Second Opinion Today

[tmasters]

My symptoms began early last summer, with muscle cramps and weakness in my right calf muscle. As of now, the weakness has spread to both legs (upper and lower) and my right arm is showing some weakness as well. I also have the muscle twitching.

The neurologist did a EMG and ordered blood work. No MRI so far. But on January 23 he told me I have "some kind of Motor Neuron Disease" and he "suspects ALS". He suggested I get a second opinion and submitted a referral.

I went through the emotional crisis that I know most of you are familiar with. I had to find out what this "Motor Neuron Disease" was (never heard of it). I was all over the internet trying to pick up whatever information was out there. Bought some books. Fortunately, I have a lot of support from family. I had to re-learn how to pray, and finally found it again. After 2 or 3 weeks, I began to receive peace and acceptance.

I was naive enough to think I would be getting my second opinion within a couple weeks. :-? Well, it's finally here and I'm seeing Dr. Michael Graves at UCLA today.

Wish me luck. I'll report back.

-Tom

[jimercat]

Tom,

Good luck and I hope the dx is not confirmed and that you have something treatable! Keep us informed!

[Icanmanz]

Good luck, Tom. Keep us posted! Prayers are helpful, aren't they? God bless!

Irma

[crystalkk]

Tom,

Good luck, let us know how the appointment went.

[sharonca]

I saw Dr. Graves for my diagnosis. He is wonderful. Makes you feel like he has all the time in the world. One thing to note - he would not give me a diagnosis without my husband being present. Good luck.

Sharonca

[tmasters]

Thanks to those of you who posted with support. I just got back from UCLA a little while ago. So the answer is ... wait. :?:

He said that I have signs which point to ALS, but not enough to definitively diagnose it at this time. I have a few LMN signs in my arms and legs. But the Babinski sign is the only UMN sign I am showing right now. It's not enough for a clinical diagnosis.

As much as I want to know what's wrong without any doubt, this is still a good thing. I have so many people who are going to be asking me what the diagnosis is, and I'm trying to figure out what to tell them and how to explain it.

I really liked Dr. Graves. I had my parents with me to help take notes and ask questions. And Dr. Graves really takes the time to carefully explain everything and make me feel comfortable.

He suggested I try an IVIG to rule out Multifocal Motor Neuropathy. I have to wait for the report to get to my neurologist and then work this through him. Any of you done this?

So I guess I really didn't expect a definite diagnosis. I will keep telling myself this this a good thing.

[CindyM]

It is a good thing, T. but I know all too well how many mixed feelings come up every time we go to a Doctor.

[Peg B]

Hi Tom,

I have been through this and have written the story many times here, so you can click on my name and see my other posts. In summary I was DX with ALS on Oct 31, 2007 (symptons since 2002) and for second opnion Dec 7, 2006 I was put on IVIG trial beginning in Jan 2007. At this point I have not gotten better and slightly worse, so I will get another EMG/NCV on May 1, in order to compare it to the 2007 EMG/NCV. If I am worse or not better, I will go off IVIG and will get ALS DX. IVIG is very expensive. It is covered by my insurance. If I get really bad fast, I will go back on IVIG. Its just so hard to DX.

If you do have other questions/concerns please don't hesitate to ask. Take Care, Peg

[andyvaughn]

Tom... we are also going to Dr. Graves for a second opinion, having some trouble getting our records over there, ... been yelping about it the past few days and seem to be making more progress. That being said, did you have a lumbar puncture? If so, do you know if you had any elevated antibodies in your spinal fluid?
My husband has both upper and lower "signs".. he has what is referred to as a bulbar onset, but no one can explain to me why he has elevated antibody levels in his CSF, and I haven't found any other PALS that has had that also...
That being said, it sounds like the world of the undiagnosed can be very frustrating, I am hoping and praying that the treatment works for you!! -
Andrea

[tmasters]

Andrea,

Be patient with UCLA. I can totally relate to the frustration of getting your records over there and getting your appointment setup. In my case, my original neuorology appt and referral was January 23 and it wasn't until February 7 that all the paperwork was ready to be submitted for UCLA doctor review. Then you have to wait another 2 weeks for the review before you can even make an appointment! And in my case 2 1/2 weeks later my appointment date arrives.

I didn't have a spinal tap and Dr. Graves told me that, at least in my case, there's probably no reason to have one.

-Tom

[andyvaughn]

Thank you Tom. I know, the process is painfully slow. We are actually leaving tonight for Jamaica... plan on leaving the beast ALS here in the states, will start the assault again upon my return
Andrea -

[juliesmile]

Tom,

We met earlier, when you first signed on as new. My husband has similar symptoms and we compared notes. He also went for second opinion on the 10th. We went to USC. Steve is showing symptoms only in his lower extremeties. No weakness in arms. He has fasiculation all over now and his over all size is diminishing. Fatigue is a huge issue.

