Weakness found on neuro exam and other concerns

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Hithere20202

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I’m a 34 year old female with some pretty scary concerns. I would be lieing if I said I wasn’t constantly analyzing myself now and anxious about it all.

I recently went to a neurologist because I was experiencing a mild left facial droop and some swallowing difficulties (feeling like liquids weren’t going down well, clearing my throat a lot, coughing after liquids at times and having to double swallow). I also have been very fatigued lately with generalized weakness. In addition, I have lost about 18 pounds in the span of 3 months without effort. I am already quite thin. The weight I lost is not due to the swallowing trouble because that is recent- within the past few weeks.

The neurologist said the droop wasn’t very noticeable but noted bilateral eyelid ptosis and did a very comprehensive exam which took over an hour. He noted fatigueable ptosis of eye lids, slight weakness of eye closure, as well as fatigue-able left neck weakness (SCM muscle) after having me push against resistance many times which he rated a 4/5 strength. All other strength testing was normal throughout body.

He also noted a slightly brisk jaw jerk and a slight droop on my left lower lip. I have also felt like my tongue is swollen lately and is getting in the way of my teeth when I talk. He noted my complaints but said he didn’t notice any speech impairment. He examined my mouth, tongue and face- and noted normal facial movements, normal tongue strength and no tongue or face fasiculations on examination and also normal palate movement.

All other reflexes and examination throughout my body were normal. I also told him of body wide fasicultions and a quick in office (20 minutes maybe) emg on upper and lower limbs was unremarkable/clean. He did not emg my mouth or face. I also have numbness of my toes which comes on and off which emg didn’t show any issue.

He feels with my eye weakness, swallowing complaints, marked fatigue and generalized weakness we are most likely looking at MG. He prescribed me a medication Mestinon which is helping with general muscle strength fatigue and eyelid ptosis but does not seem to be helping with the mouth droop much, feelings of swollen tongue getting in my way or swallowing trouble. I feel like my gag reflex is hyperactive. I also have been having some recent issues of shortness of breath.

He ordered a ton of blood work to rule out certain cancers, support an Mg diagnosis and some other auto immune stuff. He also ordered a repetitive nerve stimulation test of my face and a chest CT. He also ordered a swallow study. He did not order any other tests. I am waiting on blood work and other tests coming up in the next few weeks.

My questions are
1. Can als of the bulbar type present this way?
2. Does it sound like I should push my neurologist for more testing to specifically rule out als?
3. Does this sound like als to you?
4. Does bulbar als ever present without noticeable speech issues to others but feelings of big tongue getting in the way?
5. Can als present with weight loss in this way without atrophy? I know with swallowing issues it might but I’m eating just fine.

Thank you for your time

Also want to add that I do also choke on my saliva at times and feel like my throat is swollen or something
 

Nikki J

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20 minutes is long enough for a screening emg.

it sounds far more like mg ( which can have negative blood results btw do not panic if this happens) than any form of ALS

i would wait for my test results and even then I would let my neurologist do their job. They sound perfectly competent and thorough

no

one can never say never but what you describe is not typical of ALS

most ALS weight loss is swallow related. Some is related to respiratory failure. Some is due to metabolic changes but your whole picture sounds like mg and apparently your neuro thought so. If it were ALS you wouldn’t see improvement with mestinon
 

Hithere20202

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Thanks. Waiting is the tough part. The Mestinon helps but I was kind of hoping for complete resolution of symptoms which isn’t the case. I actually noticed it’s working because when I don’t take it/I’m overdue I can barely get up or open my eyes. So at first it felt subtle until it wears off and I realized how it was helping. The weight loss sucks and is a little scary tbh.

Also one more question if you don’t mind- in your experience- Is it likely the emg of limbs would have shown even a little *something* if als were the case?

Thanks again
 

lgelb

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Yes, the EMG would have shown motor neuron damage. That it did not and given your issues and response to Mestinon, all argue against ALS.
 

Hithere20202

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Hi again

Most of blood work came negative for MG so my neuro is looking into a possibility of seronegative MG due to my mestinon response and such. He is also testing for a few other less common antibodies that can show up. Swallow study is next week.

This morning I had a few facial muscles EMG’d- around my mouth/lips, forehead, cheek. Everything checked out. No tongue emg though. Wondering if this emg would rule out bulbar als?

Thank you
 

Nikki J

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The tongue is not the only site to look for bulbar. It isn’t even the best one. It sounds like the muscles chosen for your emg were the ones that control the movements that are problematical for you so if it was negative it indicates that your issues are not caused by lower motor disease
 

Hithere20202

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Thanks- makes sense. This might be a dumb question but could my issues be caused by UMN disease? Is that something different?
Just trying to figure this all out- still in limbo and so much feels uncertain.
 

Nikki J

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weakness is generally a lower motor sign in ALS. Upper motor neuron signs are things like spasticity and hyperreflexia. They are found on clinical exam. It really seems like this isn’t ALS. Your symptoms don’t sound like it your emg was negative and mestinon helps
 

Hithere20202

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Quick emg questions-I was reading online today that when an emg is performed the needle should stay in the muscle at rest for 90 seconds. When my screening emg was performed the needle was only in the muscle at rest for just a few seconds before the doctor had me contract and relax.
1. Would an emg performed this way pick up abnormalities?
2. The doctor said to me- Your motor units look good. Is this important/relevant when it comes to ruling out als?

My swallow test is next week and I’m still waiting on MUSK and a few other antibodies. I’ll update again after my swallow test. Thank you.
 

Nikki J

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I have had many emgs for research. I do not believe that anyone ever spent 90 seconds on a normal muscle as you describe. If it was a reputable source I can only guess this is one of the rules that are on paper and not real world. My emgs were done by experts

yes that is relevant. People with ALS do not have normal motor units.

again you have a normal emg, you improved with mestinon which wouldn’t happen with ALS and your symptoms are consistent with mg not ALS. Seronegative mg exists. You need to let this go. Please only post when you have a definite diagnosis and stop googling for your own good
 

Hithere20202

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Thanks for the response.

Yes I understand. It is the swallowing troubles that are not really resolving with mestinon and are most concerning. They do fluctuate though which I know is more typical of Mg. I will post again once I have a diagnosis.
 
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