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Could really use your insight and direction...

Knowing the ravages of ALS, I would much rather be diagnosed with Myasthenia Gravis any day (even though that DX is terrible but it is at least somewhat manageable compared to ALS). I had to go into the ER today with difficulty breathing and swallowing (I mean this is the worst it has ever been and I walk on water to avoid the ER). The neuro who tended to me believes I have symptoms of motor neuron damage based on my global muscle weakness, bulbar symptoms, and widespread 24/7 muscle fasciculations (normal MRI, EMG, NCV, abnormal neuro testing). This has been going on for two years, but just flared bad 3 weeks ago. Do people with ALS even have flares and then recover...not recover as in "wow what a miracle you are healed!" No, by recover I mean do they return to where they were at before the flare or even just a bit worse before the flare? Or is it 100%, one you lose that negative ground, flare or not, there is not getting it back?

Also, do ALS patients have hours of the day where symptoms are not as bad? FOr me, the late evening hours (7-9 typically), I show some improvement. But mornings, noon, early afternoon... always a huge bear for me. I have been so week I have been on bedrest over a month by now.

To help you get a better understanding of what has been going on with me, here is a pretty detailed medical history.

REALLY APPRECIATE YOUR REMARKS AND THOUGHTS. it may be 6 weeks before I get in to see the neuro, and I don't know if I can make it that long without being intubated (if things keep progressing as they have).

Medical History:

Profound muscle weakness – started in my legs and hands almost two years ago (August of 2009), it has spread to my entire body by now. Early on, I would have difficulty clearing the steps with my feet/shoe tips walking up stairs and trip incessantly – additionally, I began dropping things, even when I tried not to (I initially thought nothing of it until I realized something was unmistakably wrong as I began to drop things all the time). The muscle weakness quickly spread to my legs, then to my upper torso, neck, everywhere; there was a point where I could barely hold up my head or get out of bed on my own in the morning. Then I noticed something strange looking in the mirror during the course of this flare – my right eye was halfway shut and I could not get it to stay up. This flare happened in August of 2009 but the months preceding this flare there were subtle symptoms in spades that would come and go. As the months went by, my body seemed to adapt to these limitations, but I never recovered (although I did experience a brief period of a few weeks where my symptoms, while not gone, were minimized to a degree). The muscle weakness greatly affects my ability to chew/swallow (especially fluids), my ability to breath, my ability to speak (my voice has become very weak, monotonous, shaky). My facial expressions have become limited (lips and cheeks sometimes quiver when trying to smile). Walking has become increasingly difficult, most especially up/down stairs. Bending over has become extremely difficult, as has lifting. Any movement requires great effort. Resulting loss of muscle mass. Profound fatigue/exhaustion (chronic). I have been to the ER twice with difficulty breathing and swallowing (and dismissed without a diagnosis other than “it’s probably related to your autoimmune illness”).

Muscle twitches – incessant muscle twitching (fasciculations), all over, ranging from very strong to moderately weak. Thumbs and forefingers twitch as well.

Difficulty breathing – made inordinately worse lying down flat on my back. I can breathe easiest lying down on my side; the moment I switch over to my back, breathing becomes very labored and I cannot exhale or inhale fully. My diaphragm feels completely spent, as do all of my abdominal muscles. Have been close on two occasions to having my wife take me to the ER. In both instances, I was insistent on waiting for things to improve (which they did after approximately 30 minutes).

Difficulty Swallowing – has gotten dramatically worse in the past two weeks (liquids are the hardest to swallow, pills are a close second as my throat has a tendency to close on them – I have never had a problem with this until just the past three months).oss of

loss of dexterity/coordination (this is particularly problematic as my profession, not to mention everyday living, requires the constant use of my hands).

Severe headaches that don’t go away (feeling of tremendous pressure inside my head) – I have had these headaches for almost 3 months now. Ibuprofen, which normally works on normal headaches, seems to have little or no effect on these headaches. Possibly CO2 headaches from incomplete exhalation?
Involuntary muscle jerks (myoclonus)
Severe muscle cramps (chronic and severe at times)
Feeling of electricity/vibration in my limbs – a few times throughout the day (most especially legs/feet, hands; arms more increasingly as of late as my arm and hand strength have greatly been reduced).
Medical history:
• Autoimmune condition (Post Viral CFIDS - chronic fatigue immuno dysfunction syndrome), diagnosed in April of 2007 (several years after initial onset which was in August of 2002, after an acute viral infection)
• Hypothyroidism
• Allodynia/central sensitization (upper torso, in consequence of CFIDS, being treated by Dr. Irving of Swedish, Seattle)
• Numerous tender points throughout body (mostly around connective tissue, some muscle tissue – these are not the tender points associated with fibromyalgia)
Things that have been partially but not completely ruled out thus far…
• MS – 3 clean MRIs including a 3T MRI (care of Dr. James Bowen of Swedish Medical, Seattle)
• Myasthenia Gravis (only partially ruled out through negative anti-AChR antibody testing, negative EMG, negative NCV). Would like to have a Tensilon test administered to definitively rule MG out (20-30% of all MG patients are seronegative and pass the aforementioned tests, MG is only detectable in these cases with a Tensilon test).
• CIDP (negative EMG and NCV)
• MMN (negative EMG and NCV)
• Sarcoidosis (normal lung x-ray)
• Borreliosis (ELISA was negative, still need to run Western Blot as ELISA has been proven to be unreliable, quite often missing a large percentage of positive Borrelia cases)
Additional related info:
• Normal chest X-ray
• Elevated liver enzymes (chronically elevated since acute viral infection that caused CFIDS)
• Elevated myoglobin levels in latest blood test
• Chronically elevated white blood cell count
Tests that still have yet to be performed…
Tensilon Test – My PHP would like this test to be performed to rule out seronegative myasthenia gravis
Spinal tap and analysis of CSF
Muscle tissue biopsy (to rule out Mitochondrial Myopathy)
Nerve tissue biopsy
TH1/TH2 assay
Heavy metal toxicity testing
Genetic testing (to rule out Kennedy’s disease)
Correct me if I'm wrong, but you stated you had a normal MRI, EMG & NCV. If you have lower motor neuron damage, it would show up on the EMG. I'm a little confused.
Not being a doctor at all, but global weakness and ptosis (dropping an eyelid) are hallmark of MG. However, fasciculations are not common with MG, but as Zaphoon said, if they were caused by lower motor neuron damage, EMG would pick them up.
Normally, if you present to an ER with shortness of breath, they do several tests--including blood gasses. These levels should have shown if your body isn't being properly oxygenated. With the SOB you're describing, I'd have thought a pulmonary function test would have also been done.

