Does anyone have any idea what I have?

[brian32]

I am a 32 year old male and would like to share my story & hope that someone out there can relate to what I am going through & that maybe you have some possible answers.

In May of this year I started noticing some weakness in my thumb & index finger of my right hand (dominate hane). I didn't think much of it, but over a period of a month it started affecting my bicep in the same arm. In September I went to an Orthopedic Doctor & he said that he thought that it was nerve related so he referred me to a Neuro. By the end of September, I had lost alot of strength in my hand and bicep. My Neuro performed an EMG & Nerve Conduction Study on my arms and around my neck. He said that my nerves appeared to be "sick". Blood was drawn & several tests were performed. All blood work came back okay. He also pointed out some muscle atrophy between my index finger & thumb of my right hand & some in my left hand.

He then had me sent for an MRI of my neck & spine. It came back okay. My Neuro next performed an EMG & Nerve Conduction Study on my leg and up and down my back. He also did an EMG of my tongue. He said that he saw some of the same things in my leg that he saw in my arm ("sick nerves"). Next was a myelogram & cat scan of my neck & spine. It also came back okay. He sat my wife & I down and told me that he wasn't exactly sure what I had, but he did talk to us about ALS. He referred me to higher power over at the Medical College of Georgia (MCG). This Doctor teaches & specializes in Neuro Muscular diseases. He diagnosed me with CIDP (autoimmune disorder) & he and his partner assured me with 99.9% certainty that I didn't have ALS. I had a spinal tap done to confirm the diagnosis. Unfortunately, my spinal fluid had some abnormal white cells in it so he wanted to perform another tap about a week later to study these cells. In between having spianl taps, I had my 1st IVIG infusion (80 g. a day for 2 days). This was the day after Thanksgiving.

After my 2nd spinal tap, I received a call a couple of days later from my Neuro at MCG. He said that my white cells were typical of Leukemia or Lymphoma. I was set up the next day to see a Hematologist. Based on my presentation, symptoms, & physical examination, the Hematologist didn't think that I had cancer before even doing any tests. Nevertheless, I had a bone marrow biopsy performed & an MRI of the brain. Both came back okay. I was also seen by an Infectious Disease Doctor who, between the 2 Doctors, ordered an additional spinal tap & they drew 15 vials of blood. All of which were sent to Mayo Clinic for testing. I have an appointment with this Hematologist tomorrow (12/13), hopefully not only to find out that I don't have cancer, but to also find out what I do have. I also have a follow up with my Infectious Disease Doctor to find out the results of all of my blood work. Meanwhile, I can barely curl my right arm & my left hand and bicep are going through the same progression that my right hand and arm has went through. I have problems buttoning my pants, brushing my teeth, turning the ignition in my truck, etc. & I have a good bit of atrophy in my right hand and arm and some in my left. I also have had fasciculations through all of this. I also have a burning pain going down the right side of my outer thigh (nerve pain). So far, I have not noticed any weakness in my legs, but I fear that it's coming. I don't understand why it seems to be primarily focused in on my biceps and hands. Even though my Neuro is sure that I don't have ALS & that all of these symptoms & through his physical exam are indicative of CIDP, ALS is still in the back of my mind & it concerns me. Does anyone have any thoughts of what this could be? If it is CIDP, is it possible for the disorder to continue to progress after an IVIG treatment?

Thank You,
Brian

 

[Jamiet]

Brian,

It's pretty scary, we know where you are. It seems, you may have something treatable, which i guess, is the good part. It's even more difficult having absolutely no answers. Keep the faith, as you have "some" sort of direction to head and to me, it sounds 'OK'.

White cells in your spine sounds like infection of some sort.

A few questions related to ALS:

1) How were your reflexes, were they hyperactive or decreased?
2) What about Hoffmans sign or babinski sign?
3) What did your neuro mean about "sick" nerves? Did the EMG show fibs & PSW's or were your NCV's slow? I would call him and ask.
4) Could you have come in contact with some substance?


We know where you are, we are here to support you thru this.

In the meantime, how are you coping? Do you have some meds to help. I got on Wellbutrin, it helped drastically.

Another question,,,, how has the IVIG helped, have you noticed anything?

