Diagnosis discussion. Moved from CIHALS thread

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I will only add that both of the nueromuscular specialists who diagnosed me seemed dismissive of the El Escorial criteria when I raised it. Like it was just one of several possible methods of analysis at best, or an annoyance at worst. They both were pretty sure what they were seeing in me. Perhaps this speaks to the incomplete nature of my 2nd EMG, or maybe to the need to modify the criteria. At a minimum, Kristina's comment above re "possible-probable" terminology makes me think the criteria is seriously misleading. Words matter. Personally I feel the criteria discussed on this forum are more helpful than the official one.
 
Karen, can you help me understand the upper and lower motor neuron situation with ALS. My doctor has explained it to me but I still feel fuzzy in my understanding.
 
Karen, can you help me understand the upper and lower motor neuron situation with ALS. My doctor has explained it to me but I still feel fuzzy in my understanding.
Sure. Upper motor neuron signs include hyperactive reflexes, spasticity (increased tone), pathological reflexes such as Babinski, Hoffman sign, clonus. Also pseudobulbar affect, laryngospasm. Lower motor neuron signs are muscle weakness and fasciculations. The EMG reflects LMN pathology which is why it can miss pure PLS.

The upper motor neurons come from the brain and end (synapse) on the spinal cord. The lower motor neurons start in the spinal cord and end (synapse) on the muscles. That’s why the EMG picks up the LMN activity. Other structures are part of the pathway, so I’ve given you a simplified explanation.
 
Thank you, Karen!
 
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