progressive spinal muscular atrophy
Introduction
Introduction to progressive spinal muscular atrophy The disease is a motor neuron disease, and the degeneration is limited to the alpha motoneurons of the anterior horn of the spinal cord. It is characterized by muscle atrophy and muscle weakness, usually starting from the small muscles of the hand, spreading to the entire upper and lower limbs, the reflex disappears, and the sensory disorder does not appear. It is characterized by weak hands and weak hands, and the internal muscles of the hands are atrophy. There may be "claw-shaped hands", "hands-on" deformities, clothes buckles, small objects and writing difficulties. Later, muscle weakness affects adjacent muscle groups, involving arms and shoulders. , and then to the lower limbs, there are also from the foot disease, extended to the lower limbs, and then the upper limbs. basic knowledge The proportion of illness: 0.0035% Susceptible people: good for middle-aged men Mode of infection: non-infectious Complications: Dysphagia, pneumonia, gastrointestinal dysfunction, respiratory infection
Cause
Causes of progressive spinal muscular atrophy
Cause:
The exact cause is still unclear. Some people describe the autosomal dominant or recessive genetic features of the disease. Different cases can be treated for different reasons, such as cold, fatigue, infection, lead poisoning, trauma, as well as secondary syphilis and polio.
Prevention
Progressive spinal muscular atrophy prevention
The main preventive measures for this disease are as follows:
1. Maintain optimistic and happy emotions. Stronger long-term or repeated mental stress, anxiety, irritability, pessimism and other emotional changes can make the balance of cerebral cortex excitability and inhibition process imbalance, make muscle jump aggravate, and make muscle atrophy develop.
2, rational allocation of diet structure, muscle atrophy patients need high protein, high-energy diet supplement, provide the necessary materials for nerve cell and skeletal muscle cell reconstruction, to enhance muscle strength, increase muscle, early use of high protein, rich in vitamins, phospholipids And trace elements of food, and actively cooperate with medicated diets, such as yam, glutinous rice, lotus seeds, dried tangerine peel, ginseng, lily, etc., fast food spicy food, quit smoking, alcohol, middle and late patients, high protein, high nutrition, rich The energy-containing semi-liquid food and liquid food are mainly used, and the method of eating small meals is adopted to maintain the nutrition and water-electrolyte balance of the patient.
3, work and rest, avoid forced sexual function exercise, because forced sexual exercise will be due to skeletal muscle fatigue, not conducive to the recovery of skeletal muscle function, muscle cell regeneration and repair.
4, strict prevention of colds, gastroenteritis, muscle atrophy patients due to low autoimmune function, or there is some kind of immunodeficiency, muscle atrophy patients, once the cold, the disease worsened, prolonged course, muscle weakness, muscle weakness, especially the ball Paralyzed patients are prone to complicated pulmonary infections. If they are not treated in time, the prognosis is poor, and even the life of the patients is endangered.
5, gastroenteritis can lead to dysfunction of intestinal bacteria, especially viral gastroenteritis has different degrees of damage to the anterior horn cells of the spinal cord, so that muscle atrophy in patients with muscle atrophy, muscle strength decline, disease recurrence or aggravation, muscle Atrophic patients maintain normal digestive function and are the basis of rehabilitation.
Complication
Progressive spinal muscular atrophy complications Complications dysphagia pneumonia gastrointestinal dysfunction respiratory infection
In the early stage of the disease, the main manifestations are simple limb function limitation. There may be complications such as claw-shaped hand and squatting hand deformity. If the condition continues to develop, it may lead to respiratory muscle dysfunction and difficulty in swallowing. At the same time, there may be aspiration pneumonia. If the intestinal muscle function is limited, intestinal dysfunction may occur, resulting in digestion and absorption of macromolecular protein and fat. At this time, the enteral nutrition dosage form should be adjusted, and the factor diet should be selected. It is also possible to have respiratory infections and respiratory insufficiency. At this time, ventilator assisted ventilation and intravenous nutrition support are required.
Symptom
Symptoms of progressive spinal muscular atrophy Common symptoms Spinal muscular atrophy Muscular atrophy Muscle tone reduced muscle bundle tremor Hands weak and weak
1, the disease onset, hidden in the middle-aged male.
2, the main performance is the weak hands and hands, the internal muscles of the hand atrophy, can have "claw-shaped hands", "hands" deformity, clothing buckles, picking small objects and writing difficulties, after the muscle weakness affects adjacent muscle groups, involving The arms and shoulders are then developed to the lower limbs, and there are also cases from the foot to the lower limbs and then the upper limbs.
3, muscle atrophy is weak and symmetrical development, sometimes only involving one hand, the muscle tension is reduced, and the tendon reflex is weakened corresponding to the affected muscle.
4, sphincter no dysfunction, pathological reflex does not appear, but can be seen in frequent fasciculation tremor, fasciculation can occur indefinitely, cold, mood swings or mechanical stimulation can induce and aggravate fasciculation.
5, no spontaneous pain and paresthesia occurred in the course of the disease, tongue muscle atrophy, soft palate dyskinesia with pronunciation and swallowing symptoms rarely produced.
Examine
Progressive spinal muscular atrophy
The main methods of examination for this disease are some serological tests and neurological tests:
1, CSF can have a slight increase in protein.
2. The nerve conduction velocity measured by SEP is normal.
3. EMG shows the characteristics of motor neuron lesions.
Another scholar suggested that the disease can be used for genetic diagnosis, mainly by SSCP silver stain detection of SMA gene deletion, this method is fast and safe, the results are reliable, the diagnostic rate is high, but it is still impossible to apply to clinical.
Diagnosis
Diagnosis and differentiation of progressive spinal muscular atrophy
Diagnosis is based on clinical performance and examination.
The diseases that need to be differentially diagnosed with this disease are:
1, chest export syndrome can occur with both hands symmetric muscle atrophy, but there are many limb pain and sensory disturbances, the scope of muscle atrophy is limited, no fasciculation.
2, cervical spondylotic myelopathy and radiculopathy of cervical spondylosis may have both hands muscle atrophy, hand fine movement disorders, tendon reflexes weakened, but also often limb pain, numbness, no fasciculation.
