Hirayama disease is also known as distal upper limb muscular atrophy in young people. It is a benign self-limiting motor neuron disease first reported by Japanese scholar Keizo Hirayama in 1959, which is clinically similar to motor neuron disease amyotrophic lateral sclerosis and spinal progressive myasthenia with a very different prognosis. The disease occurs in adolescence and is more common in males. Clinical manifestations and diagnostic criteria: typical Hirayama disease is characterized by muscle weakness of the hand and distal forearm with insidious onset in early adolescence, with progressive atrophy of the corresponding muscle groups, mostly unilateral damage, some may also show asymmetric bilateral damage. Most patients have “cold palsy”, i.e., the weakness is significantly aggravated by exposure to cold; bundle tremor is not present in the quiet state, but often occurs during finger extension; the tendon reflexes of the affected limb are normal or occasionally low, and there is usually no pain, numbness or other sensory impairment, nor are there cone fasciculation signs or sphincter dysfunction. The disease progresses slowly for several years after onset, and is easily confused with motor neuron disease, such as amyotrophic lateral sclerosis or progressive spinal muscular atrophy, but the majority of patients have spontaneous discontinuation of the disease within the next 5 years, and the prognosis is significantly different from that of motor neuron disease.
Overview of muscle atrophy
Muscle atrophy is defined as transverse muscle dystrophy, where the muscle volume is smaller than normal and the muscle fibers become thinner or even disappear. Neuromuscular disease hypertrophy. In addition to the pathological changes in the muscle tissue itself, muscle dystrophy is more closely related to the nervous system. Spinal cord diseases often lead to muscle dystrophy and muscle atrophy.
Classification of muscle atrophy
According to the pathogenesis of classification
1.Muscle atrophy caused by systemic nutritional disorders, wasting, endocrine abnormalities and other causes of muscle degeneration and muscle structural abnormalities.
2, genetic, poisoning, metabolic abnormalities, infection, metabolic reactions and other causes of muscle atrophy, such classification has little clinical significance, because the etiology is difficult to clarify.
Classification of muscle atrophy according to its distribution
1, generalized diffuse muscle atrophy.
2, head and facial muscle atrophy.
3, head and upper limbs or proximal muscle atrophy of upper and lower limbs.
4, distal muscle atrophy of upper and lower extremities.
5.Limited muscle atrophy.
Classification according to the primary lesion causing muscle atrophy
1, neurogenic muscle atrophy.
2, myogenic muscle atrophy.
3, disuse muscle atrophy.
Neurogenic muscle atrophy mainly refers to the lesions of lower motor neurons such as anterior horn cells and peripheral nerves of the spinal cord, which belong to primary neurogenic muscle atrophy. The three are interrelated with each other, and although upper motor neuron lesions also appear as muscle atrophy, some people classify them as secondary, late stage disuse atrophy. Myogenic muscle atrophy is caused by a lesion of the muscle itself. Disuse muscle atrophy can also be postulated in systemic wasting diseases.
Health care measures for patients with muscular atrophy
Patients with myasthenia gravis are bedridden for a long time due to muscle atrophy and muscle weakness, and are prone to complications such as pneumonia and decubitus ulcers, which, together with the symptoms of medullary paralysis in most patients, pose a great threat to their lives. Myasthenia gravis patients, in addition to the doctor’s treatment, self-treatment is very important.
1, maintain an optimistic and happy mood. Strong long-term or repeated mental tension, anxiety, irritability, pessimism and other emotional changes can make the balance of cortical excitation and inhibition process is out of balance, so that the muscle jump aggravated, so that the development of myasthenia gravis.
2, reasonable deployment of dietary structure. Myasthenia patients need high protein and high energy dietary supplements to provide substances necessary for the reconstruction of nerve cells and skeletal muscle cells in order to enhance muscle strength and muscle growth, early with high protein, vitamin-rich, phospholipid and trace element foods, and actively with medicinal food, such as yams, coix seeds, lotus hearts, Chen Pi, prunus seeds, lilies, etc., prohibit spicy food, quit smoking and alcohol.
For patients in the middle and late stages, high protein, high nutrition, energy-rich semi-liquid and liquid food is the mainstay, and a small number of meals are used to maintain the patient’s nutrition and water-electrolyte balance.
