ALS with Paraneoplastic LEMS?

[mattb]

My father was recently diagnosed with ALS at 71. His symptoms started one year ago with left drop foot followed by overall declining strength and weight loss (70lbs over the past 12 months). Originally we thought it was a pinched nerves in his spine, then a Statin myopathy. He was hospitalized 6 weeks ago for shortness of breath and difficulty swallowing and breathing when lying down. After a 10 day hospital stay, MRI, CT, CSF, Blood and Nerve Conduction Tests (3rd one to date), and a 5 day regimens of IVig therapy (no noticeable benefit) he was released with a Bip-Pap machine, thick-it and a provisional diagnosis of ALS. This was to be confirmed at a MDA/University ALS multidisciplinary clinic where he was to seek care and ongoing treatment.

4 weeks later we finally got our appointment with the ALS clinic and spent a great deal of time with the pulmonary specialist (my dad is at 50% lung capacity sitting up) and is scheduled to get a G tube radiographicly placed in 10 days. His arm strength is weak, but good. Proximal and distal function seems to be unimpaired. He can still walk with a cane and has an orthopedic device for his drop foot. The Bulbar symptoms are most concerning. He has a weak voice and cough, but no slurred speech. He aspirates thin liquids with every swallow and frequently chokes on phlegm and food. No cognitive impairment, some psusonulbar affect. He is not in pain, often has dry mouth, and has had very little appetite and taste for over 6 months.

Here is the kicker... When we met with the head program neurologist yesterday after a thorough evaluation from the residents assisting we were told that they JUST RECEIVED THE ATHENA LAB RESULTS FROM SOME PREVIOUS BLOODWORK, AND MY FATHER TESTED "POSITIVE" FOR THE LEMS ANTIBODY (Lambert-Eaton Myasthenic Syndrome). She spoke with the Pulmonologist and discussed dads lung scan that a radiologist noted an irregular area, but this was ruled unremarkable by a different Pulmonologist and forgotten about when the IVig treatment was unsuccessful.

My dad is now scheduled for a PEG and after successful PEG placement and inflammation goes down, the neurologist wants to get a PET scan to see what lights up. This could be as long as 4-6 weeks away. I had done a lot of research on every other MND and treatment options as we were progressing to the ALS diagnosis. I asked the neurologist if we could consider doing the PET scan sooner, and begin 3, 4 Dap drug therapy immediately to see if he responds favorably thinking that a LEMS diagnosis could be a blessing versus ALS. Her response was:

"Let's just be clear. I have seen many LEMS patients and you dad does not have LEMs. On the other hand LEMS antibody is not something we see in ALS. You dad meets all criteria for ALS at this point electrophysiologic and clinical. The question that exist is - 'Are we dealing with a paraneoplastic presentation of ALS or not ? In other words is ALS in your dad caused by an underlying cancer? In that the LEMS is a cancer marker in some instances( small cell carcinoma most likey). Therefore we just need to ask ourselves at this point have we searched for cancer diligently.
Unfortunately even when ALS is a paraneoplastic presentation the prognosis is still sadly grim."​

I CANNOT FIND ANY LITERATURE OR CASES WHERE "ALS IS A PARANEOPLASTIC PRESENTATION". It seems to me that my dad either has both, just LEMS, or a false positive LEMS test. Has anyone ever heard of this?

Can anyone on this forum give me some additional insight, opinions or experience with LEMS vs ALS, the Athena LEMS test, and or the 3,4 Dap drug to treat LEMS? What would you do if you were me? My dad is wasting fast.

Thanks for reading the way too long post.

 

[mattb]

My dad had a PET Scan today and it was clean, not cancer, just plain old ALS. What should we make of the positive test for P/Q-type voltage-gated calcium channel antibodies? His doc is throwing it out, says it has no significance.

Does anyone have experience with an ALS diagnosis with positive LEMS antibodies?

 

[Barbie]

so sorry about your dad's diagnosis. I have no idea what the LEMS is, but ALS is ALS, so approach it like that and treat it like that, especially if the doc said it is of no significance.

Hang in there.

 

[old dog]

Fairly early in the diagnostic process, I, too, had extensive bloodwork through Athena. The initial results showed an elevated YO level. The neurologist I was seeing at the time was quite alarmed and told me this could indicate cancer. More blood was drawn, and the test was rerun and came back within the normal range. I have never been able to find out what "YO" (or "Yo") is. When I asked my present neurologist (ALS Clinic) about it, she said I have never had a high YO level; in fact, it was low. I don't know if my file doesn't contain all the reports from Athena or if different people have conflicting interpretations.

Like Barbie, I have no idea what LEM is. The reason I mention the Athena testing is that now, 3.5 years later, I am in treatment for breast cancer. Fortunately, what I have is one of the less aggressive types, and there was no evidence of lymph node involvement. The team of cancer doctors believe this cancer is slow growing and has been present for quite some time--maybe as long as 15/20 years. I'm left wondering if the initial blood test indicating elevated YO was correct.

 

[mattb]

Old dog, thanks for the reply. The Yo antibody is associated with Gynecological cancers.

The following autoantibodies are found in paraneoplastic syndromes6-8:
a) anti-Hu, anti-neuronal nuclear antibody type I (ANNA-1) is associated with Small cell lung cancer resulting in PE
b) anti-Ri, anti-neuronal nuclear antibody type II (ANNA-2) is associated with Neuroblastoma (Children) and Fallopian or Breast cancer (Adults) resulting in POMA
c) anti-Yo , purkinje cell cytoplasmic antibody type I (PCA-1) is associated with certain gynecological cancers resulting in cerebellar degeneration.
Presence of one of these antibodies supports a clinical diagnosis of paraneoplastic syndrome and prompts a search for underlying neoplasm. These markers also help discriminate between true paraneoplastic disorders and other inflammatory disorders of the nervous system that mimic a paraneoplastic syndrome.

 

[old dog]

mattb - Don't know what happened to my post. Thank you for the information. Wishing the best for you and your dad.