ALS Prevention And Treatment Guidelines

ALS isa chronic progressive degenerative disease that affects the upper motor neurons(brain, brainstem, and spinal cord) as well as the lower motor neurons (cranialnucleus, anterior horn cells of the spinal cord) and the muscles of the trunk,limbs, and head and face they innervate. It is often clinically manifested asmixed paralysis of upper and lower motor neurons combined with damage. Thedisease affects the muscles of the throat and causes patients to have difficultyspeaking and swallowing; Respiratory muscles can also be involved, resulting indyspnea and death. The onset is insidious, the progress is slow, and it is morecommon after the age of 40. The etiology of the disease is unknown, and deathusually occurs within a few years of onset. There is no effective ALSprevention and treatment measures in modern western medicine to prevent theprogress of this disease. At present, the early diagnosis of motor neurondisease is still difficult, and misdiagnosis is common. So the ALS preventionand treatment is a difficult problem at this stage.

AlthoughALS is still an incurable disease, there are many ways to improve the qualityof life of patients, which should be diagnosed early, treated early, andprolong survival as long as possible. In addition to the use of drugs to delaythe development of the disease, the treatment also includes comprehensivetreatments such as nutritional management, respiratory support andpsychotherapy.

1. Drugs that delay the development of the disease

①Riluzole,whose mechanism of action includes stabilizing the inactive state ofvoltage-gated sodium channels, inhibiting presynaptic glutamate release, andactivating postsynaptic glutamate receptors. body to promote the uptake ofglutamate, etc. A clinical study in France in 1994 reported for the first timethat the drug could be used for ALS prevention and treatment. In 1996, the U.S.Food and Drug Administration approved rilutek for the ALS prevention andtreatment. It is currently the only drug that has been proven by a number ofclinical studies to delay the progression of the disease to a certain extent.The dosage is 50mg, orally twice a day. Common adverse reactions are fatigueand nausea. Individual patients may have elevated alanine aminotransferase, andliver function should be monitored. It is not recommended to continue taking itwhen patients with advanced disease have already used invasive ventilator toassist breathing.

②Otherdrugs: In animal experiments, although a number of drugs have shown certainefficacy in the prevention and treatment of ALS animal models, such ascreatine, high-dose vitamin E, coenzyme Q10, lithium carbonate, ciliaryneurotrophy Factors, insulin-like growth factor, lamotrigine, etc., but theyhave not been proved effective in clinical studies on ALS patients.

2. Nutrition management

①Wheneating normally, a balanced diet should be adopted. When swallowing isdifficult, a high-protein and high-calorie diet should be adopted to ensurenutritional intake.

②Forpatients with difficulty in chewing and swallowing, the diet should be changed,and soft food, semi-liquid food should be eaten, and small and frequent mealsshould be taken. For those with limb or neck weakness, the eating position andutensils can be adjusted.

③Percutaneousendoscopic gastrostomy should be performed as soon as possible when the patienthas obvious difficulty swallowing, weight loss, dehydration, or the risk ofchoking and aspiration, which can ensure nutritional intake, stabilize body weight,and prolong survival. It is recommended that PEG should be performed as soon aspossible before the forced vital capacity drops to 50% of the predicted value,otherwise it is necessary to assess the risk of anesthesia and perform it withventilator support. For those who refuse or cannot perform PEG, nasogastrictube feeding can be used.

3. Respiratory support

①It is recommended to regularly check pulmonary function.

②Payattention to the early manifestations of respiratory muscle weakness inpatients, and use Bi-level positive airway pressure as soon as possible.Indications for initiation of non-invasive ventilation include: orthopnea, orsniff nasal pressure <40cmH20, or maximal inspiratory pressure <60cmH2O,or nocturnal oxygen saturation Decreased, or forced vital capacity <70%.

4. Comprehensive treatment

Inpatients with ALS course at different stages, the problems facing different,such as depression, anxiety, insomnia, salivate, dysarthria, communicationdifficulties, limb spasm, pain, etc., should be according to the specificsituation of the patients, to give specific guidance and treatment, select theappropriate drugs and auxiliary facilities, improve the quality of life,strengthen the nursing, prevention of various complications.

Even thoughmodern medicine has not yet developed effective ALS prevention and treatmentmeasures, patients should not give up their confidence and actively cooperatewith doctors for treatment.