Doctors suspect ALS

[lululatreuc]

Hi all
New here. Doctors investigating for als.
I've lost my ability to swallow. Swallow tests show dysmotility in lower oesophagus but probably not causing my inability to swallow.
I'm very hyperreflexic with clonus and tongue fasciculations. Thenar wasting ?split hand. Muscle wasting of legs. But I can still walk ok. I'm possibly a little clumsier than normal but tbh not too worried.
I had surface emg in clinic and the dr said my face and hand were ok but was very evasive of the leg results. I'm having needle emg and autonomic tests next week.
Can anyone describe to me what their dysohagia felt like and what surface emg results are abnormal? She wrote numbers that were in the 70s for my hand but in the 20s for my legs.... she did both legs but only my right hand and left side of my face. Apparently I will have needle studies in my tongue too. Has anyone had this? Very freaked out. Trying to reassure myself but it isn't looking good.

 

[Nikki J]

Surface emg os something many of us have not had. I have but only for research and I have never seen a report. I don’t know if the number difference is usual or not.

There are different ways to test bulbar and it is up to the doctor what they are comfortable with. A tongue emg is possible but the doctors I have encountered do supraclavicular or submental ( under the chin). There are different size needles for facial emgs. An emg is not fun but done by an experienced examiner should be tolerable

 

[lululatreuc]

That's interesting. I should have needle emg on weds. I just need to know now. I've had every gastro test under the sun and we thought we had the diagnosis with dysmotility but now they think the level of swallow difficulty and the fact my symptoms are oropharyngeal mean that the oesophagus probably isn't causing this. Then someone did a neuro exam and found lots wrong and here we are looking at possible autoimmune autonomic ganglionopathy or MND.

Can anyone with bulbar als describe the dysphagia? Mine is like I can't get the swallow to start. Once it's started, aside from a little pooling in the pharynx it's ok. I'm told pooling can be a neuro thing but mine is mild. For me the problem feels further forward. If I put a tablet on my tongue for example I simply cannot move my tongue correctly to swallow it. I end up moving everything I wasn't to swallow to the front of my mouth in front of my teeth and letting tiny bits back.

I can't manage solids at all. Puree or softer (or chewed to excess). Even swallowing liquid can be hard. The easiest is puree because it sticks together. Things like scrambled eggs are hard because they disperse in my mouth and it's hard to keep control of it.

 

[lululatreuc]

It's not ALS. I'm pleased to report my EMG was clean but my autonomic tests were not. So I have autoimmune autonomic ganglionopathy with some neuropahtic overlap. It's not a great diagnosis but I think prognosis is around 10 years and there are some treatment options to improve things, IVIg and possibiltiy of a stem cell transplant as i have lupus too. I'm relieved It's not ALS.
Sending so much love and well wishes to all of you- the stress of having drs just suspect this disease was bad enough. I wish you all to be as well as you can.

 

[Nikki J]

Thank you for letting us know. I am glad you don’t have ALS and wish you luck with your treatment. Do consider a second opinion as it is a very rare disease. Best wishes