Following up from a previous post…..would appreciate your time

[ccslacum]

Previous thread

Greetings - I apologize for posting a second time. A lot has transpired since my last post around the beginning of the year and I’m seeking to learn more from this very knowledgable group.

Prior to the summer of 2023 I was an extremely active 51 y.o female. Working full time in oncology sales and balancing a very busy family of 3 teenage boys. I’ve been an avid exerciser all of my adult life. I used to hike, bike, run, take pilates classes and was very active with my sons sports. Here’s where things changed:

In late 2022-early 2023 I started having periodic episodes of dexterity issues with my computer mouse. This was followed by a weakness/dexterity in my right foot when moving from the gas pedal to the brake. This would come and go.

Early Summer of 2023: Driving a long distance my left leg began to have tremors. One episode and it didn’t happen again until a few months later.

Fast forward to late July 2023 I had a sensation of whip lash on my left side of my neck. That resolved only to be followed by numbness, tingling and pain down into my right arm. There was a feeling of heaviness and uselessness in the right arm. I saw Ortho mid August who thought it was a pinched nerve and prescribed prednisone. After a week this sensation seem to move down into my right leg. I then saw a chiropractor with no relief.

Around the end of August I started waking up with leg tremors in both legs. It was a frightening experience. I saw my PCP who prescribed gabapentin and refereed to Neuro. I also started experiencing severe cramping in my right calf and tightness in the right shin.
I also woke up one morning and had blood in my mouth from biting my tongue. This is when I noticed that my tongue was trembling and had scalloped edges which it had never had before.
August and September the fasiculations started along with leg weakness going up and down the stairs into my garage and my back deck.

Getting into local Neuro proved very difficult so I went to Raleigh. After an examination he ordered 2 MRI’s (both normal) and a Nerve Conduction and EMG. He observed brisk reflexes, normal Hoffman and Babinski. EMG and NC was normal. EMG was performed on my right side and underneath my chin. EMG was performed by a very respected Neurologist who was the previous head of the Duke EMG program and trained the fellows before joining a private practice. They suggested anxiety meds and f/u in 4 months.

In the meantime I finally got an appointment after a month of trying with my local neurology office and was seen by a PA. The private practice in Raleigh was comprised of mainly general neurologist so I thought it was a good idea to establish myself locally with a practice with a neuromuscular specialists.
She also observed the same brisk reflexes and noticed I was trembly. She also noticed muscle wasting in my right hand and at this point I was starting to drop weight. By this point anxiety was setting in and I was struggling to eat and seemed to be having trouble swallowing and moving food around in my mouth. The Neuro PA ordered a speech evaluation with swallowing which was “borderline normal” and suggested a modified barium swallow which showed “flash penetration with thin liquids”. I also saw an ENT who suggested “silent reflux”.The Neuro PA wanted me to be seen by the ALS specialist in her practice but due to my recent normal EMG she said not until 6 months.

During this time I also noticed my heart rate was extremely elevated and I was having shortness of breath. I’m normally very low with HR and Blood Pressure. Both are elevated. I’ve worn 2 heart monitors and both have showed tachycardia. Any exertion at all and my heart rate is extremely high…..showering, unloading the dish washer, and doing laundry. I’ve never experienced this. My heart rate would even wake me up with my chest pounding and heart rate of 130-150 in the middle of the night. This went on for about 3 months and is mostly better but does happen periodically. PA also ordered Lymes, Mysathenia Gravis, Copper and other tests to rule out other etiologies. NFL was 2.18 (range 0.00-3.78) on 11/3/03

In the meantime PCP ordered autoimmune titers which came back “higher than he ever seen”. Referred me to Rheumatologist who ruled out every possible autoimmune disorder and suggested a neurological component and said to go to Duke. Because of the nature of her referral I end up in Neurology. This Neuro examined me and said, “ no ALS”. However, she did offer a repeat EMG and MRI’s and possible EEG due to the tongue biting. The other Neuro in her clinic suggested FND.

By Christmas I was down to 95 pounds which is 20% of my body weight. I am now back to within 5 pounds of previous weight.

PCP did a Pulmonary Function test in the fall and FVC was 79%. A f/u with pulmonologist 3 months later rendered a 78% FVC. Follow up SNIFF test as normal. Cardio/Pulmonary fitness test was cut short due to me maxing out my heart rate at 9.5 minutes. Report showed diminished exercise capacity. Co2 has historically ran 24-26 and since we started running so many labs it has consistently measured 31-32 x 7 times.

I had the 2nd EMG early February at Duke and it showed 2+ fasciculation's in my right tricep. Neuro said this was normal and suggested cramp fasiculation syndrome. She also suggested essential tremors as the cause of my ever trembling fingers. Suggested a beta blocker and wants to see me back in July after another Rheum work up.