Anyway, we had thorough exam from Dr. Beydoun including EMG/NVC studies. I figured we would get the same answer you did ie more waiting more tests. Boy were we in for a surprise. He pulled us into his office and basically without fanfair or sympathy said it looks like Motor Neuron Disease. "Unfortunately that is what it looks like". I'm going "O.K. like what do you mean." I expected a little more explaination. So finally after digging around we get him to say it was Progressive Muscular Atrophy which (after going home and reading) is a variant of ALS. It affects only your lower motor neurons, so he doesn't have any brisk reflexes, clonus, spastisity,normal babinski etc.

He will suffer the same fate as an ALS patient and 50% of the time he will progress and his UMN will become involved and at that point it becomes ALS. Life expectancy is a bit longer more like 5-10 years.

Anyway as for knowing or not knowing it's mixed bag. We have been a mess, but at least we are not in limbo any longer. We are trying to get over the shock, cry a lot, tell friends and family and then just go about the business of living, in a hurry.

I would be happy that they are still looking for something else to pin these symptoms on and then pray they find something. I would give anything to go back to the 10th and not have a diagnosis.

By the way we are going for a third opinion at UCSD. There was no support for him. No counseling, no meds, he could use somethig to help him sleep. The nights are the worst. I couldn't believe they would give him this dx and then just send us home. "Be strong, try not to think about what is to come, do no more do no less, just try and be happy". Literally that's what he said. I would not recommend USC.

[CindyM]

I am sorry, Julie. Sorry for the DX and sorry for the way it was handled. Sometimes I think doctors are in their line of work to cure folks, and when they find out that is not possible they are at a loss for how to act, some of them. I have great respect for the docs and nurses that go into pallitative care, knowing their interventions are important but they will not get the same sort of success as their peers who are busy curing people. theirs is a differnt success, and it is too bad you did not meet a doctor who understands this. Cindy

[juliesmile]

Cindy,

Thank you for your quick response. WE can always count on you to respond. You are always so kind and understand our need for someone to hear us. You are such an asset to this forum. I hope you have in your life what you offer to all of us. I would have given anything to have you by my side on the 10th. Thank you.

Julie

[CindyM]

Gosh, Julie. You have me blushing. And I would have gone with you in a heartbeat, too!

But here is a tip for everyone who posts an unkind remark or says something they regret. It happens to us all, of course. But I find that I am better on a forum such as this. No so good in real time, you see. I am one that does not think guick on her feet. SO I am much better in this delayed-response sort of thing, where I can sit back, formulate my answer, post it, read it, and revise it if necessary. That's the secret. :-D

Anyway, I hope you and DH are recovering from your visit. No matter how you cut it, it is still a shock!

[juliesmile]

Tom,

Do you have any atrophy? And if I remember right, no pain, numbness or tingling?

[tmasters]

Hi Julie,

Yes of course I remember your reply to my original posting. And I read about your 2nd opinion yesterday on the other thread. I feel a special connection, as mine and Steve's stories and timelines are very similar. I actually asked Dr. Graves about PMA and he said "some people call it that, but it's ALS". So a lot of this is up to the doctor's interpretation and semantics. But I've also read that the "PMA" or "LMN ALS" whatever you want to call it, gives you more time.

I received a copy of Dr. Graves report in the mail Friday and he said "Since he does not meet the El Escorial criteria, he does not qualify for ALS, however I am very suspicious that that will be the ultimate diagnosis". He sounded more hopeful and optimistic in person. It's a report to another neuro, so I understand why he has to tell it like it is. When we read that, my wife and I had a good cry and then moved on.

I'm relieved and somewhat hopeful about the possible DX of MMN but statistically, the chances are low. According to the University of Washington website, MMN is ~50x rarer than ALS, and 80% of the time symptoms appear first in the hands and arms rather than the legs. Doesn't really fit my case. So it's 250:1. But it's something to hope for. You should ask UCSD about it.

http://neuromuscular.wustl.edu

I'll certainly pursue IVIg treatment, but I'm not going to waste time running after MMN, and rather I've decided to start Lithium right away. Maybe false hope, but false hope is better than no hope.

Regarding trouble sleeping, etc. you should talk to your family doctor. Mine is more than willing to work with me on this type of thing, especially after hearing my neuro's dx. I just take an over-the-counter to sleep now, but he's offered me more.

