Progression discussion

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lisa g

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I had 4 EMG's☹. First one in 2013 due to swallowing issues and loss of taste. I had a bad motorcycle accident in 2007. That was normal. Than in 2019 had one that said definite ALS. Went for a second opinion had another EMG that was inconclusive so neuro sent me to her partner for another EMG, that showed abnormalities in one area so from there I was sent to the head neuro at Cleveland Clinic in Weston. Was dreading the thought of another EMG. That neuro said another EMG wasn't necessary after reading all previous notes, test results and doing a through exam she said UMN.

Since then I switched to UA Miami for clinic. I was seen by Dr. Benatar who confirmed ALS UMN dominant. Didn't show LMN at that point. I go back to see him in August and wouldn't be surprised if LMN is involved now. Feeling spasms in arms and every now and then they lock up at the elbows when turned a certain way.

It's been no picnic and took awhile to finally get diagnosed. That was over 2 years ago and am now strapping in for the unpredictable rollercoaster ride.
 

KevinM

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Nikki, I was referring only to the many forum posters that said they had normal EMG’s. You are correct that an abnormal clinical exam even with a clean EMG would indicate that something is not right.

It just seems to me that some responses have indicated that EMG’s can’t be done too early, and that a clean EMG means you’re in the clear. My ALS doctor and many posters on other forums have said otherwise, so this isn’t an off the wall opinion I concocted.

I couldn’t discern from the Facebook posts if any had abnormalities on exam, just that they had had one or more clean EMG’s before having an abnormal one. In any event, the absence of EMG abnormalities appears to have delayed their diagnosis, even with other symptoms that might have been present upon exam.
 
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Nikki J

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There are certainly expert ALS neurologists who adhere to El Escorial. From all I know I believe mgh does and will not diagnose without a diagnostic emg meeting the strict criteria along with the exam findings. Other doctors apparently do and I have seen this reported here and by others elsewhere too. If you are seeing those with stricter views and have an atypical presentation it will take longer. However those people seeing the strict diagnosticians are being followed. This is really really different than the average dihals saying they had seen a neurologist had a non worrying exam and a negative emg

the terminology of possible probable definite may contribute to people saying diagnosis took a while. too. I knew someone ( FALS even) who was told probable ALS and really thought it was still a maybe until her husband asked when they would know. The newest criteria have dropped that terminology. they say with emg and umn findings widespread in one area or more and everything else ruled out it is all ALS. It turns out the original terminology was taken from the MS diagnostic criteria that were in use at that time
 

nona

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I am following this thread and had to go back to the neurologist's notes because I thought my first EMG was normal. Wrong! From 12/2014:

"On exam, she is a very pleasant, healthy-appearing female who looks her stated
age. She is bright and alert with a normal mental status. She had no
emotional lability while here in the office. She was dysarthric and had some
mild facial weakness. She had fasciculations and atrophy of her tongue. Her
jaw jerk was mildly increased. She had no neck weakness. Extraocular
movements were intact. Her sensation was normal. Her reflexes were mildly
brisk with downgoing toes. She had no focal weakness in her extremities nor
any atrophy. Her tone was normal. Gait and stance were normal. Romberg was
negative. Cerebellar testing was normal.

She underwent some nerve conduction studies, all of which were normal. She had
EMG testing done in her left upper extremity, right lower extremity, and left
lower extremity, all of which were unremarkable. EMG testing of her glosses muscle did
show some chronic changes consistent with denervation.

The patient appears to have bulbar findings of tongue atrophy and
fasciculations and some facial weakness.

She is not meeting the criteria of motor neuron disease at this juncture. She
has had prior testing for myasthenia, and tongue atrophy and fasciculations
would not be considered to be part of a myasthenic syndrome. What I would like
is for the patient to come back and see me again in the next few weeks. I gave
her my email and phone number so she can contact me with any questions. I made
it clear to the patient that she might have a neuromuscular disorder but that I
was unable to come down hard on a diagnosis at present."


And then six weeks later: "I repeated some EMG in the right upper extremity [NOTE. IT WAS NOT A REPEAT] to see if there was any
interval change between those done in December versus those today. She did
have active denervation in the right arm with a mild decrease in recruitment in
the FDI and APB. The patient has progressed somewhat since I last saw her. There is now
objective evidence of motor neuron dysfunction in the right upper extremity. I
believe her dysarthria is slightly worse."
 

Nikki J

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Thanks for sharing Jessie. That is good example of someone who doesn’t initially meet diagnostic criteria but had suspicious findings and how they might be followed.

was that mgh? I know I was there for a visit in 12/14. Wonder if we were there on the same day/at the same time? 😀
 

nona

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@Nikki J ., No, that was Dartmouth. I started with MGH two months later. I'm sure we overlapped at some point though.
 

Nikki J

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Sonne

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I agree Nikki. I think with this diagnoses there are too many variants to set a definite progression. I see my neurologist once a year and tell him what I can atill do and what I can't. I've had 2 emgs and was told only that I didn't have MS. That was 5 years ago now. He says I'm doing well and I already know this as I have seen ALS go very quickly when I did home-care. I don't ask for anything from my doctor except when I need different aids. I figure the more accepting I am of my diagnose the easier the ride will be. Of course that doesn't take the frustrations, anxieties or fears away but it does allow one to feel that one has done they best to live a meaningful life to the end. If that is important to you- it is to me as without some meaning why do we do anything.

They (the doctors) are primarily interested in progression as how it relates to science and possible therapies and medications down the road. They know they can mostly offer support and guidance but little if any actual help. It's about fact accumlation, which is what our part in the whole is.

As I have said it is what it is and we can only do our best. Sunshine
 

ChesterB

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I was diagnosed with ALS in October 2019 and my symptoms began over a year before then. I have limb onset in my right (dominant) hand and arm. Since diagnosis my progression has been super-slow, with fasciculations in both arms and legs and muscle atrophy affecting my right hand and forearm, particularly my thumb and forefinger. My handwriting is difficult and sloppy and I have problems grasping things. My FRS hasn't changed more than a couple points in the last year. Limb onset progression is usually asymmetrical, and I seem to be experiencing a gradual increase in weakness in my left leg and hip. It's difficult to judge since I have lumbar spine issues as well. It's taken me three years to get to this point, so I wonder if it will be three more years until I start using a walker? I take Riluzole and I'm in the oral Radicava trial. I think the drugs might be helping me. Who can say for sure?
 

EricInLA

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Well ChesterB, it seems we are still on identical slow-moving tracks (I commented on this a year ago). You are still describing my situation exactly. I am also 3 years along in this mess, and I would LOVE to get another 3 years without having to use a walker/wheelchair. It's possible with my slow progression, though I do feel stress and pain in my left leg and worry that these could be threatening to bring my carefree walking days to a close! I am also on Riluzole, though I chose not to do the oral Radicava or any other trial. I hope the Riluzole is doing something, but like you say, who knows? My neurologist told me to treat it like a Flintstones vitamin as a kid. You don't know why you're taking it, other than someone tells you to. You just do it and assume people know what they're talking about.
 
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