This is an automatically translated article.
Amyotrophic lateral sclerosis, also known as ALS, is a degenerative motor neuron disease caused by damage to peripheral motor neurons combined with damage to the pyramidal system that can cause loss of sensation. Amyotrophic lateral sclerosis eventually leads to death due to paralysis of the respiratory muscles, respiratory complications (aspiration pneumonia...) or other complications due to immobility.
1. Causes of disease
Currently, the cause of the disease is still unknown, however, many experts believe that genetic diseases or infections, trauma, metabolic disorders ... can also cause disease.
When you have lateral sclerosis, atrophy is the degeneration of motor neurons located in the anterior horn of the spinal cord, especially in the cervical and lumbar spinal cord aneurysms, and the cranial nerve nuclei in the brain stem (nuclei of the cord). XII, IX, X and motor nuclei of the V cord), damage to the conduction pathways in the lateral column of the spinal cord, especially the pyramidal bundle, may involve Betz cells located in the fifth and fifth layers. III of the cerebral cortex.
2. Manifestations of the disease
When amyotrophic lateral sclerosis begins:
Starts slowly with weakness and atrophy of the hands and forearms being the most common form, accounting for about 50% of all amyotrophic lateral sclerosis, however begins Initially with mild, incomplete symptoms, then gradually other symptoms appeared with increasing severity. At first, weakness and atrophy are present in one hand, then after a few weeks or possibly months it is transferred to the other hand, sometimes with simultaneous onset in both hands, but the severity is uneven.
Bệnh xơ cột bên teo cơ khởi phát khi bàn tay, cẳng tay bị yếu và teo cơ
When the disease is advanced, all of the following pathological symptoms appear:
Paralysis, muscle atrophy: appearing in the late stages, most evident in the hands, muscle atrophy associated with decreased muscle strength, Muscular atrophy of Aranduchenne syndrome, concave hand, first phalanx of the fingers is usually in the extensor position. Vibration of muscle fibers: Patients themselves feel spontaneous tremors of muscle fibers or tremors of muscle bundles, which are very uncomfortable and cannot be controlled by themselves. Pyramid syndrome: Due to damage to the pyramidal system, there is a marked increase in tendon reflexes and a marked pathological reflex of the pyramidal bundle. Medullary syndrome: Most of the cases of medulloblastoma appear in the late stages of the disease due to damage to the IX and X cords. In addition, the patient also has other pathological symptoms such as: mental disorders. such as emotional changes, easy crying, laughing without reason, severe intellectual impairment.
3. Rehabilitation and treatment
Principles of rehabilitation and treatment: to improve the quality of life of patients with symptomatic treatment, rehabilitation exercises, patient care
3.1. Specific treatment Riluzole (the drug officially approved by the US FDA for use): 100mg dose divided into 2 times a day, should be used early when the diagnosis is confirmed, treatment for 18 months, prolonging survival time for a few more months, especially for patients with symptoms of oropharyngeal paralysis (choking, choking), but the effect is very limited
When using this drug, you must monitor liver function regularly because the drug can cause liver damage.
Using stem cells to treat diseases is still in the research stage.
Sử dụng thuốc Riluzole có nguy cơ gây tổn thương các chức năng của gan
A guide for patients and caregivers on stretching and range-of-motion exercises (ROM) ) daily. Educate patients and family members on safe, effective and energy-efficient ways to move around. Strength and endurance exercises, should be performed below maximal muscle strength without weakening the muscle, and should be indicated only in slow-progressing patients. Exercise with moderate intensity and specificity for each patient The technician needs to anticipate the patient's needs in advance and use assistive devices (AFO brace, KAFO brace...) and wheelchair at the right time. . Amyotrophic lateral sclerosis activities:
Educate patients and caregivers on techniques that help conserve energy. Adaptive assistive devices should be indicated early and appropriate to the evolving needs of the patient. Speech therapy used for amyotrophic lateral sclerosis :
Speech therapists teach patients with medulla oblongata how to slow speech and exaggerate pronunciation to help caregivers understand. For patients with respiratory failure, technicians can teach each sentence so that the patient is less tired. Exercises to strengthen the submandibular tongue and diaphragm exercises to help improve pronunciation and vocalization. If patients and caregivers already have their own communication patterns (eg, gestures), they should be encouraged to continue using them effectively. Specify communication aids such as communication boards, scripts, or assistive technology. In patients with dysphagia due to medulloblastoma, speech-language pathologists perform oropharyngeal evaluation to evaluate clinical signs of dysphagia. Oropharyngeal endoscopy is necessary to identify specific problems in the different phases of swallowing. The technician teaches you how to swallow, changing the consistency, taste, and temperature of food to improve swallowing and prevent aspiration.
