can use the resistance of water for proper exercise training, and for serious patients, you can use some floating tools for support and protection, so that you can float freely in the water; According to the patient's situation, needs and progress, different degrees of water sports therapy are given, from simple to difficult, designed by physiotherapists. Spa must first have a good barrier-free environment, suitable water temperature and room temperature. First of all, doctors should make a correct diagnosis, and physiotherapists should give patients different underwater sports treatments according to their needs and progress. In terms of revitalizing the rehabilitation medical center, the whole body spa pool is 1 1 m long, 6.5m wide and 90cm-1/0cm deep. Stainless steel handrails are arranged around the spa pool, and a set of parallel bars are arranged on both sides of the spa pool, which can be used for standing, walking, training or sports therapy in the water. The underwater training chair is used for patients to rest and exercise in the water. An automatic lifting shifter is added along the pool edge to facilitate wheelchair users to move from the outside to the inside of the spa pool. Non-slip floors and seating, changing clothes and showering are all necessary facilities. Nursing methods Nutrition nursing 1) Olfactory stage: the foraging motivation caused by food aroma stimulating forebrain. This motivation can be enhanced by the stimulation of gastrointestinal hormones caused by the decrease of liver sugar, or weakened by depression and melancholy. Patience: Be optimistic and enterprising, and ask without hesitation. Medical staff: use all effective communication channels to communicate. Nutritional countermeasures: Eat a normal diet and avoid a diet high in tryptophan, such as corn. Nutrition goal: keep appetite. 2) Chewing period: the patient can't chew effectively, and can't start swallowing effectively because of the strong mandibular and lingual muscle reflex. Patient: Do soft massage, hot compress and oral and dental care with family members before and after meals. Medical staff: Use effective muscle relaxants and painkillers. Nutrition countermeasures: increase liquid diet and strengthen the ratio of fat and protein in diet. Nutrition goal: keep weight. 3) Swallowing stage: Patients are prone to aspiration pneumonia due to glottic muscle weakness and poor coordination. Patient: Gastrostomy should be carefully considered, and family members should be asked to clean the wound after operation. Medical staff: provide detailed information of gastrostomy for patients' reference and hand it over to relevant operation departments. Nutritional countermeasures: Full liquid diet, strengthening the ratio of fat and protein in diet, special formula to improve immunity, and total parenteral nutrition should be considered several weeks before and after operation. Nutrition goal: keep weight and prevent infection. 4) Gastrointestinal peristalsis: Patients are prone to intestinal obstruction, constipation and diarrhea due to long-term bed rest, insufficient drinking water and bacterial infection. Patient: Total parenteral nutrition should be carefully considered, and family members should clean the bathroom regularly. Medical staff: provide detailed total parenteral nutrition data for patients' reference, and avoid using central intestinal drugs such as Novartis. Nutritional countermeasures: Whole liquid food, special formula to enhance the ratio of fat and protein in diet, and intermittent total intravenous nutrition at home should be considered. Nutritional goals: keep weight, prevent infection, avoid imbalance of body fluids and electrolytes, and supplement rare elements and vitamins. Respiratory nursing patients with motor neuron disease are attacked by inspiratory muscles and expiratory muscles, so ventilation and cough will be affected. Therefore, if the patient catches a cold, it is not easy to cough up sputum, which is easy to cause lung atrophy and lead to pneumonia. Generally speaking, hospice care is only for patients with terminal cancer and painful symptoms. In fact, hospice care is just an idea and movement. At the end of any disease, as long as the curative treatment has been given as far as possible, those who still have discomfort symptoms such as pain and dyspnea and need palliative medical care and psychological and spiritual help belong to the scope of hospice care. Therefore, patients with motor neuron disease have the care needs of body, mind, spirit, family members and patients in the course of disease development, which is in line with the care spirit of hospice care. In other words, to provide a home-like environment, in addition to the companionship and care of relatives and friends, the medical professional team also includes neuromuscular doctors, rehabilitation doctors, physiotherapists, occupational therapists, speech therapists, social workers, respiratory therapists, nutritionists, practical nurse, mechatronics experts, psychological and spiritual tutors, volunteers and so on. Take care of patients together, so that their body and mind can be fully taken care of. In the development of each course, other related motor neurons are nerve cells that control our body activities, which can be divided into upper motor neurons and lower motor neurons; Generally speaking, motor neurons in the brain are called upper motor neurons, and motor neurons in the brain stem and spinal cord are called lower motor neurons. The lower motor neurons in the spinal cord control most of the body, while the lower motor neurons in the brain stem control the muscles of the head, face and throat. The upper motor neuron finely and complexly controls the lower motor neuron, which makes the movement stable and direct under various tension changes. The lower motor neuron is directly connected with the muscle and sends motion signals to the muscle. Generally, when motor neurons are eroded, the adverse effects are mainly the symptoms of muscle atrophy, which usually appear in the palms and fingertips, and gradually deteriorate into atrophy of shoulder, neck, tongue and swallowing muscles, leading to dysphagia and respiratory failure. If the upper motor neurons are also damaged, the patient will become prone to spasm, stiffness and enhanced reflexes due to high muscle tension, which is manifested as jumping when the patient walks, and sometimes the patient's knees will keep shaking. For ALS, due to the gradual death of upper and lower motor neurons, these symptoms usually appear together, including muscle weakness and atrophy, as well as muscle stiffness and spasm. At present, there are 200,000 "frozen people" in China. In fact, there may be more. This is a malignant disease. Just like its name, sick people lose any ability to act, just like the famous "gradually frozen man" Professor Hawking, frozen by snow and ice. But this process is not rapid, but a part of the body shrinks and is weak. Today is the leg, tomorrow is the arm, and the day after tomorrow is the finger, even the tiny muscles that control eye movements are no exception. What finally awaits them is respiratory failure. However, all this happened when they were conscious and awake, and they clearly stared at the whole process of their gradual death. Frostbite is a kind of motor neuron disease, and its scientific name is amyotrophic lateral sclerosis (ALS). It is also called Gerecke's disease because the famous American baseball star Ron Gehrig suffers from it. Cui Liying, director of the Department of Neurology, Peking Union Medical College Hospital, said that the incidence rate of the disease is four in 100,000, mostly after the age of 40, and there are about 200,000 patients in China. As of 20 10, among the 3000 cases of gradually frozen people registered by Beijing volunteer organization "Melting Frozen Heart" in the past six years, there are 100 cases in Zhejiang Province. At present, human beings know very little about this disease. No cause can be found, and there is no way to treat it. The average survival time of patients is only 3 years. The most famous person suffering from this disease is the Englishman Hawking. Unfortunately, this scientist can master the mystery of the origin of the universe, but he can't stop the power of life from disappearing from his body bit by bit.