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tag0620

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Nov 18, 2008
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175
Diagnosis
11/2008
Country
US
State
ut
City
park city
Hi Everyone!
Sorry I've been absent...but I feel like we're in a "fight" for my husband's quality of life!

We've had so many Dr.'s not willing to look at other things...they are just deferring to that very hasty als diagnosis. We're in a weird "catch 22". We need surgery on this thoracic herniated disc to prove that he doesn't have als, but no one wants to do this very invasive surgery if he has als because it would be deemed un-necessary if he has it.

What I've found on this herniation...it's rare, it's usually caused by trauma (which means he's most likely been walking around with this spinal cord compression for 2 1/2 years!), it most likely could be compressing the anterior spinal artery that runs to the front of the spinal canal between t4/t9 (his herniation is at t6/t7 pressing directly back into the spinal cord, compressing it by at least 1/3), and it can present with upper AND lower motor neuron symptoms (ala als) as well as some other symptoms that he is now presenting with as well (cold legs and feet, purple feet, pins and needles in the left foot, no sensitivity to temperature changes in the left foot/lower leg).

There is a syndrome called "anterior spinal artery syndrome"

"Anterior Spinal Artery

On the anterior surface of the medulla, two branches from the vertebral arteries unite in the midline to form a single anterior spinal artery that descends the length of the spinal cord in the anterior median fissure (Figure 2.10). The sulcal arteries arising from the anterior spinal artery enter the spinal cord through the anterior median fissure. Successive sulcal arteries generally alternate in their distribution to the left and right side of the spinal cord (Figure 2.11), but occasionally a single sulcal artery will distribute to both sides (Figure 2.12). The sulcal arteries supply the anterior two-thirds of the spinal cord at any cross-sectional level. This is anclinically important feature of the anatomy of the spinal cord, because occlusion of the anterior spinal artery or its sulcal branches could result in anterior cord (spinal artery) syndrome (Figure 2.13). As in most vascular problems, the onset of signs and symptoms is rapid. Figure 2.13 shows the zone of distribution of the anterior spinal artery in the cross-hatched area. The posterior funiculus and horns are spared because these areas are supplied by the posterior spinal arteries. Initially, there is flaccid paralysis of the muscles in the body below the level of infarct because of spinal shock. In time, however, spastic paralysis and other upper motor neuron signs develop because of bilateral destruction of the corticospinal tracts. A variable degree of bowel and bladder dysfunction exists because of the interruption of the descending autonomic pathways. Initially, however, incontinence may be due to spinal shock. A cardinal sign of anterior cord syndrome is a dissociated sensory loss characterized by a loss of pain and temperature sensations (bilateral lateral spinothalamic tract lesion) with preservation of kinesthesia and discriminative touch sensations (sparing of posterior funiculi) in the body below the level of injury. Some patients develop painful dysesthesias about 6 to 8 months after the onset of neurologic symptoms. The source of this pain is unknown, but has been suggested to be attributed to the activation of previously latent pathways that mediate pain sensation. The anterior spinal artery is dependent on segmental contributions from anterior radicular arteries along the length of the spinal cord."


Here is a letter that our GP just wrote for us trying to get us the help we need on this thoracic injury...

"To Whom It May Concern,
My patient "J", has had bilateral lower extremity weakness, left greater than right, with associated muscle atrophy, that was intially progressive over months starting in Feb 2007. Since that time he has had waxing and waning weakness, and has had an impressive return of strength and muscle mass while taking a course of anabolic steroids. Mr. "J" has also developed slowly progressive bilateral, left greater than right lower leg parathesias including pain and coldness that is affected by body position. He has not at any time complained of upper extremity symptoms nor has he observed upper extremity muscle wasting.

Mr. "J"'s past medical history is only significant for a lumber spine fracture from a ski accident in Dec '06 with subsequent surgical fixation in Apr '08.

Mr. "J" has had a complete workup for his symptoms including a normal brain MRI, normal lp, normal labs except for positive antibodies for Rocky Mountain Spotted Fever for which he was given antibiotics, and a thoracic spine MRI which showed a large anterior disc protrusion which impinges on his spinal cord.

Mr. "J" was diagnosed with als after a brief meeting with a neurologist and since that diagnosis he has been unable to find anyone who will consider any other possible cause for his lower extremity weakness. Given the atypical symptoms and response to treatment, along with the presence of a significant thoracic disk protrusion, further workup as well as surgery to correct the spinal cord impingement is certainly warranted.

