BethU
Extremely helpful member
- Joined
- May 11, 2008
- Messages
- 2,646
- Reason
- PALS
- Diagnosis
- 05/2008
- Country
- US
- State
- California
- City
- Los Angeles
Sorry ... this is going to be a long one.
As I’ve reported here ad nauseum, my bulbar symptoms started with both slurred speech and blurry vision, sometimes double vision. It was the double vision among other things that led to my mis-diagnosis with myasthenia gravis before I was diagnosed with ALS.
In the last couple of weeks, I’ve been experiencing “ptosis,” a drooping eyelid. It is another “signature symptom” (my term) of MG. Mine does not seem to occur the way it does in MG, where the eyelid is too weak to hold itself open. Mine is a twitch that lowers my eyelid almost closed, and sometimes seems to forceably hold it down. Also, in the last couple of weeks my blurred vision and double distance vision have started up again. My vision had been stable for several months, making me think it was safe to get new glasses. Apparently it wasn’t!
There is no doubt about my ALS, but I have these ocular symptoms left over. They don’t fit in with ALS. So of course, knowing that Wright encourages us all to consult Google whenever possible ), I Googled “ALS ptosis,” and came up with a paper on the subject.
It was a report about “two old women” (one a couple years younger than me :-( ) who had bulbar-onset ALS and also ptosis and diplopia (double vision), which the neurologists who wrote the paper felt were due to MG. In other words, they felt these women had both conditions and that the conditions were somehow related (if I am understanding the use of the word “association” correctly). The difference between the women in the case study and me is that their bulbar onset ALS progressed very quickly, while mine has been slow so far, knock wood, fingers crossed, from my keyboard to God’s ear..
________________________________________________
Here are two interesting quotes from the report.
Finsterer described that 13.6% of patients initially diagnosed as ALS had mitochondrial myopathy [12]. This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy. Our cases, however, were characterized by a rapid [ALS] progression rate, which precluded the performance of muscle biopsy. We believe that both our patients suffered from an association of ALS with ocular MG.
(Some lines are missing from the articles.)
[I]Several reports elsewhere have described the association of autoimmune disorders with ALS [16]. MG is probably underdiagnosed in old people [17]. Both our patients were old women with bulbar-onset ALS and rapid progression, which might represent a particular phenotype prone to this association. A chance association would be very improbable (based on the incidence of the two diseases the estimation for a chance occurrence would be 1/6 ×1018, for 2 patents). Thus, although the association between ALS and MG may be exceptional, we speculate that some cases have been missed. It is a possibility that the neurologist should be aware of.[/I]
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What this does for me is convince me that maybe I’m not entirely crazy (yet), and my ocular symptoms do have some significance ... but I also was struck by the report that ALS is sometimes associated with (other) autoimmune diseases, which I know is often a subject of discussion among PALS. Also, I’m baffled by the assertion that 13% of diagnosed ALS patients had mitochrondial neuopathy. I wonder, is that instead of ALS, in addition to ALS, or what?
This sentence is interesting, too: This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy.
I don’t know what lactate stress testing and muscle biopsy would prove (MG?), and I can’t see myself wandering into the ALS clinic with this report tucked under my arm and saying “looky here, doc, what I found on the Internet.” I still have not been able to communicate to any neuro that in 3 out of 4 trys, Mestinon greatly improved my physical strength. In fact, my UCLA neuro's report of our first interview wrongly states that Mestinon had no effect. I let it slide because these unaccounted-for symptoms were always just shrugged off.
But, for what it’s worth, I thought the article was interesting, and might be pertinent to others on the forum who seem to have cross-over symptoms with MG. Even if they're not the proverbial "old woman.":roll:
--------------------------------------------------------------------------------------------
Here is the citation for the article:
Clinical Neurology and Neurosurgery 110 (2008) 168–170
Case report
Amyotrophic lateral sclerosis patients and ocular ptosis
Susana Pintoa, Mamede de Carvalhoa,b,*
a
Neuromuscular Unit, Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, Portugal
b
Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal
Received 1 June 2007; received in revised form 24 August 2007; accepted 27 August 2007
Abstract
Ptosis is not a feature observed in amyotrophic lateral sclerosis (ALS). We describe two old women with bulbar-onset ALS and rapid progression, in whom ptosis and diplopia were noted. They did not improve on pyridostigmine or steroids. Antibodies against acetylcholine receptor were negative, thymoma was excluded, but neurophysiological showed marked neuromuscular transmission failure in orbicularis oculi. We discuss the association between ALS and Ocular Myasthenia Gravis in these cases.
