ALS as a (possibly treatable) syndrome

[Searcher]

Note: posted this in response to another thread, but thought some who might be interested might not see it there, so re-posting it as a new thread.

Searcher

Interesting article mentioning treatable "ALS"

Dug up old ALS articles, including one titled "The Syndromic Nature of Amyotrophic Lateral Sclerosis" by D.r B.H. Patten at Baylor College. It is a discussion of 10 patients diagnosed with ALS who Dr. Patten and colleagues present as examples of varying conditions causing or masquerading as ALS, some treatable.

Among the case histories are two patients with suggestions of MG. One was treated successfully with mestinon, prednisone, cytoxan and thymectomy. Patten concludes that he had "an autoimmune disease with features of MG and ALS" and that "with aggressive treatment of the myasthena, the ALS syndrome disappeared. Therefore the patient probably had an ALS syndrome of autoimmune origin."

He presents a second case which had suggestions of MG findings. Treatment with Mestinon and Predinisone failed, but subsequent treatment with Cytoxan and thymectomy resulted in progressive improvement and he became essentially "normal." Patten concludes that the patient probably had a combination of motor neuron disease associated with myasthena. Both conditions responded to intensive and prolonged treatment."

This article is really something to think about, for several reasons, including Patten's main point that ALS is a syndrome, which diverse, sometimes treatable conditions can cause or mimic. If you can't find it and are interested, I could fax it or something.
This article is from the late 80's, I believe. I also wonder whether most neurologists - even ALS specialists - spend the kind of time and resources that Patten seemed to have spent searching for treatable causes of ALS, and of trying to treat these. I'd bet that there are more constraints of time and money for most neuros to look this carefully. I hope I'm wrong about this.

 

[rose]

That school of thinking, or approach ~ however you want to word it, is still out there. In my own experience it was not so much of there being an ALS syndrome, but rather a trial to see if I would respond to high dosage of prednisone. If I had, my doctors felt that no matter the name placed on it, I had a treatable inflammatory condition, and I would have begun I.V. treatments of Rituxan. However, I had no positive response to the prednisone. and it was felt that any more meds or trials of different meds had risks that outweighed any possible benefit.

 

[Searcher]

How long did you have bulbar symptoms before you were diagnosed? Did they tend to steadily progress or fluctuate?

There are a lot of things which can mimic ALS. It stands to reason, therefore, that there are things which a) not all neurologists actually test for, among the known things which might mimic ALS and b) there are things yet discovered which can mimic ALS. For example, it is not crazy to think that many, maybe most, autoimmune diseases can mimic ALS or maybe even cause it in many cases.

Thanks,

Searcher

 

[rose]

About 20-21 months from first problems with voice until told it was bulbar palsy.

I had some other non specific issues that pre-dated that time by a few more months.

I was under the constant care of an excellent rheumatologist for about 18 of those months. I had second opinions by two other top rheumatologists in the nation, one of which is the chief of rheumatology at Johns Hopkins. They are satisfied that there is no basis for an inflammatory cause in my particular case.

My symptoms have been progressive, they level off at times, but I've not ever had improvement. There has not been fluctuation, other than some days I feel better than others. Good day, bad day kind of thing. I was also on two other immunosuppressants for about a year, Plaquenil and Methotrexate.

I've written about this several times on this forum, if you're interested in more of the details, you could possibly do a search. I just can't write it all out again!

I hope this helped to answer some of your questions.

take care

 

[Searcher]

Rose,

Thank you for your response. Sorry to make you repeat yourself. One last question - what were you being followed for by a Rheumatologist and (well, a second question) why were you tried on Plaquenil? I ask the latter because new Rheumie wants to try me on Plaquenil for antiphospholipid antibodies. She (an APS specialist) says that APS can cause muscle problems, but won't say whether it might cause or mimic ALS.

Searcher

 

[rose]

Hi Searcher,
I took Plaquenil for suspected Sjögren's syndrome, as I have dry eye and dry mouth. I did not have elevated ANA's.

The Plaquenil had no effect on my dryness or other problems, with the exception that it helped with the muscle cramps (it is a quinine drug).

take care,

 

[Searcher]

Rose,

In the article by Dr. Patten I mentioned he gives a case of Sjogren's simulating ALS. The person had complete remission twice after administration of iv Cytoxan and methylprednisolone. He cites this case as an example of how Sjogren's (among other things) can simulate ALS and mentions two other cases that responded to treatment of those diagnosed with ALS who complained of dry mouth or dry eyes. He recommends a tear test and the rose bengal test for any ALS patient who complains of dry eyes. If those tests are positive, he recommends a minor salivary gland biopsy to prove the Sjogren's diagnosed "followed by intensive treatment of the autoimmune condition."