So my 6 month appointment rolls around with the ALS specialist. EMG completely normal. Even after the one at Duke showed fasiculations. She said she did not need to see me back. Her PA reached out and offered LP.

New local Rheum did another extensive workup and send blood to a specialized autoimmune lab in California. Results: ANA igG “ strong positive” speckled pattern. Because both of my hands are so stiff and tight we tried 12 weeks of Plaquenil. Hands are no better, probably worse. Now they are trying methotrexate. I continue to find articles that reference the possibility of high auto immune labs with ALS. The hand stiffness and tightness has gradually progressed since November. Now my right ankle is experiencing weakness and stiffness. If I attempt to take a brief walk I immediately notice.

In a nutshell, here are my symptoms:
Body wide fasciculation's (even flickering in the atrophied area between thumb and forefinger of both hands) - started in August with legs & is now widespread.
Hand tightness and stiffness (both). Nights are really bad, and mornings and again in the evening. - tightness started in Rt. Only in the fall
Right shin tightness (feels like shin splints) Started in August and went away but back basically all the time.
Ankle weakness (primarily right; however left is beginning as well)- last 3 months
Leg weakness; especially stairs. - since August
Tongue twitching (while at rest); sometimes times seems to have a life of its own. Just moves around oddly.
elevated Co2 ( 31-32) since October - coincides with beginning of SOB; had elevated bicarbonate in Oct during an ER visit for HR and SOB.
Tachycardia
Excessive fatigue
Late in the day my voice becomes hoarse, my pitch is off and volume is not what it used to be.
Increased saliva production ( I wear aligned at night and have never had an issue with them filling with saliva before).
Taking extra swallows
Brisk reflexes (all)
This week at Rheum my CK was 248. Previous draw in October was 64.

I apologize for the length of this post. I really value your thoughts and would love your insight.

All my best

 

[ccslacum]

Hi, my biggest concern is the discrepancy with the EMG’s. One showing only fasiculations in one region. The right tricep fasiculations were also visible and noted by the physician at this time, And the one with the ALS specialist showing no fasiculations. I also did not have any active fasiculations during this EMG. My Neurologist has clinically reported breakaway weakness in lower extremities 2+ reflexes in all limbs and hand atrophy. Now the elevation in CK from 64 7 months ago to 248. My fasiculations are widespread including my tongue and my dentist and ENT both recommended f/u with Neuro. I’ve seen a highly qualified hand orthopedic physician (trained at Duke) for my loss of right hand dexterity. X-rays and bloodwork have ruled out RA. NCS ruled out carpal tunnel. He did report clinical atrophy and said he had no idea why but it was definitely not due to weight loss. I’m trying to compensate and purchased a smaller computer mouse. Using a stress ball to try and increase strength.

The EMG is perplexing me. Is it possible they have been done to early? Or not thorough enough?

Thank you

 

[Nikki J]

Whether they happened to catch fasciculations or not doesn’t matter. Neither emg showed anything worrisome which if it were ALS given your reported atrophy and weakness it should have done

What is in the differential of your main neuro? What is their plan?

 

[ccslacum]

Hi Nikki, my main Neuro is at Duke. I will see her again in late July after following up with Rheumatology. She suggested propanol for my hand tremors which are pretty constant. She said it’s “ essential tremors”. She said it would also help my tachycardia. She also said it’s likely cramp fasciculation syndrome and I haven’t been able to find any information pointing to that process causing body wide fasciculation's. She referred to the EMG conducted by her department as normal and “not ALS”. She also noted the EMG completed by the Neuromuscular Specialist was also normal. Duke Neurology will not refer to Duke Neuromuscular with normal findings on the EMG so the ALS Specialist I saw was local to me in the Novant Healthcare system. She trained at Wake Forest University.

Rheumatology has ruled out pretty much all autoimmune processes. We tried 12 weeks of Plaquenil for the hand tightness and stiffness with no results. I started methotrexate Friday night. My ANA remains 1:1280 for the fine speckled pattern. My IgG is extremely elevated.

My pulmonary function test, cardio pulmonary fitness test, SNIFF test, and modified barium swallow were all done locally but forwarded to Duke so she has all the results even though they were done outside of the Duke system. No one seems concerned about my FVC of 78%. Unfortunately we don’t have a baseline because I’ve never had breathing issues. I first noticed it and the tachycardia in early September 2023. One month I was hiking the NC mountains and the next month I had to stop all forms of exercise.

Thank you so much for your time.

 

[lgelb]

From what you have said, ALS has been ruled out.