Well, hang in there buddy

Take care,

Jamie

 

[brian32]

Jamie,

Thanks for your prompt reply. Here are the answers to your questions:


1) How were your reflexes, were they hyperactive or decreased?
Decreased
2) What about Hoffmans sign or babinski sign?
Toes didn't curl up or down. Just null.
3) What did your neuro mean about "sick" nerves? Did the EMG show fibs & PSW's or were your NCV's slow? I would call him and ask.
Demyelinating
4) Could you have come in contact with some substance? Possibly. I work at a tire plant. I am a Supervisor, but was in production for 8 years.

If you're asking me do I have any upper neuron symptoms, I don't. I have pure motor symptoms. Do you know if a person can start with pure motor symptoms in the onset & later develop upper neuron symptoms? In other words, start fitting the picture of ALS. Or do you think that I would have had some of the upper symptoms by now (8 months since onset)?

I am coping the best that I can. I am the sole income provider. This disorder has put me out of work about 6 weeks ago. Once I go to short term disability, my income will be drastically cut. I'm a bit concerned about that.

I am currently not on any meds.

My 1st IVIG treatment was on the day after Thanksgiving. This Friday will be 3 weeks. I haven't noticed a difference. If anything, it seems like the progression has sped up. Hopefully I will see some improvement soon. Unless my diagnosis changes, I am scheduled for my next IVIG in January & again in March. Then I guess I'll be evaluated. I'm worried that I won't respond to this treatment.

Thanks for your reply & encouragement,
Brian

 

[vmd]

Brian:

I'll keep you in my prayers. That's quite a lot of testing you have had. This is good since they will be able to give you a better picture of what's going on. Apparently, however, they don't think it is ALS or cancer. Keep us posted.

 

[AHands]

Mine started with muscle atrophy in the left hand, especially around the thumb.

burning pain going down the right side of my outer thigh (nerve pain).

My understanding is that these symptoms you mentioned are NOT attributable to ALS:
pain, abnormal white blood cell count, demylination

Did they check you for Lyme disease?

 

[brian32]

Thank You for your reply. Actually, I am currently being tested for lyme disease & a whole lot of other infections, viruses, diseases to determine exactly what I have. Does lyme disease mimic what I have presented to you?

 

[brian32]

Brian:

I'll keep you in my prayers. That's quite a lot of testing you have had. This is good since they will be able to give you a better picture of what's going on. Apparently, however, they don't think it is ALS or cancer. Keep us posted.

Thank You for your thoughts & prayers. I'll keep you posted on what I find out.

Brian

 

[JohnKelly]

Brian,

My condition has caused doctors to go back and forth between MND, CIDP and other possibilities. I have had all the treatments for CIDP.

 

[AHands]

Does lyme disease mimic what I have presented to you?

Hey, I'm no doctor, just another guy going through something similar. Its my understanding that Lyme is frequently mistaken for ALS. Only about half the people with Lyme report noticing the distinctive lyme ring rash. Lyme is an infection, and does affect WBC and cause pain.

Demylenation would be a symptom of MS, but not ALS, I think.

I've been dealing with this for almost three years--mostly atrophy primarily in the hands. Not much pain--though my muscles ache as though overworked, which i guess they are since they're trying to do the work despite the atrophy. No burning pain.

I guess you know that ALS affects upper (brain-to-spine) and lower (spine-to-muscle) motor nerves (as opposed to MS for example that only affects lower) and that upper motor nerve damage shows up as hyper or spastic reflexes.

I don't know if there are other ways to check for upper motor nerve damage...anybody? I've had a new EMG/NCSs and I don't think they've ever told me that they can / can't see evidence of upper-motor-nerve damage.

 

[Peg B]

Hi Brian,

Just a clarification. When you say your income will be significantly decreased when you go on Short Term disability, are you sure about your understanding. My short/long term (LTD) disability is 60% of my regular pay. That sounds like a lot, but with all of the funds that are taken out of "regular" i.e. 100% pay, and ARE NOT taken out of LTD I took home $100 dollars more a month on the 60%. You may want to check out what this means in your case as you may not have as much worry. Just a thought.