3. Combine labor and rest. Avoid forced sexual exercise, because forced sexual exercise will be due to skeletal muscle fatigue, and is not conducive to the recovery of skeletal muscle function, muscle cell regeneration and repair.
4, strict prevention of colds, gastroenteritis. Myasthenia gravis patients due to their own immune function is low, or there is some kind of immune deficiency, myasthenia gravis patients once the cold, the disease aggravated, the disease prolonged, myasthenia gravis, muscle jump aggravated, especially ball palsy patients prone to complications of pulmonary infection, if not timely prevention and control, the prognosis is poor, and even endanger the lives of patients.
5, gastroenteritis can lead to intestinal strain dysfunction. Especially viral gastroenteritis has different degrees of damage to the anterior horn cells of the spinal cord, thus making myasthenia gravis patients with increased muscle jump, decreased muscle strength, and recurrent or aggravated disease. Maintaining normal digestive function in patients with myasthenia gravis is the basis for recovery.
Chinese medicine treatment for muscular atrophy
In modern medicine, polyneuritis, spinal cord cavitation, myasthenia, myasthenia gravis, lateral sclerosis, motor neuron disease, periodic paralysis, myotonic dystrophy, hysterical paralysis and the sequelae of central nervous system infection manifesting as flaccid paralysis are all within the scope of “impotence evidence”, “impotence evidence Impotence” is a condition in which the tendons and veins of the limbs become weak and ineffective.
Impotent evidence refers to a type of disorder in which the tendons and bones are impotent and soft, the muscles are thin, the skin is numb, and the hands and feet are not used. Clinically, impotence and weakness of both feet and inability to move freely are more common, hence the name “impotence”. Muscle degeneration and muscle structure abnormalities caused by motor neuron disease, systemic nutritional disorders, disuse, endocrine abnormalities, genetics, poisoning, metabolic abnormalities, infections, metabolic reactions and other causes can cause muscle weakness, muscle atrophy, etc.
Regulation of muscle atrophy
Impotence is a condition in which the tendons and veins of the limbs are weak and ineffective. The key to the care of impotence is to regulate the qi and blood of the limbs and restore the functional activities of the limbs. The functional training of limb activity can be done by both active and passive practice, and the content can be different from traditional sports training and life work training. If the limbs are thin and withered, weak in movement and unable to walk, passive training can be adopted in the recumbent position to change the posture at any time to prevent the occurrence of “deformity”.
After that, active practice training is adopted, such as sitting, standing and walking practice. According to the condition, you can choose the corresponding guide, massage, qigong and traditional physical exercise methods such as Five Animal Play and Eight Duan Jin. Life work methods are more practical and easy to learn. If the upper limbs are impaired, writing, throwing, catching, playing the piano, playing percussion, knitting, plucking the abacus, etc. If the lower limbs are limited, tricycles, sewing and other work training methods are used.
Impotence is a condition in which the tendons and veins of the limbs are sluggish, weak and useless. The key to care for impotence is to regulate the Qi and blood of the limbs and restore the functional activities of the limbs. The functional training of limb activity can be done by active and passive training, and the content can be different from traditional sports training and life work training. If the limbs are thin and withered, weak in movement and unable to walk, passive training can be adopted in the recumbent position to change the posture at any time to prevent the occurrence of “deformity”. After that, active practice training is adopted, such as sitting, standing and walking practice. According to the condition, the corresponding guide, massage, qigong and traditional physical exercise methods such as Five Animal Play and Eight Duan Jin can be used. Life work methods are more practical and easy to learn. If the upper limb activity is impaired, use writing, throwing, catching, playing the piano, knitting, dialing the abacus, etc. If the lower limb activity is limited, use tricycles, sewing and other work training methods. Self-treatment is very important.
1.The development of Hirayama is closely related to the flexion of the neck, try to reduce the frequency of lowering the head, it is better to wear a neck brace to effectively prevent the further development of the disease. (Focused attention)
2.Maintain an optimistic and happy mood. Stronger long-term or repeated mental tension, anxiety, irritability, pessimism and other emotional changes can make the balance of cortical excitation and inhibition process is out of balance, so that the development of the disease.