Thank you.

Sincerely,
Dr. R"


So...anyone have a take on this new info? Anyone think this letter from a "lowly" (haha) family practitioner will make any difference to a neurologist or specialized neuro surgeon?

AAARRRGGGHHH!

Hope everyone is doing ok!

Tracy
 
Tracy

Is there a any kind of test to check and see if this is what's going on?

Have you tried contacting a large facitilty Like Mayo or John Hopkins?

When you say he has no sensitivity to temperature changes in the left foot/lower leg. He can't tell the difference between hot or cold?
That is something that usually doesn't happen in ALS.
 
Hi Crystal,
We don't know..from what I can tell..normally, if you have this type of herniation and are presenting with neurological symptoms..it calls for immediate surgery. BUT..this is where our catch 22 comes into play and how dangerous a hasty als diagnosis can be to people.

One of the neuro's that we've seen did a test with a stick that had a metal tip and an identical non-metal tip. He had my husband close his eyes and then asked him which end of the stick felt colder to him. He was guessing the metal tip consistantly on his right leg, but then got them mixed up on his right foot, and couldn't tell the difference on his left leg/foot. This is also presenting with purple feet, cold feet and legs (more pronounced on the left than the right), and pins and needles in his left foot.

I asked for a full body mapping angiogram to check his arterial system and was told no...I might keep pushing this but don't know if it would be the best test.

We have another appt on Fri with ANOTHER new neuro...but again, he's down at the U with the dr. who diagnosed him with als. The difference is that this neuro called us. He heard about our case from his accountant who is also our accountant (haha) and said he isn't afraid to challenge a collegue. In fact, he scheduled us in at 5pm on Fri when all of the other dr.s are gone for the weekend since we are uncomfortable there. He has a backround with Johns Hopkins...and even asked my husband if he had any thoracic injuries going on...which makes us think MAYBE he knows about this.

I found a thoracic specialty micro-neurosurgeon in AZ, but he's going on a year long sabatical and is not taking new patients....but....the dr. who trained him is actually here in UT at the U in their spinal neurosurgery center...so we're hoping we may be on the right track.

It's just so frustrating that WE'VE had to come up with all of this and try to find our way to help. We have our family physician who seems to be listening..but that's it..and he's very limited in what he can do...so he's just documenting what he sees for us.
 
Tracy,

For a nuero to contact you and give you an appointment after hours he sounds very interested in seeing the case
especially saying he is not afraid of callenging a collegue. Good luck with the appointment!

Did you get a second opinion on the ALS Dx from another ALS specialist? Especially with all of this other stuff going on.
 
No..the Dr. that diagnosed this is "the" als specialist in our state. We've had two other neuro's and an orthopede all sypathetically say they understood our desire to look further...but als just makes sense and won't do some of the testing that we've requested...it really really comes across to both of us like half hearted pity and it's so frustrating.

We went to an orthotics place to get a foot brace for my husbands left foot drop...and the prescription came over from the orthopede's office saying that my husband had als and to expidite it. We ended up paying $750.00 out of pocket for the damned brace so THAT didn't show up on our insurance rendering what we were trying to prove as un-necessary and thus uninsurable.

Our next stop if this new neuro doesn't pan out will be the Mayo...but the longer we have to wait to get this herniation fixed, the more damage it's likely to do (we're worried about the bowel, bladder, and sexual functions as well as complete lower body paralysis).
 
Tracy,

I think I've said this before, but I agree with Crystal. I think a lot of these doctors are falling back to Dr. B.'s diagnosed since he is the Utah ALS expert. Its good that you have persuaded one U of U neuro to challenge him, but if its within your ability to go somewhere else I think you should. Not starting again, but to have the ALS diagnosed weighed in on.

Although a place like the Mayo thrives on mystery cases, you may even benefit hopping one state over and having a NMD specialist at UC Denver take a look.

As always, hoping for the best,

Robert
 
Just a follow up. The reason I feel seeing a second NMD/ALS expert is critical, is imagine a letter from them saying essentially what your GP, that the intial diagnosed was hasty. Even if it is not definitive, it might open the door to some for someone to explore other options.

Robert
 
Thanks Robert!
That's why we were thinking Mayo...they can perform tests for multiple departments and confer with multiple specialists all at once without the "pass around" that we've been experiencing. Does this make sense? Would this be the most logical if we need it?
 