-Tom

[juliesmile]

Tom,

Those reports are quite impersonal aren't they? WE got ours as well. Pretty harsh.
Steve doesn't meet the El Escorial criteria either, that's why he has the Motor Neuron Disease/PMA diagnosis. At this point he is only showing LMN symptoms/signs. No brisk reflexes, spasticity, tone etc. But he has fasiculation body wide, atrophy, and weakness and the EMG/NVC results all point to MND/PMA. But our Dr. ndicated tat the UMN may become involved at any point. I realize he will suffer the same fate as a full blown ALS patient, as it is the LMN problems that actually get you in the end. But I have to admit getting this DX at first has made it easier for him and his family. Just being able to add the little statement, "but the up side is PMA isn't as aggressive as ALS and he has 5-10 years rather than 3-5" makes it a little more bearable.

We have found the Tylenol PM and it is working fine. He actually has slept the past 2 nights without anything.

Our Dr. ruled out MMN. But good luck with your IVIg treatment. We will ask about lithium at our next appt.

Take care Tom, and best of luck to you and your lovely family.

Julie

[bluebottle]

Tmasters - excuse me for jumping in on your post but I still dont have to many privlages on this site yet - I want to speak to Juliesmile as I am early on this journey and my symptoms and those of your husband Steve are very simililar.

I was recovering from a leg operation and so my initial weakness and foot drop was put down to that trauma but as I have recovered from the op my leg weakness remains despite the concerted efforts of physio's. I have foot drop in both feet and so wear AFO's. My quads are very weak and never recovered from being immobilised following the op. I had NCS which showed no neuropathy but after the EMG was told that my neurologist would be asked to look at my back - that was 8 weeks ago and have heard nothing more from the hospital. I am not sure whetherI should chase it up - I have an added complication in that my thyroid results are not normal either.

Julismile - any advice being that bit further down the raod - I am managing this alone and feeling very alone at the moment as can't say anything to friends as no diagnoses. Thanks

[1Mother]

Hi Bluebottle. Interesting about your symptoms developing after "leg surgery". Did you receive Nitrous Oxide? Also how old are you? Can you describe a pattern of weakness, sensitivity or temperature changes consistent with neuropathy in your legs? Your operation might hold the key to what ails you. Hope you had a nice weekend in spite of this stress.

[bluebottle]

Hello Mother1,

Thank you for your reply. I am not sure what I had - it was a general anesthetic with the needle in the back of the hand. I remember waking up and having an injection due to shaking. I am now 41. You ask about sensitivity or temperature changes in my leg - I don't have that. Also I was told I definately don't have a neuropathy after the NCS and after the needle EMG was told that the report would recommend that my back is looked at. Not sure if this helps any. I have a reflex in my knees but only just move - there was no reaction to the bottom of the feet test. I think I am a conundrum!

[juliesmile]

Bluebottle,

I would definitely chase after a follow up. If the Dr. recommended they look at your back after doing EMG and NCS, then in all liklihood the problem stems from something pressing on nerves in your back. I'm no Dr. by any means, but I am hopeful that ALS is not a path you will have to travel. The good thing you have to focus on is the EMG and NCS came back normal. If those showed denervation or conduction block youmay need to consider ALS. But get in to Dr. and get an MRI of your spine. The fact they haven't followed up on this is inexcusable.

Take care and stay in touch.

julie

[bluebottle]

Thanks for the reply Julie and I am grateful for everyone reading and replying to posts despite their own situations.
During the EMG the doctor said that there was difficulty in getting some activity on insertion of the needle. At the end of the NCS I was told I definately dont have a neuropathy but I was not told that I definately didnt have something at the end of the EMG test. Excuse my ignorance - does ALS always have to have a positive NCS and a neuropathy? I am not sure now, does PMA have a negative NCS. From what was said I don't know that I had a 'normal' EMG but desperately need that appointment to know for sure. Thank you for letting me stay in touch - I really hope I am in the wrong forum, but just now dont know where I belong.

[wright]

Hello bluebottle

Typically, the sensory part, as well as the conduction part of the NCS is clean with ALS (motor amplitudes can be affected, though).
It's the EMG that will show acute (positive waves and fibrilations) and chronic denervation/reinnervation (increased motor unit amplitude) and fasciculations.
Just so you know: you can have acute and chronic denervation/reinnervation with a radiculopathy which can be caused by a slipped disc, for example. Also, if you have muscle atrophy and just acute denervation, then it's not going to be ALS.
Bottom line: "dirty" EMG's don't always mean ALS; there are many other things that can cause "dirty" EMG'S. You have to look at the clinical picture and the EMG together.

Hang in there.