Kỹ thuật viên tiến hành nội soi hầu họng nếu bệnh nhân có dấu hiệu của khó nuốt
Treatment of hypersalivation (Sialorrhea): due to natural limitation of swallowing saliva, use Amitriptyline at a dose of 25-50mg, daily 2- 3 times or central nervous system stimulants (such as Methylphenidate) Treatment of muscle spasticity: use Baclofen oral dose up to 80mg/day, can use Dantrolene, Tizanidin, Benzodiazepine, Clonidine. Treatment of myoclonic tremor: In patients with severe symptoms of tremor, benzodiazepines may be effective. Treatment of pain for amyotrophic lateral sclerosis:
Pain may be caused by muscle spasms, contractures, or altered biomechanical mechanisms of the joint. First-line treatment is acetaminophen and NSAIDs. In some cases of pain of nerve origin: antiepileptic and tricyclic antidepressants are also indicated for this form of pain Respiratory care: Majority of patient deaths are related to complications on the respiratory system due to severe respiratory or pharyngeal muscle weakness.
Prolonged mechanical ventilation is necessary to open the trachea and aspirate sputum. Supportive respiration (eg, noninvasive intermittent positive pressure ventilation, intermittent intra-abdominal pressure ventilation...) Oxygen Breathing Vital capacity should be measured on examination in case of weakness of respiratory muscles. Depression, anxiety and insomnia due to amyotrophic lateral sclerosis:
Early recognition of psychological disorders is important, allowing appropriate interventions to assist patients and carers. Selective Serotonin Reuptake Inhibitors (SSRIs) are the most commonly used antidepressants, with tricyclic antidepressants used as second choice. Psychotherapy: Can be alone or in combination with drugs
Hỗ trợ điều trị chứng trầm cảm và mất ngủ do bệnh xơ cột bên teo cơ
In addition, nutrition for people with amyotrophic lateral sclerosis is also particularly important:
- In case of weakness of the pharyngeal muscles, the patient eats poorly, leading to weight loss and malnutrition, so it is necessary to is encouraged to use calorie-rich foods, soft foods,
- If it is solid foods, it should be made loose by adding more water before swallowing. When swallowing disorders, it is necessary to put a nasal tube - stomach to feed, Consider percutaneous endoscopic gastroscopy when the patient's swallowing disorder persists.
When treating amyotrophic lateral sclerosis, it is necessary to have a follow-up visit every 3 months, the follow-up schedule may be shorter when the disease progresses faster, during the follow-up visit to assess the respiratory status, other symptoms such as excessive salivation, muscle spasticity, affective disorder, nutritional status, then consider consulting open percutaneous gastroscopy for feeding.
Vinmec International General Hospital is a high-quality medical facility in Vietnam with a team of highly qualified medical professionals, well-trained, domestic and foreign, and experienced.
A system of modern and advanced medical equipment, possessing many of the best machines in the world, helping to detect many difficult and dangerous diseases in a short time, supporting the diagnosis and treatment of doctors the most effective. The hospital space is designed according to 5-star hotel standards, giving patients comfort, friendliness and peace of mind.
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Reference source: msdmanuals.com
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