Need some advice and insight!

[steveche42]

Hi,

I want to mention that I did read over the "common concerns before posting". This was super informative.

My wife (31 years old) was hospitalized about 13 weeks ago for GBS with signs and symptoms of bilateral hip weakness with shortness of breathe and difficulty picking legs up. That is what they diagnosed her with at that time. It all started from a bacterial or viral infection in the stomach about 4 weeks prior to her hospitalization. This all presented like GBS. She received IVIG and it appeared it helped. She was doing well and d/c from the hospital that week.

About 5 weeks later her symptoms returned. She had only motor symptoms. Started with profound fatigue and then just weakness in both of her legs (particularly her hip flexors on both sides). She explains there is a "disconnect" and just cant pick them up (walking super awkward). From then on, her symptoms literally stayed for about 10 days. then slowly gradually got better. I absolutely understand that this does not sounds like ALS since symptoms gradually got better. However, I was researching published articles on instances of "ALS reversals". Based on its definition it seems that it has been recorded that symptoms come and go with someone dx with ALS.

Since then my wife's symptoms kept coming and going almost like 7 days of symptoms that had a peak weakness causing great difficulty to walk and then gradually go away. Then 4-5 good days and keep repeating until recently her symptoms haven't gone away. There is a noticeable poor gait and weakness.

Some objective data: 5 weeks from being discharged from the hospital is when she had her first episode- at this time we went to a regular neurologist who did the EMG and NCS- which was normal. We got a neuromuscular specialist who did the EMG and NCS last week and it also came back normal. Both of her knees are hyporeflexia, she has some ankle reflexes and all upper body reflexes are normal(no symptoms). All blood panels, MRI (without contrast from the hospital), Lumbar puncture (protein levels were high end of normal), copper, B12, potassium, ANA, Antibodies IGG, came back normal. Isn't it true that ALS is mainly dx by ruling everything else out? Based on symptoms and EMG?

Just 3 days ago my wife is c/o some fasciculations in her R quad and today was in her calf. She never had this before. All along with her main complaint of both legs feeling weak.

With that being said, could the EMG be done too early- and this is slow progression?

The current neurologist is dismissing us based on the EMG and NCS results and doesn't know. He is referring us to a place that specializes in more rare disorders and he mentioned an ALS clinic.... But, i even asked him "so, you do you think this is ALS?" He replied "no i don't think so, but those places will see much more rare disorders"

 

[ShiftKicker]

Hello-

It sounds like your wife has really been through the ringer. This really doesn't seem like something that can be connected to ALS, given how things started. It does sound like your wife is being referred on to a clinic where her pattern of symptoms might be recognized and where there will be more resources to help figure things out.

The cycle of impairment, then improvement and impairment again means it's something other than ALS. I am sure others more knowledgeable will weigh in here, but post viral issues can definitely cause a whole variety of symptoms. The improvement with ivig would support something other than ALS as well.

With regards to your question about EMG being done too early to detect ALS- with your wife's symptoms being pretty profound, the emg would have picked LMN issues up if that is what had been causing the weakness. EMGs are pretty sensitive and can pick up signals before a limb is obviously affected. The person conducting the EMG/NCS will have provided a conclusion on the report that might give you a bit more info as well.

When is your spouse's next appointment?

 

[lgelb]

Your assumption that negative tests + symptoms can equal ALS even with normal EMGs is not true. There are specific clinical criteria, beyond what she perceives, that she evidently does not meet.

So the notion of "ALS reversal" that you think you're reading about, can't apply to your wife, both because she doesn't meet ALS criteria and because waxing/waning symptoms are not a "reversal."

Acute immune-mediated polyneuropathy syndromes, including those with a viral prodrome, that wax and wane and are responsive to IVIG (was another course considered?) are a real thing, just to name one possibility. EMG results with these can wax and wane as well.

I'm glad you're going to a clinic that sounds like it can shed more light.

 

[steveche42]

Hi,
Thank you very much for your responses. We are at the moment trying to make an appointment with the clinic. I am unfamiliar to motor neuron diseases but of course the big one is ALS. I've read through this forum and it is common that a lot of people mention the EMG would pick up ALS even before weakness begins. Is this true? Is there any research on this or mainly anecdotal?

As for the IVIG, even though my wife responded to that in the hospital, this neuro we just saw doesn't believe in the dx of GBS or CIDP- mainly because the EMG is coming out normal. Which is why he is referring to another clinic such as one that is MND. I asked if the EMG can not pick up CIDP because its too early or based on the type of variant she got from the viral infection? He said based on his 30 years of experience that he never seen GBS or CIDP with normal EMG.

Its tough, because the history of onset points a lot to an acute immune-mediated polyneuropathy syndrome. And because he gets a normal EMG he wants use to go to a motor neuron disease clinic!

 

[lgelb]

There are other acute PNs beyond GBS and CIDP. And there is literature showing EMGs may show findings in only a limited time window.

But that said, any neurologist in an ALS clinic has seen and treated more than ALS, so think of it as getting a second opinion on whatever neuromuscular condition(s) she may have.

Most PALS present with clinical motor impairment so there is limited data of the kind you are asking for. What we can say is that among those presenting with localized weakness, the vast majority of PALS show abnormal EMG findings in muscles that they think are fine.