Clinic Update

[ShiftKicker]

Just sticking my clinic update here for those who have questions about pure UMN MND/PLS. This section of the forum has been relatively quiet these past few weeks, but perhaps this will prove a useful reference to someone.

I had my ALS clinic check up yesterday. I had a list of things to discuss with the neuro and team, but only got to about half of what I wanted to know. Most importantly, I had an EMG to make sure no LMN signs indicating ALS.

The clinic in Vancouver is awfully hands off and conservative when it comes to assessment and treatment. I had to ask directly for a variety of things- nothing was volunteered. The nurse essentially watches you walk down the hall to the examining room and that's the extent of a physical assessment. As I was not out of breath walking slowly for 20 feet, I was not offered a breathing test- either sitting up or lying down- despite stating I had night time breathing difficulties.

Anyhow, the doctor offered some very heartening, and disappointing, information with regards to her thoughts on what I had. I let her know I was still thinking/hoping it was all in my head (formal term "functional") and she unequivocably stated I had MND- and stated that if there is UMN involvement it is very easy, even if it's mild, to observe with a few simple tests. UMN issues are not something that can be caused by stress or some form of somatization (dammit!), though they definitely can be made worse by stress and fatigue (cue recommendation for rest, meditation and stress relieving techniques). Again she indicated that emg can pick up UMN signs via abnormal "recruitment" patterns along with a clinical exam, but did not give me any more than that. I hope to get a full report from my GP when the neuro sends it to her. I'll attach here when I do, as I feel that this info may be important for others in the DIHALs forum who think neuros can miss UMN signs. She felt, with my sudden onset in one leg, with subsequent all over currently mild, but increasing, dispersal pattern of spasticity that what I had was PLS- though she would have to wait 2 more years to confirm. Her experience with others with PLS gave her confidence in this opinion because of the similarity in pattern. Her thoughts were if I had ALS, another limb would be more seriously involved by now instead of the even spread of symptoms she observed. While I had visible faciculations, these did not show up on the EMG she conducted- which was of great relief to me. I will be checked again in 6 months.

So, good news (no LMN signs) and bad (still motor neuron disease). I still await a sleep study and a visit with the physiatrist within the next two months to get some real life useful info about how to deal with disturbing and uncomfortable symptoms.

 

[Gorby]

That is really mixed news. A big relief that the EMG is still not pointing to ALS.

I also have a pattern of mild, all-over loss. I hope the specialists can get you some relief, especially with your sleep.

 

[hopeagainsthope]

Thanks Fiona, for this update, it is incredibly helpful and selfless of you, as it does indeed provide valuable clues for others. I am so very glad you are staying "upper", but as you say still not good. It was so interesting that you mentioned "somatisation". My family have been discussing this here over my diagnosis, as this has all come to a head since the loss of my Mother last year.It was a terrible time.We were thinking it was possibly linked. But...... I feel that this has been sneaking up quietly before all the stress I know the mind is powerful, but not this powerful surely, to cause such pathological symptoms!

 

[ShiftKicker]

Hope, It's my understanding that yes, the disorder/disease can be triggered by serious illness, surgery, injury or high stress- but it already exists and is just lurking in the background until then. It will happen, but doesn't get a foothold till your body is compromised by a stressor of some sort. So not somatic, in this case, but triggered by. Before my physical symptoms (foot drop) I had been experiencing serious fatigue for a couple of years (which was probably caused by mild spasticity). I went through three separate stressful experiences (injury, surgery, work place upheaval) in the Summer/Fall of 2014, which culminated in meningitis- after which my leg really went wonky. Perhaps, if I had not experienced the short intense period of stress/illness, the MND would not have reared its ugly head for another few years. Or maybe I would have just woken up one day with foot drop and not had illness to pin it on. One thing I have heard, when communicating with others in the MND community, is that many people can look back to just before their symptoms started and recognized a high stress period. Others can not. I wonder if it's just looking for meaning or cause where there is nothing.

My neuro spoke to me about another patient she had where the symptoms were exacerbated another underlying condition. When her other condition was addressed, her symptoms of PLS were greatly reduced, though still present. Doctors sometimes need to be reminded that MND may be the main cause of symptoms, but it doesn't stop people from having other health issues that make things worse- or cause them to miss powerful or see confusing signs in their search for diagnosis.

