Adequate EMG and testing to diagnose definite ALS?

[glupavomomiche]

I am seeking your opinion on the thoroughness and reliability of my EMG regarding a diagnosis of ALS. I am 32, female. Symptoms started in Feb 2010 in right hand, stiff and slow moving. Gradually progressed to stiffness (now know this is spasticity) in all of arm over the next 3-4 months with some atrophy in hand and arm. Right foot drop started in July 2010, then progressed to leg stiffness, and a few months later, significant atrophy evident in lower leg, some atrophy in thigh. Fasiculations were first felt in both right arm and leg around September 2010. First saw neurologist at this time and was found to have hyperreflexia on the right, positive babinski and hoffman, and positive jaw jerk. In December 2010, stiffness noticed in left hand and arm, with fasiculations as well. In spring of 2011, fasiculations felt in back, butt and neck/chin as well. Left leg is fine so far. All fasciculations are intermittent and random. Increased eating time and difficulty chewing began around this time. Neurologist noted tongue atrophy and fasics in tongue in June 2011, as well as hyperreflexia on the left side with positive babinski and hoffman. All MRI’s were normal, tons of blood labs were all normal, CSF spinal tap was normal, All Evoked Potentials normal, and NCV and repetitive nerve study were normal. This is the result of the needle EMG (I don’t know how to make a chart on a post like this, so I have listed the order of the information and then given the data below (hope that is not too hard to read):

Muscle, Ins Act, Fibs, PSW, Fasics, Polyph, MU Amp, MU Dur, Recruit

R Deltoid, normal, 1+, 0, 0, none, normal, normal, normal
R Biceps, inc, 1+, 1+, 1+, none, normal, normal, normal
R Brachioradialis, normal, 3+, 0, 1+, present, inc, inc, dec
R Ext Carpi Rad, normal, 3+, 0, 1+, present, inc, inc, dec
R Flex Carpi Rad, normal, 2+, 1+, 0, none, normal, normal, dec
R Triceps, inc, 3+, 1+, 2+, present, inc, inc, dec
R FDIM, inc, 3+, 1+, 1+, present, inc, inc, dec
R Vastus Medialis, normal, 3+, 1+, 2+, none, normal, normal, dec
R Anterior Tibialis, inc, 3+, 2+, 0, present, inc, inc, dec
R Posterior Tibialis, inc, 1+, 0, 0, none, inc, inc, dec
R Medial Gastroc, inc, 2+, 1+, 2+, none, inc, inc, dec
R Lateral Gastroc, inc, 2+, 0, 2+, none, inc, inc, dec
R Genioglossus, normal, 3+, 1+, 0, normal, normal, normal, dec

My questions are:
1. The doctor performing the EMG had originally said it wouldn’t take very long based on my neuro’s orders, but as he began testing he said it may take longer (it was 3 hours!), and at the end he said he would like to go ahead and check my tongue so I wouldn’t have to come back (he wanted to do some spinal and neck muscles but by this point I was ill from all the needling and felt I would pass out and/or throw up). Based on the symptoms I have, do you feel the appropriate areas were tested? Should I try to have the rest of the muscles tested?

2. The neuro’s original thought was myasthenia gravis based on ptosis and strabismus and the way I tire so quickly (but the eye problems occurred when I was about 12 years old). Would these EMG findings also be found in myasthenia gravis (even with a normal RNS)?

3. Are these findings consistent with ALS? Do you see anything that would make you think it is something else (neuropathy or myopathy)?

I appreciate your expert opinion and time to answer my questions (and yes, I’m in denial and hoping it is something else! Still several weeks before my second opinion). Thank you!

[MG (Admin)]

ALS cannot be diagnosed by EMG alone.
The neuro exam together with the EMG are consistent with ALS although other possibilities including a combination of 2 different conditions could be possible.
Your neurologist will want to put the EMG study in the context of everything else he found.
Let us know how it goes..wishing you all the best.

[glupavomomiche]

Thank you for speedy response!! You mention in could be a combination of two conditions. Which two? And how likely would it be that they would happen at the same time? Thank you again.