運(yùn)動(dòng)神經(jīng)元病英文課件

1、運(yùn)動(dòng)神經(jīng)元病英文課件運(yùn)動(dòng)神經(jīng)元病英文課件2022/9/102022/9/42022/9/10BackgroundThe incidence of the disease is one to two per 100 000. The disease predominantly affects middle aged and elderly people.younger people are occasionally affected. The disease is sporadic in 90% of cases, but about 10% are familial, usually with

2、 an autosomal dominant mode of inheritance. 2022/9/4BackgroundThe incidenc2022/9/10The disease causes progressive injury and cell death of lower motor neurone groups in the spinal cord and brain stem and usually also of upper motor neurones in the motor cortex. Those affected typically develop a com

3、bination of upper and lower motor neurone signs, with progressive muscle weakness and wasting usually accompanied by pathologically brisk reflexes, eventually involving the limb and bulbar muscles2022/9/4The disease causes pro2022/9/10Etiology and pathogenesis Genetics : More than 60 different mutat

4、ions, mainly point mutations, have been described the most important findings has been that mutations in the gene on chromosome 21 encoding the enzyme Cu/Zn superoxide dismutase (SOD1) underlie 20% of familial cases of motor neurone disease and 2% of all cases.2022/9/4Etiology and pathogene2022/9/10

5、Etiology and pathogenesis Excitotoxicity: Glutamate is the main excitatory neurotransmitter in the human nervous system. Excessive stimulation of neuronal glutamate receptors can damage and kill these cells. Immune factors viral infection2022/9/4Etiology and pathogene2022/9/101.Aamyotrophic lateral

6、sclerosis ALSMotor neuron of Anterior horn,brain stem,pyramidal tract.40 years old,malefemalweakness and wasting, The interossei and the muscles of the thenar eminence are affected early. spread proximally to involve the muscles of the shoulder girdle and the trunk.2022/9/41.Aamyotrophic lateral2022

7、/9/101.Amyotrophic lateral sclerosis ALSFasciculation is a prominent sign.Upper motor neuron signs in the lower limbs . There is usually a spastic weakness of the legs with increased tendon jerks and extensor plantar responses. Upper motor neuron signs may also extend to the upper limbs so that ther

8、e is a paradoxical increase in the reflexes accompanying the wasting and fasciculation.2022/9/41.Amyotrophic lateral 2022/9/101.Amyotrophic lateral sclerosis ALSEventuallye motor nuclei of the medulla are affected, giving rise to a bulbar palsy. Aspiration pneumonia is the most frequent cause of dea

9、th. This occurs within 4-5 years of the onset of the disease in the majority of cases; some patients live for 8-10 years after the diagnosis is made.No sense problem.2022/9/41.Amyotrophic lateral 2022/9/102.Progressive spinal muscular atrophy ,PSMAAnterior horne only.30 years oldIn progressive spina

10、l muscular atrophy, the signs of disease are limited to a lower motor neuron disorder, which often remains localized for several years. Apart from the absence of upper motor neuron signs this form of the disease is identical with amyotrophic lateral sclerosis. 2022/9/42.Progressive spinal m2022/9/10

11、3. Progressive bulbar palsymotor nuclei of the brain-stemThe development of bulbar palsy usually results in the death of the patient within a year or two, due to aspiration pneumonia.The bulbar muscles may be involved in an upper motor neuron paralysis. The most striking difference is in the appeara

12、nce of the tongue, which is small and stiff, instead of soft and fibrillating. The jaw jerk in this pseudobulbar palsy is brisk.2022/9/43. Progressive bulbar 2022/9/104. Primary lateral sclerosisPyramidal tract onlyPrimary lateral sclerosis is a rare sporadic disorder with exclusively upper motor ne

13、uron (UMN) involvement.2022/9/44. Primary lateral scl2022/9/10Assisting examination:1.CSF:normal2.EEG,CT,SEP :normal3.MRI:atrophy4.electromyography :neuropathic disorder2022/9/4Assisting examination:2022/9/10Diagnosis progressive ,insidious, Both the upper and lower motor neurons are involved.No disorder of sensationElectromyography2022/9/4Diagnosis progressive 2022/9/10Differential diagnosis Spinal muscular atrophy SMACervical vertebral diseaseSyringomyeliaBenign fasciculation2022/9/4Differential diagnosis2022/9/10Treatment At present t