Hope, I sincerely hope you are not having to try to convince people that it's not all in your head. While, yes, people can be completely paralyzed or have convulsion with no organic cause (functional) due to what my doctor calls a software problem (brain signals are being misdirected), PLS/ UMN MND is a hardware problem in the sense that the motor neurons are being destroyed- which can be detected by the clinician with reflex issues and read in odd patterns via emg.

 

[hopeagainsthope]

This really helps me understand the whole picture re brain in UMD as I thought it was more of a software issue than actual destruction of the motor neurons in the brain. So that is even more sobering. I have fought my corner re stress after event, as I know all my family just cant believe what is happening to me in front of their eyes and want to have answers. So it is coming from a good place:???: I am seeing 3rd neuro next week and have plenty of questions. Hoping for physiotherapy to at least be proactive.I have found all your posts so intelligent and helpful Fiona thank you.

 

[Tractor Boy]

Hey Fiona...my neuros don't seem to think I need an EMG (haven't had one in 2 years). After reading your post, I think I will insist on one.

At my last ALS Clinic visit in early April, they said they'd like to see me every 6 months rather than every 3. What do you make of that?

TB

 

[billbell52]

Tractor Boy, the longer time period means they are not seeing much change and they don't need to see you as often. I went from every 3 months for the first 2 years then to 6 months now I am at 12 months. To be an MDA patient I need to go at least every 12 months. I haven't had an EMG for 4 years and I don't expect they will order one unless I show a lot of change.

 

[ShiftKicker]

Yep- Bill seems to have it right. If they don't see anything pointing towards lower motor stuff, they probably would be wasting their time with the emg. Like you, I was told to come back in 6 months too, as my progression has been all over mild and there's been no sudden change or loss of function. I see that as a positive thing as the longer I go without the LMN stuff, the less worried they are about it changing. The doc told me she had a guy in who was 11 years into a PLS diagnosis who only came in once yearly to review medication. The clinic I'm at is pretty hands off though- yours may do more assessment each time you go in. I think your doctor is not worried about your diagnosis changing to ALS - you're in it for the long haul. I'm sure mine will stop conducting EMG once I hit the 3 year mark- I've got another 18 or so months to go.

best

Fiona

 

[billbell52]

I don't know this for a fact but the EMG I had 4 years ago I think they only ordered it to give some intern a chance to do an EMG. I go to a teaching hospital.

 

[Tractor Boy]

Well, I sure hope you guys are right. My symptoms are getting worse in my eyes. I've lost my ability to use a walker and my voice is totally toast now. However, I had another swallow test and aced it! I had a bad one 3 years ago. I think that had something to do with the Vitalstim my ST used on me.

I go to the University of Michigan Hospital for Clinic.

So how bad does PLS get? Does it ever stop progression? What can I expect? TB

 

[Green Queen]

TB that question makes me so sad, as does this thread.
Progression is such a terrible word.
Do you have AFOs at all? Or are you wheelchair bound now?
I'm getting a new AFO. It's the fuggliest thing out, but gives me so much more stability when I walk.

I'm so sad for (everyone) the PLSers, always wondering about LMN findings.

I pray for you all.

 

[ShiftKicker]

TB, I am with Janelle- Progression is a terrible word to have to contemplate. It sounds like you need a more proactive clinic experience. Your neurologist/care team should be assessing you and the state and rate of your symptom progress and letting you know what to expect as things carry on. If that's not happening, your clinic needs to get a kick in the pants to get things going.

Fiona

 

[lgelb]

TB, the best available info may be in this abstract but it's kinda depressing. Basically, it says that "PLS" is a very dicey diagnosis that usually ends up being ALS. But there can be as much as a decade or more before that happens, so carpe diem.

It's pretty confusing for the clinic staff to face you, as a "PLS" patient when studies are questioning the very label, and it was rare to begin with. So it's always easier to say, "See ya in 6." That said, if you need specific support, equipment, advice that you are not getting, ask for it. It may not be at the clinic. It could be with your regular doc, a pulmonologist, physiatrist, physical/occupational therapist, anyone who wants to help with fatigue, spasticity, breathing, speech. And, then, there is always here in the digisphere!

Best,
Laurie

 

[billbell52]

I have read about 5 reports and PLS is always between .8% and 2% of ALS. This has it at 1.4%. I was told that Bulbar ALS progresses quickly and that if you have Bulbar PLS for 2-3 years with no LMN involvement it is highly unlikely to be ALS.

 

[lgelb]

Even with bulbar PLS onset, you could "convert" later but any form of ALS is likewise slower when it begins with UMN-only disease.