Distal upper extremity muscular atrophy in youth
Introduction
Introduction to the distal muscle atrophy of the upper limbs Juvenile myotrophyofdistalupperextremity, first described by Japanese scholar Hirayama Hiroshi (1959), is also known as Pingshan disease. The disease is a benign and self-limiting motor neuron disease, clinically similar to motor neuron disease amyotrophic lateral sclerosis, spinal progressive muscle atrophy, but the prognosis is completely different, the age of onset is young, the affected part is mostly The unilateral upper extremity distal hand muscle, the EMG is neurogenic damage, the course of the disease is benign, can stop by itself. basic knowledge The proportion of illness: the incidence rate is about 0.004% - 0.009% Susceptible people: youth Mode of infection: non-infectious Complications: distal maxillary muscle atrophy in young adults
Cause
The cause of distal maxillary muscle atrophy in young adults
The pathogenesis of distal maxillary muscle atrophy in young adults is unclear and may be related to kinetics, growth and development, dura mater, motor neuron disease, ethnic inheritance, immunology and other factors. Neurogenic muscle atrophy mainly refers to the lesions of the lower motor neurons such as the anterior horn cells and peripheral nerves of the spinal cord, and belongs to the primary neurogenic muscle atrophy. The three were related to each other, and the upper motor neuron lesions also showed muscle atrophy. Some people listed it as secondary, and the late stage was disuse atrophy. Muscle-derived muscle atrophy is caused by the muscle itself. Disuse muscle atrophy can still be mailed to systemic wasting diseases.
Pathogenesis (20%):
(1) Muscle atrophy caused by diseases such as systemic dystrophies, disuse, endocrine abnormalities, muscle degeneration, and abnormal muscle structure.
(2) Muscle atrophy caused by genetics, poisoning, metabolic abnormalities, infections, allergies, etc., the clinical significance of this classification is not large, because the cause is difficult to be clear.
Muscle atrophy distribution (20%):
(1) diffuse muscle atrophy throughout the body.
(2) Head and face muscle atrophy.
(3) Atrophy of the head and upper limbs or proximal muscles of the upper and lower limbs.
(4) Muscle atrophy at the distal extremities.
(5) Limited muscle atrophy.
Primary lesion of muscle atrophy (20%):
(1) Neurogenic muscle atrophy.
(2) Myogenic muscle atrophy.
(3) Disuse of muscle atrophy.
Prevention
Prevention of distal muscle atrophy in the upper limbs 1. Pay attention to proper rest, do not master the combination of movement and rest, rest well, is conducive to the recovery of the body, exercise can enhance physical strength and enhance disease resistance. The combination of the two can better recover. 2. Continue taking the medicine and take care of it. 3. It is very important to maintain a good attitude, to maintain a good mood, to have an optimistic, open-minded spirit, and to be confident in the fight against disease. Don't be afraid, only in this way can you mobilize your subjective initiative and improve your body's immune function. 4. Appropriate nutrient supply, under today's living conditions, it is not appropriate to emphasize too much high sugar, high protein, high vitamin and low fat diet. However, the nutrition should be balanced, and the vegetarian diet should be accompanied by vegetables, fruits, meat, egg milk, etc. The intake is determined by the fatness of the person.
Complication
Young upper limb distal muscle atrophy complications Complications of the upper extremity muscle atrophy
Upper limb muscle atrophy, muscle weakness.
Symptom
Symptoms of distal muscle atrophy in the upper limbs of the young common symptoms Symptoms of the sensory disorder muscle atrophy, sphincter dysfunction, inability to sclerosis
The typical manifestation is early insidious onset of puberty, and more common in men, no obvious reason, the distal muscles of the upper limbs are weak, can affect the ulnar muscles of the hands and forearms, and the corresponding muscle atrophy gradually occurs as the lesion progresses, mostly unilateral Damage, part can also be expressed as asymmetrical bilateral damage, generally within 2 to 3 years after the onset, the condition stops and can gradually ease.
Most patients have "cold paralysis", that is, the symptoms of weakness in the cold environment are obviously aggravated; the tremor is not seen in a quiet state, but often occurs when the fingers are stretched; the affected limbs are normally or even low, usually without pain, numbness Such as sensory impairment, no pyramidal tract, sphincter dysfunction.
The disease progressed slowly within a few years after onset, and the disease is easily confused with motor neuron diseases such as amyotrophic lateral sclerosis or spinal progressive muscle atrophy, but the majority of patients can naturally stop the disease within the next 5 years, prognosis and exercise. Neuronal diseases are significantly different. Although the affected muscles are widely distributed and bilaterally symmetrical, they should be followed up for at least 1 to 3 years. It is confirmed that the disease is stable and no longer progresses.
The course of the disease is benign, no sensory disturbance, and no abnormality of sputum reflex, but the affected local muscle may have fine muscle bundle tremor, and the electromyogram shows neurogenic damage.
Xiang Jing et al (1985) found 14 cases of constrictive atrophy of the lower neck to the upper thoracic spinal cord, and when the patient's head flexed, the dural sac was significantly pulled, causing the cervical spinal cord to overstretch and be compressed, resulting in blood circulation disorder. The disease is "cervical cervical disease".
Domestic Li Zuohan et al (1994) reported 22 cases. According to electromyography, 27% of the affected muscles had positive fibrillation potential, 64% had multiphase waves, 86% had an average potential time extension, and 91% had an average potential. Increased, 64% of the simple phase of the recruitment form and some patients with slower motor conduction velocity, and the sensory conduction velocity is normal, it is considered that the spinal cord anterior horn cells, especially the lower cervical segment, are most likely to be damaged.
Examine
Examination of distal muscle atrophy in the upper limbs
Cerebrospinal fluid examination was normal, and no abnormal findings were found in blood routine and immunological examination.
Electromyography is neurogenic damage.
Diagnosis
Diagnosis and diagnosis of distal maxillary muscle atrophy in young adults
The diagnosis of this disease is mainly based on the age of onset. The affected area is mostly the distal hand muscle of the unilateral upper extremity. The EMG is neurogenic and the course can be stopped spontaneously.
Initial identification should be differentiated from spinal muscular atrophy, and attention should be paid to the differentiation of various neurological damages caused by cervical diseases.
