I. The extent of motor neuron disease involvement.
1, anterior horn cells of the spinal cord
2, brainstem motor nuclei
3, cortical pyramidal cells and pyramidal tracts.
Second, the incidence of motor neuron disease:
Morbidity: annual incidence of about 2/100,000, population prevalence of 4-7/100,000, more than 90% of cases are disseminated.
Prevalence in adults: usually starts at the age of 30-60 years, more common in males.
Heredity: 5%-10% of the affected individuals have heredity, and 20% of them can be detected as SOD1 gene mutation.
Three, the cause of motor neuron disease:
1, genetic factors, SOD1 gene mutation is one of the most studied elements in the pathogenesis of motor neuron disease. sOD1 is a human all cells highly expressed protein, called copper / zinc superoxide dismutase.
2, Excitatory amino acid toxicity.
3, Free radical oxidative damage.
4, Neural filament and neuronal degeneration.
5, Abnormal mitochondrial morphology and function.
6, Environmental factors and viral infections.
Motor neuron disease is a heterogeneous disease, and many of the hypotheses involved in the studies cannot explain the whole process of MND pathogenesis.
It may only be a stage or link in the pathogenesis of motor neuron disease. What mechanisms and causes lead to the process of motor neuron damage, and what are the causes of MND?
There is no clear conclusion as to what causes the process of motor neuron damage, and whether MND is only a selective involvement of motor neurons.
Clinical typology of motor neuron disease:
1. amyotrophic lateral sclerosis (ALS, which is often referred to as “acromegaly” on the Internet, and patients are called “acromegaly”)
2, progressive spinal muscular atrophy (PSMA)
3, progressive medullary palsy (PBP), also known as ball palsy
4.Primary lateral sclerosis (PLS)
V. Auxiliary examinations:
1, neurophysiological examination.
(1) electromyography and nerve conduction velocity measurement: are the most valuable means of diagnosing motor neuron disease, especially in the early stages of the disease.
(1) Electromyography and nerve conduction velocity measurement: these are the most valuable tools for the diagnosis of motor neuron disease, especially in the early stage of the disease.
(2) Motor evoked potential (MEP): This test provides objective evidence of upper motor neuron involvement in MND.
This test provides objective evidence of upper motor neuron involvement in MND because both ALS and PLS have prolonged central motor conduction time.
(3) Single fiber electromyography (SFEMG)
2.MRI examination.
3.Cerebrospinal fluid examination.
VI. Diagnostic points of motor neuron disease:
1, middle-aged insidious onset, chronic progressive developmental disease course.
2, muscle weakness, myasthenia gravis, muscle bundle tremor and tendon reflex hyperactivity, pathological signs and other signs of upper and lower motor neuron involvement.
3. Neurophysiological examination showed neurogenic damage in at least 3 of the 4 segments (brainstem, cervical, thoracic and lumbosacral medulla).
Neurophysiological examination shows neurogenic damage in at least 3 of the 4 segments (brainstem, cervical, thoracic and lumbosacral medulla)
4.No sensory impairment and no urinary or bowel disorder.
5. Exclude other diseases that can explain the clinical manifestations.
Differentiation of diseases in motor neuron disease:
Atypical motor neuron disease cases must be differentiated from the following diseases:
1.Spinal muscular atrophy (SMA)
2.Spinal cord type combined with neurogenic cervical spondylosis
3, multifocal motor neuropathy (MMN)
4, single limb muscular atrophy (Hirayama disease)
5, ALS superimposed syndrome
6, X-linked spinal bulb neuron neuropathy (Kenney’s disease)
7, spinal cord cavitation disease
8, post-polio syndrome
9, cervical combined with lumbar radiculopathy
10.CIDP
11, benign muscle bundle tremor
VIII. Treatment of motor neuron disease.
There is no effective method for the treatment of MND. the aim of MND treatment is to prolong the life of patients through various effective methods and practical measures: to improve the quality of survival and quality of life.
1.Riluzole, also known as Lirutai, is the only drug approved by the FDA for the treatment of ALS (motor neuron disease, acromegaly), the adult dose is 50mg, 2 times/day, and laboratory tests need to be performed regularly when taking it.
2.Vitamin drugs, high dose oral, the effect varies from person to person.
3.Chinese herbal medicine treatment (some prescriptions and herbal medicines can give some help to patients).
4.Allopathic treatment.
5.Supportive treatment and psychotherapy.
6.Comprehensive treatment and effective nursing methods.