I wonder how many ALS docs go to these lengths and remain as open and on the lookout for findings that might suggest something treatable?

Searcher

 

[rose]

Searcher,

I'd mentioned in a previous post in this thread that I'd given a detailed account more than a couple of times concerning my journey. You can use the "Search" function to find the previous discussions on the forum about this.

I actually believe that most doctors that specialize in ALS would take the measures that you listed. My experience of being bounced back and forth between the two specialties (rheumatology & neurology) is not that unique.

It is true though that I had the benefit of going to top doctors in both fields.

I did have a salivary gland biopsy. In fact I had two. One showed chronic minor focal inflammation not diagnostic of Sjögren's, the other showed no inflammation. The rheumatologist at Hopkins explained that everyone, symptomatic or not, will most likely have some salivary glands with minimal inflammation, and that they know this by studying cadavers.

In reference to the dryness, I did have the Schirmer Test for dry eye, and my eyes are extremely dry, more so than mouth. The caveat is they feel the dryness would have been more extensive throughout my body, before Sjögren's would have infiltrated my nervous system. My dryness is limited to only the eyes and mouth/throat - perhaps lungs, my nose isn't even dry.

None of the medications tried had any positive effect, and there is risk involved that a doctor needs to be able to justify before continuing to try stronger and stronger drugs with even more serious possible side effects. Due to that "intensive treatment of the autoimmune condition" I developed lesions on the retina of my eye from the Prednisone use, for example.

Because I have many other manifestations of problems with my 7th cranial nerve (responsible for tear production ,and in part for saliva production); it is their feeling that in my case, my nerve involvement is not limited to only motor function, however it is predominantly motor function. It seems to be mostly Central 7th (UMN) I also had dry eye before dry mouth, which is also not typical of Sjögren's.

And, before you ask (LOL) no, my problems are not just with the 7th cranial nerve. There are problems with 9th, 10th and 12 for certain. I really don't know what the 11th does off the top of my head, and for all I know #11 isn't a happy camper either.

There are some problems with my limbs at this point.

I have to tell you that since reading your posts, and seeing your location, that I've wondered why you've not gone to Johns Hopkins? I have a son in Gaithersburg and its not even an hour's drive for him when he goes with me to appointments. Its certainly a much closer drive than I have to get there. They are rated the best in the country, and I've been very impressed with them.

take care,

 

[Searcher]

Rose,

Seems like you have a complicated situation, and now I see why they tried to treat for autoimmune. Ever had a thymectomy recommended? Always have wondered about the business about doctor's not wanting to incur added risks by trying other treatments with possible side effects. Think this should be up to the patient, since the alternative is no better than any of these side effects, as far as I can see.

Re. Hopkins, who have you seen there in Neurology? I have gone to Hopkins before, followed for some years by Dr. Hurko (no longer there) and once or twice by Dr. Corse. I would like to go back there and specifically would like to see Dr. Chaudry, since he specializes in MMN. You now need a referral, though, and I don't have one. Think I'll work on this though if Plaquenil doesn't help and things get worse.


Searcher

 

[wright]

Searcher

You must be careful not to overstep your bounds when making correlations between autoimmune conditions causing ALS. There is absolutely no correlation between the two and the mere speculation on here can cause a lot of people to needlessly panic.

Whenever there is aggressive treatment for things "autoimmune" (e.g. MMN or CIDP) it is to see if the presenting symptoms are due to autoantibodies and not to ALS.

There is no known "treatable" form of ALS.

 

[Searcher]

Wright,

I take your point, and think I said as much. Patten's point, though, was that if an autoimmune condition can be found (I think even just the associated antibodies) that treatment should be aimed at any such conditions to see if the ALS or ALS-like condition could be improved or reversed. Perhaps this is what is usually done these days when someone is diagnosed with ALS; I don't know. I hope so.

Searcher

 

[wright]

That is exactly what is done before there is a diagnosis of ALS. Every and all avenues are pursued, which is precisely why it takes some a long time to be diagnosed. The signs and symptoms of ALS are not hard to see and find . . . but there are many things that can mimic it, so it takes time to investigate all of those possibilities. Neuro's are very thorough, especially when it comes to diagnosing something like ALS.