Had MGH ALS Clinic Visit on 1/10 (Doc found UMN and LMN issues)

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Jshemnitz

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Here was my last post from December 2019 -- Pulmonary Function Testing done today -- closer to ALS diagnosis?


Summary of 2 past EMGs

3/26/2019 (Tufts / Neuromuscular doc #1) – 5 muscles tested in right arm and leg tested (Deltoid, Ant Tibialis, Vastus Lat, Gastro Medial Head, 1st dorsal intercostal) – Completely normal across the board

Nerve conduction test showed mild cubital tunnel in right arm

8/9/2019 (MGH / Neuromuscular doc #2) – 7 muscles tested in left arm and leg ((deltoid, triceps, pron teres, first dorsal int, vastus, Med, Tib Anterior, Gastroc Med (all completely normal)

Nerve conduction test was done on left side – totally normal



Summary of past Neuromuscular Specialist Visits through Dec 2019

12/20/2019 (MGH – neuromuscular doc #2) – clean clinical exam

12/19/2019 (Tufts – Neuromuscular doc #1) – clean clinical exam

8/9/2019 (MGH – Neuromuscular doc #2) – clean clinical exam (done same day as EMG #2)

3/29/2019 (MGH – neuromuscular doc #2) – clean clinical exam

2/27/2010 (Tufts – Neuromuscular doc #1) – clean clinical exam


Past PFTs

12/27/2019 – FVC 95%, 94% FEV 1, MEP 115 (total #) borderline low but normal MIP 77 (total #) borderline low but normal (total #), RV 164% of expected – high (from Tufts – ordered by primary care). Pulmonology listed it as a normal PFT but wanted to dig more into the MIP/MEP borderline numbers. He was reasured that my FVC was the same if not a point or 2 higher in the supine vs upright positions.

Jan 2018 -- FVC 100%, FEV1 95% for baseline (no MIP or MEP tested or other lung volume subsets)


That brings us to the past 3 weeks


Because of the borderline low MIP and MEP from Dec 27, 2019 and increasing symptoms with no answers, primary care at Tufts talked to their Neuromuscular group to have me back in for a repeat clinical. The neuromuscular team at Tufts has 3 people on their team. I had previously been seen by neuromuscular doc #1 there. My primary care knows I didn’t like the neuromuscular doc #1 there, so she had me see a different neuromuscular doc in the group. (we will call her NM #3). NM doc #3 performed a full clinical visit on 1/9/2020. The clinic visit was 100% normal but did order an EMG which ended up being done by the director of the group the same day as she didn’t have time on her schedule for 2 weeks to do it herself.

The NM Director from Tuf’s EMG on Jan 9 (EMG # 3 overall I have in the past 10 months) tested 5 muscles on my left side including Tongue, T7 Paraspinal, deltoid, Abductor Pollicis Brevis and Gastroc Medial – results were 100% normal. He had 2 different 4th year Medical students with him in the room for their neuro clinical rotation so he spent extra time on the EMG and showed them on the screen how it all works and what each of the specific categories mean on the test. He probably had the needle in for 3 or 5 minutes each spot. At the end of the EMG, he told me that because the clinic visit was totally normal and the EMG was normal, I could 100% write off any possibility of ALS so I was obviously excited. At this point, I still had MGH ALS clinic visit for Jan 17 that I had scheduled a few weeks back and was just about to cancel it because of how well the clinic/EMG visit went on Jan 9th.

MGH ended up calling me the afternoon of the Tufts EMG and said they had a last-minute cancellation and had an opening a week early (for Jan 10.. the next day. I let the ALS MGH doc know that I had a clean clinic visit and EMG done the prior day at tufts. During the clinic exam on Jan 10th, ALS doc found weakness (4 out of 5 strength) in 2 muscles in my right hand which is my dominant hand (finger extension for index finger and abduction for pinky –see below). None of the prior 3 NM docs tested individual finger extension but they did do the abduction testing so not sure how the pinky abduction issue was missed. She also detected a brisk jaw jerk reflex (she mentioned this finding in the cranial nerve testing portion of her written summary, but then she reported in the final assessment section that my reflexes were normal so I am a bit confused (see below). I read her notes on their online portal that night and sent her an email to ask about the jaw jerk and she hasn’t responded back to me yet). During the exam itself, she told me in real time that I had brisk bilateral knee reflexes (but told me they were not pathological) and listed them as 2/2 on the report (I am less concerned about that part.). I’ve had my knee reflexes tested many times before and no one ever mentioned anything about being brisk.