As I’ve reported here ad nauseum, my bulbar symptoms started with both slurred speech and blurry vision, sometimes double vision. It was the double vision among other things that led to my mis-diagnosis with myasthenia gravis before I was diagnosed with ALS.
In the last couple of weeks, I’ve been experiencing “ptosis,” a drooping eyelid. It is another “signature symptom” (my term) of MG. Mine does not seem to occur the way it does in MG, where the eyelid is too weak to hold itself open. Mine is a twitch that lowers my eyelid almost closed, and sometimes seems to forceably hold it down. Also, in the last couple of weeks my blurred vision and double distance vision have started up again. My vision had been stable for several months, making me think it was safe to get new glasses. Apparently it wasn’t!
There is no doubt about my ALS, but I have these ocular symptoms left over. They don’t fit in with ALS. So of course, knowing that Wright encourages us all to consult Google whenever possible ), I Googled “ALS ptosis,” and came up with a paper on the subject.
It was a report about “two old women” (one a couple years younger than me :-( ) who had bulbar-onset ALS and also ptosis and diplopia (double vision), which the neurologists who wrote the paper felt were due to MG. In other words, they felt these women had both conditions and that the conditions were somehow related (if I am understanding the use of the word “association” correctly). The difference between the women in the case study and me is that their bulbar onset ALS progressed very quickly, while mine has been slow so far, knock wood, fingers crossed, from my keyboard to God’s ear..
________________________________________________
Here are two interesting quotes from the report.
Finsterer described that 13.6% of patients initially diagnosed as ALS had mitochondrial myopathy [12]. This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy. Our cases, however, were characterized by a rapid [ALS] progression rate, which precluded the performance of muscle biopsy. We believe that both our patients suffered from an association of ALS with ocular MG.
(Some lines are missing from the articles.)
[I]Several reports elsewhere have described the association of autoimmune disorders with ALS [16]. MG is probably underdiagnosed in old people [17]. Both our patients were old women with bulbar-onset ALS and rapid progression, which might represent a particular phenotype prone to this association. A chance association would be very improbable (based on the incidence of the two diseases the estimation for a chance occurrence would be 1/6 ×1018, for 2 patents). Thus, although the association between ALS and MG may be exceptional, we speculate that some cases have been missed. It is a possibility that the neurologist should be aware of.[/I]
--------------------------------------------------------------------------------------------------------
What this does for me is convince me that maybe I’m not entirely crazy (yet), and my ocular symptoms do have some significance ... but I also was struck by the report that ALS is sometimes associated with (other) autoimmune diseases, which I know is often a subject of discussion among PALS. Also, I’m baffled by the assertion that 13% of diagnosed ALS patients had mitochrondial neuopathy. I wonder, is that instead of ALS, in addition to ALS, or what?
This sentence is interesting, too: This author suggests that patients with slow disease course and multi-organ involvement should undergo lactate stress testing and muscle biopsy.
I don’t know what lactate stress testing and muscle biopsy would prove (MG?), and I can’t see myself wandering into the ALS clinic with this report tucked under my arm and saying “looky here, doc, what I found on the Internet.” I still have not been able to communicate to any neuro that in 3 out of 4 trys, Mestinon greatly improved my physical strength. In fact, my UCLA neuro's report of our first interview wrongly states that Mestinon had no effect. I let it slide because these unaccounted-for symptoms were always just shrugged off.
But, for what it’s worth, I thought the article was interesting, and might be pertinent to others on the forum who seem to have cross-over symptoms with MG. Even if they're not the proverbial "old woman.":roll:
--------------------------------------------------------------------------------------------
Here is the citation for the article:
Clinical Neurology and Neurosurgery 110 (2008) 168–170
Case report
Amyotrophic lateral sclerosis patients and ocular ptosis
Susana Pintoa, Mamede de Carvalhoa,b,*
a
Neuromuscular Unit, Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, Portugal
b
Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal
Received 1 June 2007; received in revised form 24 August 2007; accepted 27 August 2007
Abstract
Ptosis is not a feature observed in amyotrophic lateral sclerosis (ALS). We describe two old women with bulbar-onset ALS and rapid progression, in whom ptosis and diplopia were noted. They did not improve on pyridostigmine or steroids. Antibodies against acetylcholine receptor were negative, thymoma was excluded, but neurophysiological showed marked neuromuscular transmission failure in orbicularis oculi. We discuss the association between ALS and Ocular Myasthenia Gravis in these cases.