1. Neurology Department of Neurology, The 2nd affiliated hospital,
kunming Medical college

2. 昆明医学院第二附属医院 神经病学教研室
熊 静

3. Motor Neuron disease
运动神经元病

4. Motor neuron disease Definition Clinic features
Diagnosis & Differentiating diagnosis
Treatment
Prognosis

5. 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。

6. 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。

7. Pyramidal cells
Pyramidal tracts
Motor nuclei
Anterior horn

8. Clinical manifestations（分型）
Upper motor neuron deficit
Primary Lateral Sclerosis
原发性侧索硬化
Lower motor neuron deficit
Progressive spinal muscular atrophy
进行性脊肌萎缩
Progressive Bulbar Palsy
Mixed upper and lower motor neuron deficit
进行性延髓麻痹
Amyotrophic lateral sclerosis(ALS)
肌萎缩侧索硬化

9. Clinic features-ALS（1）
Age: 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。

10. Clinic features-ALS（2）
Weakness of lower extremities ;
Increased muscle tone
Increased tendon reflexes;
Positive Babinski sign
Weakness of upper extremities ;
Atrophy and fasciculations

11. Clinic features-ALS（2）
Weakness of lower extremities;
Increased muscle tone
Increased tendon reflexes;
Positive Babinski sign
Difficulty in swallowing and speaking；
Drooping of the palate；
Atrophy and fasciculation of tongue
Weakness of upper extremities ;
Atrophy and fasciculations

12. Clinic features-ALS（2）
Weakness of lower extremities;
Increased muscle tone
Increased tendon reflexes;
Positive Babinski sign
Difficulty in swallowing and speaking;
Drooping of the palate；
Atrophy and fasciculation of tongue
Weakness of upper extremities ;
Atrophy and fasciculations

13. Investigative Study Electromyography shows neuropathic lesion
呈典型失神经支配改变
Imaging study, CSF, Evoke potentials are usually normal

14. Diagnosis & Differential Diagnosis
Weakness and atrophy of the affected muscles
The keys
Signs of motor neuron lesions
No sensory deficit
Electromyography

15. Treatment No effective treatment
Symptomatic therapy and supportive measures

16. Prognosis Prognosis is very poor.
Most commonly from pulmonary infections and weakness of respiratory muscle.

17. 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