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What specifically is in the spinal cord causing the weakness and paralysis in ALS patients? 

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Neurodegeneration in SOD1 ALS is likely to occur primarily in the spinal cord.
Open accessJournal ArticleDOI
M E Westarp, H H Kornhuber 
11 Citations
Loss of either type of nerve cells results in weakness, and weakness is the major symptom of ALS.
Therefore, in cases of bilateral vocal cord paralysis of unknown etiology, ALS should be taken into consideration.
Weakness and fatigue were poorly correlated in ALS patients and may be independent measures of the pathogeneses of ALS.
- ALS is a sufficiently common disease that should be considered in the differential diagnosis of focal, atrophic weakness without sensory disturbance in an adult of any age.

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