Amyotropic Lateral Sclerosis (ALS) is a progressive degenerative disease with adult motor neuron involvement, resulting in skeletal muscle atrophy and neurological dysfunction as the main clinical manifestation. The disease usually appears after 30 years of age, with a high incidence between 40 and 50 years of age and an incidence of 1 to 2.5 per 100,000 in the population. The incidence is higher in men than in women, about 3:2. 90-95% of cases in China are disseminated. The natural course of the disease is 1 to several years. The main cause of the disease is degeneration and necrosis of motor nerve cells in the brain and spinal cord, and degeneration of the corticospinal tract and the corticomedullary tract, which are responsible for motor conduction, thus affecting the functional limitations from cranial nerve activities to limb activities. The facial nerve and the linguopharyngeal and hypoglossal nerves of the cranial nerves are the most vulnerable; the skeletal muscles of the extremities are also susceptible to involvement. The skeletal muscles of the limbs are also susceptible to involvement, causing muscle atrophy, weakness, and increased muscle tone. Late respiratory muscle paralysis can cause respiratory distress and asphyxia. The patient’s activities are gradually restricted and eventually he or she loses the ability to work completely, so the folklore also has the name “acromegaly”. However, the disease hardly affects the sensory nerves and has little effect on intelligence, so it belongs to the category of motor neuron disease. Clinical manifestations The clinical symptoms of patients can be initiated by the limbs or by the abnormalities of the cranial nerves, and the common manifestations can be divided into the following aspects: 1. Cranial nerve symptoms: The cranial nerves are mainly involved in the facial nerve, linguopharyngeal and hypoglossal nerves. Patients often have reduced expression, weak speech, slurred spitting, swallowing difficulties, choking on food and water. Sometimes it is difficult to open the mouth, and pharyngeal muscle paralysis may lead to increased salivation and drooling. In the late stage, there is difficulty in extending the tongue, tremor and atrophy of tongue muscle, disappearance of pharyngeal reflex, and hyperactive jaw reflex, etc. 2. Symptoms of motor neuron damage in the lower extremity: manifested as muscle weakness, atrophy and muscle tremors, which can occur in any one or a group of muscles of the limb, but are more common in the upper extremity. The distal muscles, such as the interosseous muscles of the palm and the greater and lesser interosseous muscles, are usually atrophied, forming an “eagle’s claw-like hand”. Atrophy of the scapular muscles often results in shoulder droop. Atrophy of the lower extremity muscles often leads to difficulty in walking and squatting, and in advanced stages, urinary and bowel disorders may occur. Respiratory muscle involvement leads to decreased mobility, breath-holding and dyspnea, and ventilator assistance is often required in advanced stages. 3. Upper motor neuron damage symptoms: Upper motor neuron damage often leads to stiffness of limb activities, unstable walking, hyperactive physiological reflexes of various tendons, and pathological reflexes may appear. There is usually no objective sensory impairment, only some minor subjective discomfort of numbness, tingling, and chilliness appear. Auxiliary examinations General morphological examinations such as CT and MRI are difficult to detect abnormalities in the early stage. EEG is normal, and lumbar puncture cerebrospinal fluid is normal. Meaningful examination mainly relies on electromyography. EMG often shows fibrillation potentials and giant motor unit potentials, and peripheral motor nerve conduction can be slowed down, but sensory nerves are not affected. The diagnostic criteria for ALS, as proposed by the World Federation of Neurology in 1990, are as follows: (1) the necessary conditions: (1) lower motor neuron signs; (2) upper motor neuron signs; (3) progressive worsening of the disease course. 2. Confirmation of diagnosis: 3 of the 4 parts of the brainstem, upper extremities, trunk and lower extremities have upper motor nerve + lower motor nerve damage, and the diagnosis can be confirmed. There are 2 with upper motor nerve + lower motor nerve damage, and upper motor nerve damage in the head, it is likely to disease. If there is one with upper motor nerve + lower motor nerve damage, or three with upper motor nerve damage, the disease is likely. If there are 2 to 3 sites with lower motor nerve damage, the disease is suspected. Treatment and prognosis 1.Vitamins: such as vitamin E, C, A, B. 2.Gabapentin, lamotrigine, riluzole, etc. 3.Nerve growth factor. The above treatments can only relieve the symptoms and cannot hinder the progression of the disease, so there is no cure at present.