Here’s the portion of the ALS doc’s report talking about cranial nerve testing, reflexes and manual muscle testing and her final assessment/recommendations

Cranial Nerve examination revealed: Palate elevated bilaterally and symmetrically. Shoulder shrug was normal. Tongue bulk and strength were normal. No nystagmus. No ptosis. Speech is coherent and fluent. No dysarthria or aphasia. Jaw jerk brisk.

Manual Muscle Testing revealed the following MRC grades (R/L):

Neck flexion 5, Neck extension 5,
Upper extremity: Shoulder abduction 5/5, Elbow flexion 5/5, Elbow extension 5/5, wrist flexion 5/5, wrist extension 5/5, finger flexion 5/5, finger extension 5/5 except EI 4/5, FDI 5/5, ADm 4/5
Lower extremity (examined in log roll positions): Hip flexion 5/5, hip abduction 5/5, knee extension 5/5, knee flexion 5/5, ankle dorsiflexion 5/5, ankle plantar flexion 5/5, foot inversion 5/5, Toe flexion 5/5, Toe extension 5/5. Hip adduction was full strength bilaterally.

Deep tendon reflexes were 2 at the biceps and triceps, 2 at the brachioradialis, 2 at the knees, 2 at the ankles bilaterally. Plantar responses were flexor bilaterally. Hoffman and Tromner reflexes absent. Crossed adductor absent b/l

ASSESSMENT:
His‏‎ neurological examination today is essentially normal including reflexes, except for right Ei and ADm weakness, which I discussed may be his baseline and not related to ALS. We can obtain MRI C spine to make sure no C7-T1 radiculopathy to explain his right finger weakness.

RECOMMENDATIONS:
I would like to see him for a follow up visit in 2-3 months. I am happy to see him sooner for urgent concerns.


So now I have both UMN (Positive Jaw Jerk) and LMN signs (finger weakness). I went from having a 100% clean EMG/100% Clean Clinical on Thurs 1/9/2020 with 0 chance of it being ALS to having both UMN and LMN signs on 1/10/2020.

I am concerned with the positive jaw jerk because I know it’s a UMN sign. My previous 6 clinic visits over the past year with the other 3 NM docs never did the jaw jerk test as part of their clinical exam. In terms of the jaw jerk, starting over the past summer, I had noticed that my cheeks were all chewed up and my dentist said it looked like I was inadvertently biting my cheeks without even realizing it. I have also been getting pain in my back left part of my jaw/top left part of my throat for the past couple weeks and is just starting up on the right side now too. I also know a positive jaw jerk is often corelated with bulbar dysfunction. I have had a hoarse voice for a while that has gotten worse the last few weeks (originally diagnosed via Laryngoscope and Stroboscopy over a year back as having LPR reflux from the scopes showing edema on vocal cords and tight voice box (muscle tension dysphonia. My voice gets extremely weak after even minimal usage now. I also yawn all the time even after a cup of coffee and even when I am not tired (I have been yawning non-stop the past few weeks. I am going back to see the laryngologist on the 28th to get rescoped. My tongue was tested on the EMG on Jan 9th which would hopefully have detected bulbar issues.

I have the cervical MRI scheduled for Jan 24th. I previously had a brain MRI done in Sept 2018 after a month-long spell of intermittent headaches that was negative except for an arachnoid cyst that I was born with so that almost rules out MS. I had also had a lumbar MRI done this past summer that was relatively normal except for some mild spondylosis changes.

I also noticed over the past week that when my right hand has all the fingers extended, I cannot bend my pink finger towards my palm on its own but can on the left hand (I can bend the pinky and ring ringer together when holding all fingers out on the affected right hand. I am able to bend the pinky on its own when other fingers are in a fist. I know a lot of people have independent pinky bending skills so I am less worried by that. I haven’t had an EMG on my right side (side with clinical weakness) since late March 2019 which did include an EMG of the first dorsal interosseous in the right hand. Previous doctors had talked about anxiety as a possible cause of my symptoms but the ALS doc clearly said this does not appear to be an anxiety given the fact she found clinical weakness.

