amyotrophic lateral sclerosis
Introduction
Introduction to amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS) is the most common form of adult motor neuron disease, and ALS is predominantly progressive up and down motor neuron damage. The disease is characterized by the simultaneous involvement of the anterior spinal cord and the pyramidal tract, extensive muscle atrophy, fasciculation, and pyramidal tract signs. basic knowledge The proportion of illness: 0.001% Susceptible people: more than 40 years old, more men than women Mode of infection: non-infectious Complications: progressive bulbar palsy respiratory infection
Cause
Causes of amyotrophic lateral sclerosis
The cause of the disease includes the following different situations:
Genetic factors (10%):
5%-10% of patients have hereditary, called familial amyotrophic lateral sclerosis, adult type is autosomal dominant inheritance, young type is autosomal dominant or recessive, and clinically difficult to distinguish from sporadic cases Currently, genetic studies are used to identify autosomal dominant genotype-free copper/zinc superoxide dismutase gene mutations. The mutated gene is located on chromosome 21 long arm (21q22.1-22.2), autosomal recessive genotype Located in 2q33-q35, but these genetic mutations accounted for only 20% of FALS, other ALS genes have yet to be determined.
Poisoning factor (15%):
Excitatory toxic neurotransmitters such as glutamate may be involved in neuronal death in the pathogenesis of ALS, possibly due to a decrease in glutamate uptake by glutamate transporters, which is reported by the star, and studies have found that some patients have transport functions. Loss is caused by abnormal connection of transcripts of transporter mRNA in the motor cortex. For this excitotoxicity, SOD1 enzyme is one of the cell defense systems, which can detoxify free radical superoxide anion. Familial cases may cause valleys due to SOD1 mutation. Manganese, copper, silicon and other elements, as well as neurotrophic factor reduction, may be related to pathogenic effects.
Immunity factor (10%):
Although MND patients have detected a variety of antibodies and immune complexes, such as anti-thyroid antibodies, GM1 antibodies and L-type calcium channel protein antibodies, there is no evidence that these antibodies can selectively target motor neurons. It is currently believed that MND does not belong to the nervous system autoimmune disease.
Virus infection (14%):
Because both MND and acute polio invade the anterior horn motoneurons, and a small number of polio patients later develop MND, it is speculated that MND is associated with polio or poliovirus-like chronic infection, but ALS patients with CSF, serum No virus or related antigens and antibodies were found in the nerve tissues.
Prevention
Amyotrophic lateral sclerosis prevention
There is no effective preventive measure for this disease. The focus is on the care of patients. Systematic overall care is an important factor to ensure the success of treatment. During the whole nursing process, nurses should fully understand the characteristics and treatment methods of the disease, often with patients. Family members communicate and fully mobilize the initiative and enthusiasm of patients, so that patients can maintain a positive attitude towards treatment. In addition to careful evaluation of the patient's main statement and symptoms and signs, it is necessary to care for possible complications. Intervention and predictive care, to avoid various causes of disease development, strengthen the observation of the disease, provide dietary guidance for patients, do a good job in rehabilitation, maintain a good relationship between nurses and patients, and promote effective improvement of symptoms.
Complication
Complications of amyotrophic lateral sclerosis Complications progressive medullary paralysis respiratory infection
The disease mainly occurs in the lower extremity spasm, scissors gait, increased muscle tone, sputum radiation and Babinski sign, a few cases from the lower limbs, supervision and extension of the upper limbs, late may also have medullary paralysis, even the brain Severe function of dry function, extraocular muscles are not affected, as the disease progresses, the patient may also have respiratory muscle paralysis or concurrent respiratory infection and eventually die; the disease has a short survival period of several months, the elderly are more than 10 years, the average 3- 5 years.
Symptom
Symptoms of amyotrophic lateral sclerosis Common symptoms Lower extremity central sacral upper extremity Peripheral sacral dysfunction palsy Muscle atrophy Reflex hyperthyroidism Muscle atrophy Cough and dysphagia Difficulty
Regarding the symptoms of this disease, there are the following main points:
More than 1 after the age of 40, more men than women; the first symptoms are often finger movements and weakness, followed by large, small fish and sacral muscles and other small muscle atrophy, gradually forward arm, upper arm and shoulder strap Muscle development, large muscle twitching in atrophic muscles; extensor weakness is more pronounced than flexor, cervical anterior horn cells are severely damaged, upper extremity sputum radiation is reduced or disappeared, and both upper limbs appear simultaneously or several months apart; Later, there were paralysis of the lower extremities, gait of the scissors, increased muscle tone, hyperactivity of the sputum and Babinski sign, and a few cases started from the lower limbs, and the upper limbs were monitored.
2 medullary palsy usually appears late, even if the brain stem function is severely impaired, the extraocular muscles are not affected, and the sphincters are not involved.
3 may have subjective sensory abnormalities such as numbness, pain, etc., but there is no objective sensory disturbance even in the advanced stage of the disease.
4 The course of disease continues to progress, eventually dying due to respiratory muscle paralysis or concurrent respiratory infection; the disease has a short survival period of several months, the elderly for more than 10 years, an average of 3-5 years.
Examine
Examination of amyotrophic lateral sclerosis
The following examination methods are useful for the diagnosis of this disease:
1. Neuroelectrophysiology: The electromyogram is a typical neurogenic change. The fibrillation potential can be seen at rest, positive sharp waves, and sometimes the vibrato potential can be seen. When the small force contracts, the motor unit potential time is widened and the amplitude is increased. The multiphase wave increases, the vigorous contraction presents a simple phase, the nerve conduction velocity is normal, and the motor evoked potential helps determine the upper motor neuron damage.
2, muscle biopsy: helpful for diagnosis, but no specificity, early neurogenic muscle atrophy, late in the light microscopy and myogenic muscle atrophy is not easy to identify.
3, other: blood biochemistry, CSF examination, no abnormalities, creatine phosphokinase (CK) activity may be mildly abnormal, MRI may show that some cases of spinal cord and brain stem atrophy become smaller.
Diagnosis
Diagnosis and differentiation of amyotrophic lateral sclerosis
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
The disease needs to be differentiated from cervical spondylosis: amyotrophic lateral sclerosis can be manifested as neck discomfort, upper limb numbness, weakness, muscle atrophy, etc. due to the first involvement of the cervical spinal cord. Muscle twitch is the most substantial symptom. A characteristic symptom of ALS, extensive and long-lasting muscle tremor is the primary clinical symptom of differential diagnosis of early ALS and cervical spondylosis. Cervical spondylosis generally has no fasciculation and tongue muscle atrophy, fibrillation, difficulty in swallowing, and unclear vocalization. Such as the damage of the brain nucleus.
