Introduction
Amyotrophic lateral sclerosisIt involves the upper motor neurons (brain, brainstem, spinal cord), and also affects a chronic progressive degeneration of the lower motor neurons (cranial nerve nucleus, spinal cord anterior horn cells) and their dominating trunk, limbs and head and face muscles. disease. Clinically, it is often characterized by mixed upper and lower motor neurons with impaired mixed sputum.
Cause
Because the cause is not clear, the etiology is much closer. Recent studies have supported the immunological mechanisms of onset, and there are a variety of immunological theories. Other causes include poisoning, trace elements, amino acid metabolism disorders, and viral infections. Some cases have genetic characteristics. The incidence rate in Guam is 50 to 100 times higher than in other parts of the world, but there is no evidence of environmental toxicants and abnormal diet.
symptom
First, medical history and symptoms:
1. There are many middle-aged and old people over 40 years old, and the ratio of male to female is about 3:2. It starts slowly and develops progressively.
2. Peripheral paralysis of the upper extremities, central paralysis of the lower extremities, and the symptoms of mixed damage of the upper and lower motor neurons are characterized by coexistence.
3. Ball paralysis symptoms, the latter group of cranial nerve damage, there are unclear articulation, difficulty swallowing, drinking water cough and so on.
4. There are no sensory disturbances.
Second, physical examination found:
Cranial nerve: In addition to ball paralysis, there may be atrophy of the tongue muscle, fibrillation of the tongue muscles, strong crying, strong emotions, and so on. Most of the upper extremities were distally atrophied, with large and small muscles and interosseous muscles, accompanied by fasciculation and normal feeling. Both lower extremities showed spastic paralysis, increased muscle tone, hyperreflexia, and bilateral pathological reflexes. Breathing difficulties occur when the respiratory muscles are involved.
Third, auxiliary inspection:
1. Lumbar puncture cerebrospinal fluid examination: pressure and composition are normal.
2. Serum phosphocreatine kinase can be increased, and acetylcholinesterase is increased.
3. EMG: visible fibrillation potential, huge potential, motor nerve conduction velocity is normal.
4. MRI: visible spinal cord atrophy and degeneration in the corresponding parts of clinically damaged muscles.
diagnosis
Sometimes it needs to be differentiated from cervical spondylosis, high neck tumor, spinal arachnoiditis.
complication
The symptoms of pyramidal tract may be coexisting with the signs of motor neuron, such as muscle atrophy and fasciculation, and the quadriplegia is hyperreflexive and has pathological reflex. Sometimes the upper extremity tendon reflexes disappear or disappear, while the lower extremities reflexes and appear paralyzed. Sphincter and sexual dysfunction are uncommon and there are no objective sensory disturbances.
treatment
There is no effective therapy, mainly symptomatic.
First, those who have difficulty breathing, oxygen, and assist breathing if necessary.
Second, dysphagia, nasal feeding or high nutrition, maintain nutrition and water and electrolyte balance.
Third, neurotrophic drugs: citicoline 250-500mg, myogenic cumshot 4ml, adenosine triphosphate 20-40mg, basic fibroblast growth factor (bFGF) 1600u, intramuscular injection 1-2 times / d, Mei Luoning (Disodium cytidine triphosphate) 20 mg intramuscularly, once a day.
Fourth, Antan 2mg 3 times / d or wonderful sodium 50-100mg / d oral can reduce or improve muscle spasm caused by upper motor neuron damage, increased muscle tone.
Fifth, the prevention and treatment of complications, to prevent joint stiffness and contracture, adhere to appropriate physical exercise and physical therapy. Prevent lung infections.
prevention
The disease is a congenital disease, early diagnosis and early treatment is the key to the prevention and treatment of this disease.
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