New MND

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Hreal85

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Hi
My dad who is 61 years old was diagnosed with MND today. He saw a neuro specialist at MGH in Boston after having two abnormal EMGs one in his right arm and the other in his left leg both done in the past month.
He first noticed his symptoms last December 2019, he couldn’t pick up a quarter with his right hand and over this last year it’s progressed with difficulty writing and buttoning up clothes but the rest of his arm is fine. And he can still write and cut food up he just has to go slower. Also around the same time that he started noticing the hand issue,he was having Charlie horse cramps around his abdomen/chest and sometimes his calf. He also will notice twitches in his abdomen and right hand. Otherwise he is doing fine no pain or gait issues or weakness anywhere else, nothing in the legs even with the abnormal EMG result.
The doctor said it might be a MDS that is slowly progressing. I guess I’m looking to see if anyone else had a similar experience. I know everyone is different as far as how they progress but has anyone found that there are periods were its slow then fast with regards to symptoms? Also how long from when someone was diagnosed with a MND did you actually get a more specific condition like ALS? He is starting Riluzole twice a day.

Thank you for reading.
 

Nikki J

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This was one of the mgh ALS clinic doctors? If you ask they will explain why he apparently does not meet the criteria for ALS. Generally it requires an emg in a specific pattern plus some upper motor neuron findings on exam - abnormal reflexes, spasticity, clonus Is there a plan to repeat the emg? Sometimes if it is looking like likely als but not quite they will repeat in 3-6 months.
the Healey Center doctors are usually very good at answering questions. Also the clinical note will show up on the patient portal when it is finished so you can read that is your father allows

the nurse and if he saw one the nurse practitioner would answer what they could too.

Riluzole works best given early so good he is starting that

as you said everyone is really different and progression is not linear If the issue is a not quite diagnostic emg often the follow up in 3-6 months settles it. If it is a lack of upper motor neuron signs that can be more variable.
 

KimT

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Mayo had me come back every three months. During that time I got a second opinion. It took four EMGs and clinical exams just for me to get a probable ALS diagnosis because of my strength. That sent me seeking two more opinions, one from Johns Hopkins and another from the University of South Florida. All this in about a year. I also had a phone call with Dr. Bedlack at Duke after he reviewed my records.

Progression cannot be predicted. Mine has been slow.
 

Hreal85

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Thank you for your responses.
My dad is scheduled for a follow up EMG in 3 months but having read the doctors note with my dad’s permission, it’s says the “NCS/EMG showed widespread ongoing denervation and chronic reinnervation changes.” And MD Paganoni wrote in her note that the diagnosis is MND/ALS. So I guess the 3 month EMG is either to double check the result since they weren’t done at MGH but at another facility and/or just to check on the diseases progression.
 

Nikki J

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MGH often wants their own EMG The only exception usually made is if it was done at another ALS center when they know the original EMGer. Usually once the diagnosis is made more EMGs are not necessary unless for research purposes.

Dr Paganoni is great. Did she speak of trials?
 

Hreal85

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It was the first visit so maybe she didn’t want to overwhelm him. She mentioned clinical trials but in a broad sense. She wanted him on the Riluzole and to assess his liver function monthly for the next few months. She also started the process to see if he can get IV Radicava through his insurance. (Side note ..I saw on a post here about oral Radicava...is that in the clinical trail phase or is it an alternative to the IV?)
For the cramps in his abdomen/chest she recommended Mexiletine but wants an EKG first since it’s primarily used as a cardiac drug with off label use for cramping.

I’ll ask her but I’m wondering if she is starting him on those two first since they are FDA approved for ALS and then she will see about a clinical trial. I think she wants to see how he reacts to those two medications first before adding more in,however she did say they are generally well tolerated. I’ve been reading posts in this forum and just been writing down different medication names and clinical trial drug names so that I can look them up myself and then ask her about them in regards to my dad’s treatment. She seems like a very nice and competent doctor so I’m happy he is with her. I don’t want to overstep with my questions and asking about different medications he could try but on the other hand I want to make sure he has the best quality of life he can have for however long that is.
 

Nikki J

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It sounds like his diagnosis is pretty clear if she is doing radicava. Yes the oral is a trial/ not avsilable. Since you virtually always have to be on stable doses of riluzole and radicava for thirty days before you go for a trial qualification visit it makes utter sense to do that first.

one thing to bear in mind with supplements is that if you take something that is in a trial somewhere within 30 days of a trial visit that also disqualifies you.

Dr Paganoni is great and she has a ton of trial experience she did the Amylyx centaur trial and is the co chief investigator for the platform trial

mgh is very research oriented. If you indicate interest / willingness they will tell you if a study ( observational or interventional) is available that you might qualify for

mexilitine usually works well for cramps. They did a trial to see if it would slow ALS It didn’t but people said it worked for cramps so they did another trial for cramping. My sister used to take it. She tolerated it well
 

Hreal85

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Thank you! I’ll keep all that in mind.
 

Nikki J

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Oh just another thought Dr Paganoni probably told you but your dad should sign on with CCALS which is a patient support organization. You can do ALSA too if you want but most of us around here find CCALS more helpful
 

lgelb

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Very sorry to hear about your dad. Of course, we'll support however we can.

His progression sounds pretty typical for a slowly progressive MND. It is a hallmark of ALS that even areas that seem fine show up abnormal on EMG, like his leg. We would not expect pain at this point, since that comes from immobility, not ALS itself.

Best,
Laurie
 
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