昆明医学院第二附属医院.ppt

Neurology Department of Neurology, The 2nd affiliated hospital, kunming Medical college 昆明医学院第二附属医院 神经病学教研室 熊 静 Motor Neuron disease 运动神经元病 Motor neuron disease vDefinition vClinic features vDiagnosis Atrophy and fasciculations Weakness of lower extremities ; Increased muscle tone Increased tendon reflexes; Positive Babinski sign Clinic features-ALS（2） Weakness of lower extremities; Increased muscle tone Increased tendon reflexes; Positive Babinski sign Weakness of upper extremities ; Atrophy and fasciculations Difficulty in swallowing and speaking； Drooping of the palate； Atrophy and fasciculation of tongue Clinic features-ALS（2） Weakness of lower extremities; Increased muscle tone Increased tendon reflexes; Positive Babinski sign Weakness of upper extremities ; Atrophy and fasciculations Difficulty in swallowing and speaking; Drooping of the palate； Atrophy and fasciculation of tongue Investigative Study vElectromyography shows neuropathic lesion 呈典型失神经支配改变 vImaging study, CSF, Evoke potentials are usually normal Diagnosis & Differential Diagnosis No sensory deficit Weakness and atrophy of the affected muscles Signs of motor neuron lesions Electromyography The keys Treatment vNo effective treatment vSymptomatic therapy and supportive measures Prognosis vPrognosis is very poor. vMost commonly from pulmonary infections and weakness of respiratory muscle. Summary vMotor neuron disease is characterized by chronic, progressive degeneration of motor neurons. vWeakness of the affected muscles is the most predominant clinical symptom vWeakness may result from upper and lower motor neurons lesion vNo sens