As far a DX there is also MMN, multifocal motor neuropathy, which has similar symptoms all lower and no upper neuron and is also treated with IVIG. My DX is unclear as "Atypical ALS" or MMN both of which means progression is very slow. It is also said that it can look like MMN but be ALS except the upper neuron signs are not seen until "autopsy." :?: I have come to the conclusion that it is best to really deal one day at a time and try my best to only concern myself with a loss of function if and when it occurs. For clarification I have had symptoms since 2002, and DX since Oct 2006. I have been on IVIG since Jan 2007. Best Wishes, Peg

 

[Jamiet]

Brian,

I would say with 99.9999999% certainty, you DON'T have ALS. Congrats to you buddy.

A very specific symptom of ALS is increased reflexes. It's almost a mandatory diagnosed tool. I don't think one PALS here has decreased reflexes. I could be wrong here, but you would pretty much be in the rarest of rarest categories (almost impossible..)......Dec reflexes point to a problem at athe neuromuscular junction (i think), not a motor neuron issue.

Also, the deliminating (sp.) also points away from ALS.

The CIDP diagnosed sounds right diagnosed. It sounds as if you have good docs whom are on the right track. It will take time.

HAts off to you buddy. hang in there, treatement adn feeling better is right around the corner for you.

rgds,

jamie

 

[laurel]

Hi Brian,
Your symptoms are almost identical to my husband's symptoms and he was diagnosed with CIDP--MADSAM Lewis Sumner variant just last Friday. He was first seen in July 2007 at our local neuromuscular disease unit. He had EMG and nerve conduction tests, spinal tap, and blood work. In July he could not hold his coffee cup, use the gear shift in his car, or use eating utensils due to weakness and atrophy in his right hand. About 3 weeks after his first testing at the neuromuscular disease unit, he developed weakness and foot drop of his right foot. In September he received 70 G. daily of IVIG for 4 days. Then he received 70G. for 2 days in Oct. and November. He has had very good response to the IVIG and last Friday when he was given the CIDP diagnosis he was put on Imuran as well. From my reading, response to IVIG varies with individuals. Some have immediate response like my husband did, and others have to try other things such as corticosteroids and Imuran. And some have IVIG and the above combos. My husband's spinal tap showed elevated protein. He paid for the sensory motor blood profile from Athena labs and those tests results were negative. He also had a series of MRI's which were negative other than some arthritis in the neck. He was misdiagnosed with Carpal Tunnel Syndrome and had failed surgery in 2005. We feel blessed to receive the CIDP diagnosis as ALS was queried in the beginning of his diagnosing journey. Good luck.
Laurel

 

[laurel]

Hi Brian,
My husband just read what I posted and wanted me to add that he feels that the "big hit" of IVIG that he began with was critical in getting the positive response that he had. i.e. he had 4 days worth and you just had 2 days. He understands that 4 or 5 days of IVIG is commonly given first time around to elicit a good response. You might want to review that with your doctor. And hubby is now holding his coffee cup and eating with knife and fork again, and his grip is improving. However, he has extensive muscle atrophy in that right hand and it is unlikely that will improve according to the neurologist. Also hubby mentions that his nerve conductions showed his left arm affected as well as right arm and right foot.
And he says yes it happens that people with CIDP don't always respond to IVIG, but he queries whether your lack of response is related to the quantity given.
Laurel

 

[DAK]

Brian,

Who is your doctor at MCG? I've seen Dr Rivner several times with no diagnoses other than possible BFS or periphial nerve neropothy. I have many of the same symptoms you described but I'm progressing very slowly. Is CIDP only diagnosed through spinal fluid?

Dan

 

[laurel]

Hi Dan,
I'm not Brian, but concerning your question about CIDP being diagnosed by spinal tap, the spinal tap is something that is one piece in diagnosing i.e. elevated protein is commonly seen in CIDP. However the nerve conduction tests are a bigger piece in diagnosing in that the nerves show demylelination and various other things that indicate an autoimmune peripheral nerve disease happening. I gather that it is an illness that responds to treatment quite well, but there is often progression through the years and people have flare ups on and off through the course of their life times in which they need frequent monitoring and re-evaluation.
Laurel