MTX is a heavy-duty drug often used in chemo as well as autoimmune disorders, and not something you want to start without due consideration. Without a clear diagnosis, I'd definitely want a second/academic opinion before starting it, esp. after 3 mos of PLQ.

A beta blocker is frequently used in the setting of tremor + sinus tach, and that might improve your breathing/exercise tolerance if the heart rate is making your lungs work harder than they need to. If it doesn't suit, a calcium channel blocker might be a better fit. I would speak with your internist about this since the choice of class relates to other medical conditions, if any.

But you're in the wrong place here. A Wake-trained neuro, a Duke neuro, and two EMGs are telling you that.

Sudden onset of tachycardia and reduced lung capacity are characteristic of postviral syndromes, including long Covid, even among ppl who never tested positive, so I would follow up with your internist on this as well. There are new approaches to long Covid that may be worth trying empirically.

 

[Nikki J]

Bfs and cfs both frequently have all over fasciculations - more often than not

As you say you have no baseline for your fvc. I understand you feel symptomatic but apparently when they looked at all your pulmonary and other tests it didn’t look worrisome and 78 is very close to 80 the technical normal. And it is true even some healthy people have abnormal pulmonary values. One of the research associates at my clinic told me their perfectly healthy asymptomatic colleague had a value in the 60s % when they were doing the clinic testing as part of their training. The person was sent for a full pulmonary workup which turned out fine with the 60 number being designated normal for them.

 

[ccslacum]

Lgelb - Thank you for your suggestions. I did have a pretty severe case of Covid in January of 2021. I even had some neurological symptoms (difficulty walking) during week 3 and my physician neighbor recommended I be checked out in the ER for possible stroke. I checked out fine and after lots of fluids I bounced back quickly. I never had any respiratory issues at all during Covid…..just fever, headache, fatigue and the walking issues. Life went back to normal…..exercising, etc.. I have been evaluated by the UNC Long Covid clinic (January 2024) and went through a pretty extensive process. I have also seen a cardiologist they refer to. My two holter monitors showed that I am in tachycardia 40% of the time but they have not identified any reason why. I was very hopeful that this could be related to Covid; however, the muscle wasting, fasciculation's, dexterity issues and high CK do not seem to fit the picture of that.

Prior to and after Covid I have not had any sickness. I had the flu in middle school and once in college and that was a very, very long time ago.

My first symptom was the right hand dexterity with my computer mouse in early 2023 followed by all the other symptoms in mid 2023. My right hand is so atrophied now that I’m almost embarrassed to to show my hands because everyone asks questions. Same with the hand tremors. Oncologists and their clinical staff are my customers and I know the constant hand shaking has them wondering what is wrong with me. My BP and HR have always been super low. To the point I’m always asked by physicians if I get light headed early. The only time I had elevated BP is when I was pregnant with my twin sons 16 years ago…..and they missed my pre-eclampsia because they didn’t look at my BP trends and assumed because it wasn’t the “numbers” they normally see.

Prior to getting into Duke I saw a Neurologist at Raleigh Neurology 9/2023. The EMG was performed by the former head of the EMG program at Duke. After a normal EMG the Neurologist said you don’t have ALS “right now”. So that has stuck with me as my symptoms have greatly progressed that he thought it was too early for the EMG to pick any abnormalities up.

 

[ccslacum]

Thank you, Nikki. I have a friend who has her PhD in nursing and started the states first Asthma Risk Reduction program and she has also said that 78%-79% is normal. My last hike was the first of August 2023 and I had no issues at all. We went to Asheville in May for a soccer tournament and I attempted a very small hike at the Biltmore Estate (so it was very mild with barely an incline ) and I couldn’t finish it.

With EMG’s do you typically see additional abnormalities in addition to the fasiculations? It’s so hard find anything online that describes the different parameters they are measuring and what comes first and what you should expect.

 

[lgelb]

EMGs test for many conditions, of which ALS is one of the rarest. An EMG supporting ALS would have multiple abnormalities that yours evidently did not, and there are entire textbooks on interpreting EMGs. If you are curious about the diagnostic criteria for an EMG in ALS, that information is available online.

But there is no point in going backwards. Treatment for the tach/tremors and continued search for a treatable etiology for your issues would be going forward, but is beyond the scope of what we litigate here.

The fact that your Covid wasn't respiratory and how long ago it was, has no relationship with whether you have long Covid or not. Nor is concern about multiple issues mean they are linked to some grand answer, nor does "right now" mean anything sinister -- neuros tend toward caution in pronouncements since they are routinely sued.

If/as you have a diagnosis, feel free to return and share it. Closing this thread so you can focus on what will get you there, which isn't here.