Bulbar questions

[Avarice]

I'm still waiting for an appointment with a neurologist, which won't happen until the end of June.

My symptoms started early November when friends started asking if I'd been drinking due to increasingly slurring speech. I also noticed profuse saliva when I would sneeze. By January, the slurring got progressively worse and swallowing became difficult. Also noticed my walking gait started to be affected, right leg felt odd while walking. Also noticed the right side of my mouth didn't move as much as the left, suddenly had a crooked half smile where it used to be pretty symmetric.

Feb went to the ER where they ruled out stroke and heart attack. Had an MRI and this is what my GP went with for the referral to the neurologist: "Impression: high signal along the cortical spinal tracts bilaterally at the upper limits of normal. This is nonspecific, but can be seen in the setting of ALS, Clinical correlation is suggested."

Speaking has degenerated to the point where the throat makes sounds, but the mouth and tongue are no help. S and SH sounds just shove out a ton of saliva. The saliva seems even more profuse. I haven't had total muscle failure short of speaking being extremely slurred. Limbs don't move as they should, but I can still stand and walk, button my shirt, etc. And there is definitely weakening all over that's progressed.

My questions are, how fast does bulbar progress? Does it often come with partial mouth paralysis on one side of the lips? Would the throat feel extra sensitivity to acidic stuff like tomato soup? That stuff burns from the acidity for me.

The GP and nurse practitioner I've seen so far seem rather focused on ALS. I've tried to ask about Lyme as at one point in the summer something left a distinctive bullseye rash. But as that's seen as an East Coast thing, every medical professional so far won't humor testing or considering it.

I guess I've worried because the progress of it all seems so fast that I wonder if I'll even make it to my neurologist appt.

 

[affected]

You are nearly there, only a couple more weeks.
The slurring is very concerning I do agree, but it's one step at a time.

Bulbar can progress fast, my husband was rapid progression bulbar onset.
However you are not about to die in the next couple of weeks, you don't report any breathing issues nor choking that may cause an aspiration pneumonia. Just calm down as much as you can and work on what you want to ask when you to do the specialist later this month.

Update us then, and I wish you all the best.

 

[Avarice]

Thank you kindly!

I guess it's been a frightening couple weeks. A more pronounced brain fog that comes and goes, along with every muscle feeling more like lead. BUT good to know I shouldn't worry about it progressing so fast as to kill me before the appointment.

 

[AP1981]

Hi there,

You will definitely still be around for your neuro appt.

Slurred speech can indicate als but there are other conditions also that are treatable.

I have bulbar onset. It may be worth while writing down questions for your neuro if speaking is a challenge.

Keep us updated. My fingers are crossed for you that it is not ALS.

 

[Avarice]

Thank you, AP1981

I am definitely writing everything beforehand. My speech has deteriorated so badly, I no longer speak at all. The throat can still make sounds, but I just haven't been able to form words that anyone can discern in well over a month. Basically had to write everything before my GP appt. Anything I tried to say in the doctor's office back then wasn't understood.

 

[Avarice]

Update:

finally got to see a neurologist today. Not good news at all.

Based on the info I gave, on the MRI and observations from the ER, as well as his physical exam of my weakness, he's certain I have ALS. The EMG is being done next Friday, but he believes because of the severity of my throat and tongue weakness, the atrophy of my back and arms, that it'll indicate ALS.

He said based on how fast symptoms set in, I may need a ventilator in about 4 months.

 

[Nikki J]

I am very sorry to hear this. Hoping that things turn out differently but start organizing your second opinion if you have not already done so. Even if this is a neuromuscular specialist you need another opinion. If it is a general neurologist you must have another opinion.
Did they test your breathing? Severe bulbar dysfunction does not always mean urgent need for vent. My sister was unable to speak or swallow at all for 2 years prior to her death. She did not have a vent

 

[Avarice]

Hi Nikki.

If the EMG confirms his opinion, I'll be asking my GP to refer me to the UCSD ALS Center. It was where I was supposed to go before I had switched GPs.

He never tested my breathing. Only things he did himself was test strength and reflexes of my limbs and extremities, then checked my eyes. He checked my tongue too and looked at my throat.

I'm not sure how he's categorized. His expertise is listed as: Alzheimer's Disease, Epilepsy, Migraine Disorders, Multiple Sclerosis (MS), Neurology

 

[Nikki J]

Not a neuromuscular / ALS specialist. I would start working on the referral now. Can always cancel. I would take that vent comment with a big grain of salt if you did not have any breathing tests

 

[Avarice]

Going to my GP's office tomorrow (or Monday if they're closed) with a note to request that referral. Thank you so much!

Also, should I mentally question this a bit that he's that confident it is indeed ALS before even running an EMG?

 

[Nikki J]

I have said before I would not accept an ALS diagnosis until confirmed by a neuromuscular specialist. We have had people who were told by a general neuro they had ALS after an EMG even only to have the diagnosis overturned by the neuromuscular doctor.

Certainly without an EMG I would have hope. That said, your symptoms are very worrying. I would prepare for confirmation but have hope this is something else.

 

[Avarice]

Update:

The neurologist ran an EMG, he stopped after testing my left arm and leg. He never gave me any specific info beyond saying the results were consistent with ALS. Per the advise given, we're working with the UCSD ALG Clinic to get a second opinion.

But it was certainly a major psychological blow. A big part of me believes that the second opinion may bear the same results, and it all seems to have set in very fast. Every muscle feels so weak. And when I think back to Feb, the physical therapist at the ER remarked that my entire back seemed very atrophied.

Thank you for the support and advise.

 

[affected]

So sorry to hear this

My Chris was bulbar onset and even though he didn't have weakness that we were aware of, he had a lot of atrophy in his back a couple of years before he started slurring. I'm amazed he used to do the things he did with how those muscles felt.

Do you know yet when you will get the second opinion?

 

[Nikki J]

I am sorry to hear this. I hope you get your second opinion quickly. We will support you as much as we can. Please keep us posted

 

[Avarice]

I have my father calling the ALS clinic tomorrow. They do already have a case number for me, but the one that coordinates appointments was off early on friday.

I'm not even sure how to categorize my onset. The mouth symptoms and the weakness in my right arm came on at the same time.