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  Motor neuron disease Definition Clinic features Diagnosis & Differentiating diagnosis Treatment Prognosis Definition(1) Motor neuron disease (MND) is a group of diseases characterized by chronic , progressive neuronal degeneration. 运动神经元病是一组慢性进行性神经元变性疾病。 Definition(2) The cause is unknown. The degeneration predominantly involves motor neurons , including upper motor neurons and lower motor neurons. 病因不清。变性主要侵犯上、下运动神经元。 Clinical manifestations（分型） Clinic features-ALS（1） Age: 30 - 60 years old Onset is insidious Course is chronic and progressive 好发于30-60岁，起病隐袭，呈慢性进行性病程。 Clinic features-ALS（2） Clinic features-ALS（2） Clinic features-ALS（2） Investigative Study Electromyography shows neuropathic lesion 呈典型失神经支配改变 Imaging study, CSF, Evoke potentials are usually normal Diagnosis & Differential Diagnosis Treatment No effective treatment Symptomatic therapy and supportive measures Prognosis Prognosis is very poor. Most commonly from pulmonary infections and weakness of respiratory muscle. Summary Motor neuron disease is characterized by chronic, progressive degeneration of motor neurons. Weakness of the affected muscles is the most predominant clinical symptom Weakness may result from upper and lower motor neurons lesion No sensory deficit It can’t be cured now. But a series of therapeutic trials are in progress * * 熊 静 Motor Neuron disease 运动神经元病 Anterior horn Pyramidal cells Motor nuclei Pyramidal tracts Upper motor neuron deficit Primary Lateral Sclerosis Lower motor neuron deficit Progressive spinal muscular atrophy Mixed upper and lower motor neuron deficit Amyotrophic lateral sclerosis(ALS) Progressive Bulbar Palsy 原发性侧索硬化 进行性脊肌萎缩 肌萎缩侧索硬化 进行性延髓麻痹 Weakness of upper extremities ; Atrophy and fasciculations Weakness of lower extremities ; Increased muscle tone Increased tendon reflexes; Positive Babinski si