1、昆明醫學院第二附屬醫院 NeurologyDepartment of Neurology, The 2nd affiliated hospital, kunming Medical college昆明醫學院第二附屬醫院昆明醫學院第二附屬醫院昆明醫學院第二附屬醫院神經病學教研室神經病學教研室 熊熊 靜靜昆明醫學院第二附屬醫院Motor Neuron disease 運動神經元病運動神經元病昆明醫學院第二附屬醫院Motor neuron diseasevDefinitionvClinic featuresvDiagnosis & Differentiating diagnosisvTrea

2、tmentvPrognosis昆明醫學院第二附屬醫院Definition(1)vMotor neuron disease (MND) is a group of diseases characterized by v運動神經元病是一組運動神經元病是一組昆明醫學院第二附屬醫院Definition(2)vThe cause is unknown.vThe degeneration predominantly involves v病因不清。變性主要侵犯病因不清。變性主要侵犯上、下運動上、下運動神經元。神經元。昆明醫學院第二附屬醫院Anterior hornPyramidal cellsMotor n

3、ucleiPyramidal tracts昆明醫學院第二附屬醫院Clinical manifestations（分（分型）型）Upper motor neuron deficitPrimary Lateral SclerosisLower motor neuron deficitProgressive spinal muscular atrophyMixed upper and lower motor neuron deficitAmyotrophic lateral sclerosis(ALS)Progressive Bulbar Palsy原發性側索硬化原發性側索硬化進行性脊肌萎縮進行性脊

4、肌萎縮肌萎縮側索硬化肌萎縮側索硬化進行性延髓麻痹進行性延髓麻痹昆明醫學院第二附屬醫院Clinic features-ALS（1）vAge: 30 - 60 years oldvOnset is insidious vCourse is chronic and progressivev好發于好發于30-6030-60歲，起病隱襲，呈慢歲，起病隱襲，呈慢性進行性病程。性進行性病程。昆明醫學院第二附屬醫院Clinic features-ALS（2）Weakness of upper extremities ; Atrophy and fasciculationsWeakness of lower e

5、xtremities ; Increased muscle toneIncreased tendon reflexes; Positive Babinski sign昆明醫學院第二附屬醫院Clinic features-ALS（2）Weakness of lower extremities; Increased muscle toneIncreased tendon reflexes; Positive Babinski signWeakness of upper extremities ; Atrophy and fasciculationsDifficulty in swallowing

6、and speaking； Drooping of the palate； Atrophy and fasciculation of tongue昆明醫學院第二附屬醫院Clinic features-ALS（2）Weakness of lower extremities; Increased muscle toneIncreased tendon reflexes; Positive Babinski signWeakness of upper extremities ; Atrophy and fasciculationsDifficulty in swallowing and speaki

7、ng;Drooping of the palate； Atrophy and fasciculation of tongue昆明醫學院第二附屬醫院Investigative StudyvElectromyography shows neuropathic lesion呈典型失神經支配改變呈典型失神經支配改變vImaging study, CSF, Evoke potentials are usually normal昆明醫學院第二附屬醫院Diagnosis & Differential Diagnosis No sensory deficitWeakness and atrophy o

8、f the affected musclesSigns of motor neuron lesionsElectromyographyThe keys昆明醫學院第二附屬醫院TreatmentvNo effective treatmentvSymptomatic therapy and supportive measures昆明醫學院第二附屬醫院PrognosisvPrognosis is very poor.vMost commonly from pulmonary infections and weakness of respiratory muscle.昆明醫學院第二附屬醫院SummaryvMotor neuron disease is characterized by chronic, progressive degeneration of motor neurons. vWeakness of the affected muscles is the most predominant clinical symptom vWeakness may resul