So now at this point, in addition to the issues noted above, I have twitching (both legs across calves and thighs mostly and on butt), cramps in my right and left hands, feet and some in my calves. I also continue to have muscle pouring off my entire body which has now been going on for well over a year now. I have been dropping things in my right hand for several weeks now and my right hand shakes when I perform many activities with it (I am a righty)..I am finding it hard to keep a fork steady in my hand while eating over the past week. I also get a lot of Charlie horse pain in both my thighs, especially the right. Also, I’ve had 0 appetite the last couple weeks but I know I need to eat so I have been forcing myself to eat and getting my normal calorie intake. My left leg had been feeling very tight the past couple weeks, and now all the sudden as of yesterday, it feels kind of wobbly, to the point where I stumbled going down some stairs last night (I was fine, just startled)

Even if the Cervical MRI on the right side shows issues, it wouldn’t be a cause of my whole body muscle loss that I’ve had the past year that has caused me to buy new shirts and pants 2 different times since 2018. The muscle loss is the main reason I went to see my doctors in the first place over a year ago before I even knew what ALS was. I have seen a ton of non-neuro specialists, and had several CTs, MRIs and vials of blood taken to find the cause of the weight/muscle loss with no answers. Cervical neck issues also wouldn't casuse my left leg issue

Jason
 

Nikki J

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She was clearly not as impressed as you by her findings. Who did you see by the way? To characterize that exam as umn and lmn findings is a stretch to say the least
 

Jshemnitz

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Hi Nikki,

I saw Suma Babu - Suma Babu, MBBS, MPH - Department of Neurology. I had previously seen Dr. Paloma Gonzalez-Perez over the course of 2019 in their general NM practice. She seems very nice and easy to talk to, but she is horrible about responding back to messages. I emailed her on Friday after I saw the jaw jerk in the notes and haven't heard back. I tried to call her office and leave a voicemail, but they said just message her on the patient gateway online system instead.

In terms of her findings, isn't Clinical Weakness a LMN sign? Also, is jaw jerk not a UMN sign?

Jason
 

Nikki J

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You were extremely lucky to see Dr Babu who is a fabulous doctor. weakness has many many causes and not all weakness is lower motor neuron by any remote stretch. a lone brisk jaw jerk ( which is another body area from the minor weakness) is an iffy umn sign at best.

what did she say after the exam ? I will tell you she is terribly busy running clinical trials and studies but if she really thought you had something to worry about she would have answered by now
 

Bestfriends14

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No one thinks you have ALS but you. Go back and re-read all of your many posts, and the answers provided. Can you see a troubling pattern? You simply will not take the answer "you dont have ALS". Why is that? A rational minded person would be ecstatic, yet you are not. You are bound and determined to be diagnosed with a disease you dont evidence showing.

I actually found your last post insulting to those who have this disease. To say something so minor as a 4/5 strength ( a normal finding) as a LMN finding, and jaw jerk as an UMN finding (again normal in the context of your case), is possible ALS is seriously reaching.

Why can you not let this disease go?? You do not have anything indicative of ALS. At all. Let that sink in and be grateful for that. I'm not confident you will, though. You'll waste years of your life chasing a disease you dont have and you'll never get those years back. For your own sanity, please seek counselling. Life is too short and too precious to not get the help you need. Good luck to you.
 

Jshemnitz

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Nicki -- Next steps were the Cervical MRI which is going to be next Friday AM. She wants me back in the clinic in 2 to 3 months to see if things have progressed or not.

Best Friends - I guess I am confused how 4/5 strenth is a normal finding.. I thought 5/5 is a normal finding. I don't want ALS.. no one wants ALS.
 

Bestfriends14

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My left shoulder has a strength of 3/5 and I dont have ALS, I have a shoulder injury. You say you dont want ALS? Then actually listen to all the doctors, all the clean EMGs, and all the clear clinical findings that says you do not having this disease. It's time to move on and let. It. go.
Best of luck to you and do take good care
 

codyclan

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The doctor's own notes states that the weakness may be your baseline and not related to ALS. Seriously, I can't imagine reading that report and jumping to UMN and LMN findings. You are really reaching. If we had gotten that report with my late husband we would have been ecstatic. Follow through with the MRI and a follow-up appointment but ALS does not seem to be on the table.
 

Nikki J

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So have the Mri and do the follow up but do not obsess about these minimal findings. I am closing the thread. You may post the mri report and you may post Dr Babu’s follow up appointment results
 

KarenNWendyn

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Jason, just because the doctor found some abnormalities on exam and gave you a followup appointment does not mean she thinks you might have ALS. She is trying to be thorough and find some alternative explanation for your symptoms. You’ve already been cleared of ALS by multiple neuromuscular specialists. The evaluation you had at Tufts was very thorough and definitive. As someone who is dying from this disease, I find your obsession with it sad and offensive.
 
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