You mention 'tender points' but say they're not the fibromyalgia tender points. How do you know this? Has a rheumy or neuro already ruled it out? Pain and the exhaustion are hallmark for fibro.

Clear MRIs doesn't mean MS is off the table. There are other tests--including CSF and perhaps evoked potentials that would be done to totally rule it out. Even then--you can test 100% negative today for MS and still by symptomatic with lesions showing up later.

Clear EMG/NCV mean a lot of things are NOT on the table--including ALS. With two years of symptoms--SOMETHING would have shown on EMG/NCV

I'd suggest a good rheumy and a good neuro -- they'll get you sorted out. But--despite what an ER doc said--MND doesn't come and go. It's insidiously progressive and debilitating.

In MG, the symptoms usually get 'worse' when you exert your muscles--and get better with rest.

I'm assuming they've ruled out Gullian-Barre?

Don't take it wrong--but are you sure the breathing issues aren't anxiety driven in some way? Shortness of breath is never treated lightly in an ER--and testing confirms the issue--usually requiring admission unless you have asthma that a simple breathing treatment abates.

At any rate--I wish you luck finding an effective treatment.
You should have the tensilon test......insist on it. It doesn't really sound like ALS, but you need to see an ALS specialist to rule it out. ALS does not flare with any recovery, once ground is lost it does not return. You can get muscle loss from lack of use, which could well be what's happening.
You need to talk to your neurologist and get him to answer your worries. Go with a list and make sure everything is answered. It sounds like you have all sorts of complicated symptoms, get it sorted :)
Your symptoms don't sound much like MND to me. The normal EMGs rule out LMN problems and you don't seem to be reporting UMN problems other than muscle cramps. Plus, the multifocal onset and lack of progression over a two year period are not typical of MND. Additionally, all the sensory and pain issues you are having point away from MND as well.

You also don't mention any results of neuro evaluations over the past two years or having been under regular or followup neurological care. You've had neuro testing (EMG, NCV, etc.) -- what did those neuros say? You also mention "abnormal neuro testing" today. Abnormal how? What specifically was abnormal?

At this point, your story is much too unfocused for anyone to make enough sense of it to give you any advice other than "Get thee to a neuro." Here are the ALSA and MDA recommended neuromuscular centers in your area. Hopefully, you can get referred to one.

Virginia Mason Medical Center Neurology Department
An ALS Association Certified Center of Excellence
1100 9th Avenue
P.O. Box 900, X7-NEU
Seattle, WA 98111
Medical Director - John Ravits, MD
Phone: 206-341-0420
Fax: 206-625-7240

(206) 598-4590
(206) 598-2813 FAX
E-mail: gtcarter -at- attbi -dot- com or gtcarter -at- u -dot- washington -dot- edu
Greg Carter, M.D., Co-director
Department of Rehabilitation Medicine
(206) 598-4211
(206) 598-4102 FAX
E-mail: mdweiss -at- u -dot- washington -dot- edu
Michael Weiss, M.D., Co-director
Department of Neurology/Electromyography
1959 NE Pacific
Seattle, WA 98195-6115
Dang, Allen, you sure did go to a lot of trouble with the -dot- com stuff! LOL

I agree with trfogey.

I wouldn't trust an ER doc to suggest MND, AT ALL! In fact, I think that was very irresponsible to do so.
Dang, Allen, you sure did go to a lot of trouble with the -dot- com stuff! LOL

Yep, wasted effort trying to deal with the eccentric moderation policy. Try to save Al some work, but they'd rather keep him overworked. Screw it.
You mentioned 2 things that jumped out at me... if it's not showing up on an emg, then it probably isn't ALS is first... and second, you mentioned the ELISA test, and that's a total waste of time from what I've learned. Insist on getting the Western Blot test, and if at all possible, get to a "Lyme Literate" doctor to run the test. They may put you on antibiotics (usually up to 3 different ones) for a month before the test, with the theory that Lyme is a great mimicker, and hides in your tissues in long-term cases. The antibiotics will kill some (not all) of the bacteria, which then can be picked up by the Western Blot test. If you test positive on ANY bands, you should be treated with antibiotics, sometimes for a long time.

Good luck, and keep us posted!
Yesterday we found out my mom, who we thought had ALS based on a muscle biopsy, breathing test and EMG, actually tested positive for the antibody that indicates Myasthenia gravis. She never had any twitching and has symptoms that don't go along with ALS/MND so her neuro kept digging. It seems like MG is something they can diagnose with blood tests and CT scans, so maybe ask your doctor about performing those tests specifically looking for MG.
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