3, reasonable deployment of dietary structure. High protein, high-energy dietary supplements to provide substances necessary for the reconstruction of nerve cells and skeletal muscle cells, in order to enhance muscle strength and muscle growth, using high-protein, vitamin-rich, phospholipid and trace element foods, actively with the medicinal diet, such as yam, coix seeds, lotus hearts, Chen Pi, prunus seeds, lily, etc., less spicy food, quit smoking, alcohol.
4, the combination of work and rest. Avoid forced sexual exercise, because forced sexual exercise will be due to skeletal muscle fatigue, and is not conducive to the recovery of skeletal muscle function, muscle cell regeneration and repair.
5, prevention of colds, gastroenteritis. Due to low autoimmune function, once the cold, the condition plus will be heavy, the course of the disease is prolonged.
Combined with medication: vitamin B1, vitamin E, vitamin B12 (or Micronutrients).
Clinical manifestations
The typical presentation of Hirayama disease is an insidious onset of muscle weakness in the hand and distal forearm in early adolescence, with progressive atrophy of the corresponding muscle groups, mostly unilateral, but some may show asymmetric bilateral damage. Most patients have “cold palsy”, i.e., the weakness is significantly aggravated by exposure to cold; bundle tremor is not present in the quiet state, but often occurs during finger extension; the tendon reflexes of the affected limb are normal or occasionally low, and there is usually no pain, numbness or other sensory impairment, nor are there cone fasciculation signs or sphincter dysfunction. The disease progresses slowly for several years after the onset of the disease and is easily confused with motor neuron disease such as amyotrophic lateral sclerosis or progressive spinal muscular atrophy, but the majority of patients can spontaneously discontinue the disease within the next 5 years and the prognosis is significantly different from that of motor neuron disease.
Pathogenesis
The pathogenesis of Hirayama disease is not very clear, and the generally accepted pathogenesis is.
1, kinetic factors: Hiarayama et al. believe that the pathogenesis of the disease is the repeated flexion of the neck or long-term maintenance of the flexion of the neck posture has led to the anterior displacement of the dura mater from the posterior push on the cervical spinal cord, resulting in impaired circulation, chronic ischemic necrosis of motor cells in the anterior horn of the lower cervical medulla. The effectiveness of clinical trials of cervical collars in the treatment of this disease supports the possibility of this mechanism.
2, growth and developmental factors: Shinibo et al. suggested that Hirayama disease may be related to a developmental imbalance between the spinal cord and the dura mater. The basis is
(1) The disease occurs mostly in young men in a period of rapid growth in arm length or height, suggesting that the age of onset of Hirayama disease is closely related to the period of rapid growth in height.
(2) muscle atrophy appears 2 to 4 years after onset and stops progressing at the end of the rapid growth period.
(3) The relative shortening of the anterior cervical medullary roots determines the self-limiting course of the disease. Thus, it is believed that the asymmetric atrophy of the anterior horn of the spinal cord, the displacement of the anterior dura in the low cervical segment, and the changes such as the dilatation of the epidural plexus in Hirayama disease are caused by the imbalance in growth and development between the spinal cord and the dura and the relative shortening of the anterior root of the cervical medulla due to the rapid growth.
3, inelastic, restrictive dural compression: Konno et al. suggest that Hirayama disease is the result of abnormal dural traction restriction that not only affects in the upright position, but also aggravates spinal cord injury during cervical flexion. This view is confirmed by the apparent immediate and long-term results of cervical spinal laminectomy.
4, motor neuron disease theory: some scholars currently believe that Hirayama disease for motor neuron disease, is between amyotrophic lateral sclerosis and progressive spinal muscular atrophy in a special type, this view is now increasingly questioned.
5. Ethnogenetic factors: The incidence of this disease is gradually increasing in Japan, so the influence of ethnic factors cannot be excluded. Some scholars examined the genes of two Hirayama brothers with a family history of amyotrophic lateral sclerosis (ALS). Attempts have been made to show that the occurrence of familial Hirayama disease is related to gene mutations, especially superoxide dismutase (SOD) gene variants that lead to aspartate substitution for alanine (D9OA), but there are no conclusive findings.
6, immunological mechanism: Some scholars found that patients with Hirayama disease have allergies, and they all have a family history of allergy or abnormal allergic reactions, suggesting that abnormal immune mechanisms may have some influence in the pathogenesis of Hirayama disease, and that the theory of allergic reactions to explain some phenomena of Hirayama disease may be reasonable, because the number of cases is small, there is no definite conclusion.