I think so, though I think there are a variety of options out there. I think whatever option you choose needs to involve an ALS/NMD specialist specifically reviewing the diagnosed.

I was actually surprised at how quickly you can get in to the Mayo. It's much shorter than a couple of the other specialists I've seen. You might be able to get some feedback from other members who have gone there. From what I understand based on my prep work, and my brother's experience, the Mayo reviews all medical data to date, then dives in looking at all the alternatives. My neuro, who has had a handful of his cases go there, called it the "shotgun" approach. He was expecting repeats of all the imaging and EMGs at the very least.

Normally they assign you to a more general case manager, but given your husband already has an ALS diagnosis, I'm sure they would work in a consult with one of their NMD experts early on as part of the visit.

Take care,

Robert
 
Tracy,

Absolutely get a referral to Mayo! .... I'm sitting here just shaking my head as I read what you've written. I agree that valuable time could be slipping by. Just ask Erica (perplexed) how she feels about cancer being overlooked with her husband, and him missing earlier treatment, because of a specialist insisting it was ALS.

And ~ just me with no medical training, and less knowledge than many on this forum~ I do not remember reading one single person, diagnosed (or not, actually) who has talked about losing their sensory perception for cold. That he had any problems detecting this has to be significant.

You guys fight for more opinions, and know you've got your friends here pulling for you. (BTW, I read in another one of your posts how you twitch after exercise now, Don does the same thing, and he didn't use to, stress is just awful on everyone, isn't it!)
 
Thanks Rose!
We don't even know if that lack of feeling temperature differences and the purple foot were noted on the clinical exam report because we've requested that one twice and have never received copies or calls back about it...but I was in there during the exam...so I know what happened and I do know that that's a significant event.

We are fighting...my husband jokes that he married a "lion" or calls me "mama bear" because I'm so protective, tenatious (he says), and persistant. The Dr.'s have to be getting tired of me huh? Oh well!

I appreciate everyone pulling for us...I just need to figure out which direction to go next!
 
Tracy,

I can not say anything better than the others have already offered; all of you are such great advocates for yourselves and your loved ones. I can't get over how bad I am at this, and reading your latest Tracy provides more proof about what one can accomplish when you have that special something (gumption? balls?)

I am very excited about that neuro contacting you! I can not wait to hear what happens. And the insight and sensitivity to schedule you at 5 on a Friday. Gotta love him already! Just maybe...the visit with him will be enough and you won't have to leave the state and go through all that extra effort. Crossing my fingers for you both!

Lydia
 
I'm new to this forum and I hope you don't mind the reply. Are you looking at the Mayo in Rochester, MN or Scottsdale, AZ? My neuro experience in Rochester was amazing. They move along at an extremely agressive pace and leave no stone unturned. You will go from a GP to a neuro consult and then directly to labs, imaging and so forth. Your appointments are scheduled in the best possible manner to suit your stay. My best friend has also doctored there for the last three years with a Stage 3 Astrocytoma (malignant brain tumor). She is stable and credits everyone in the neuro department and her cancer team with how well she is doing. You will get your answers there, I just know it! Good luck to you and your husband.

Stacy
 
I think your GP's letter will make some difference. It lends credibility to your concerns. Good luck Tracy, and keep being the mama bear.

I just got hold of all of my husband's medical consultations over the past 3 years. It was tedious due to our Freedom of Information laws and having to sign releases etc. But what an eye opener as I waded through his reports. It proved to me what an inexact science neurology is. He is had a plethora of "guesses" from 4 different doctors. We started with Carpal Tunnel (with surgery being done needlessly), we moved onto Progressive Muscular Atrophy, then Monomelic Atrophy, maybe some sort Anterior Horn Cell Disease, then to a differential of ALS, differential of MMN, then to CIDP MADSAM with a proviso of it being an unusual variant of an unusual variant(sheesh)and that he would continue to be monitored for any developing upper motor neuron symptoms. I find that I am still shaking my head over what I read--much of it had never be verbalized to us. Actually I feel like my faith in the whole diagnostic system with neuromuscular disorders is shaken. If we get anymore guessing, I think we will be aiming for the Mayo too. But we are lucky in that he is continuing to respond to IVIG.
Laurel
 
Tracy,

Mama bear, you tell him he is one lucky guy to have a wife being so persistant. (I'm sure he knows it already)
Who cares if the drs get tired of you!
 
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