Atypical Parkinson's like ALS

Neutrino

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Jul 16, 2021
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12
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Learn about ALS
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00/0000
Country
UK
Hi! Whilst im waiting to be admitted back to hospital, i do have a question.

Just on the planning side of things really. Assuming it does turn out as one of the MND's.

So in 9 months i have gone from healthy, to bout a 30% loss of muscle mass/widespread atrophy in arms and legs (more in some areas, less in others, but legs are more affected than arms at the moment). In the last 6 weeks i have lost the ability to stand on heels or toes. In the past couple of weeks i now cant use a fork (index finger cant put any pressure on a fork to position it), and opening bottles/caps is near impossible. The fasciculations are also in a orderly fashion working their way up my legs, and increasing in frequency. Foot inversion has increased, and i now walk with a limp, plus toes are curling downwards permanently on both sides.

So the question is, im wondering what peoples experience of this kind of progression is. Linear, exponential, up and down? As even if linear, id look at the above, and assume in another 9 months (or even less) id be struggling to walk/hold my own weight up, and hands a fair bit more worse off than they are now.

I will of course put the same question to the consultants in a few weeks, and im sure once any diagnosis is made, the occupational therapists will be available. But would be good to get jist if this is the normal kind of speed it goes at, and i should be expecting to be not so mobile in another 9 months.
 

Nikki J

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PALS
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04/2014
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US
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ALS progression is not usually linear and every PALS is different. Some have plateaus, some don’t Some start fast and then slow down. My sister did. Some start slowly and speed up. one of my aunts did. My progression although ultra slow goes in spurts. Some, sadly, progress at lightning speed. We had a member here whose FALS family all went from fine to dead in 2-3 months.

generally speaking it is good to prepare in ALS. You don’t have a diagnosis but since they haven’t yet found anything treatable it is good to look ahead and if it takes a long time to get there all the better. I hope you have discussed what services you might get. Without an MND diagnosis you won’t get those but the UK is infinitely better at helping you manage at home than the US
 

Neutrino

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Learn about ALS
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Thanks Nikki. That is good info to know. We'll, not good of course, but usefull.

Yes, they have mentioned what will be available in terms of people once diagnosis is made. As you say, not a lot can access until a diagnosis. Will just depend if its mnd, ataxias/motor neuropathys or msa, as would then be at different hospitals/centres.

I think the plan is I will get a diagnosis at the end of the week I'm admitted. I'm assuming, if they exclude cancer, and the genetic panels, plus have an explanation seperatly for the autonomic symptoms. It then leaves me with upper and lower motor neuron signs/abnormalities. Is that enough to meet the criteria? I had that mildly abnormal emg couple of months ago. So guess as well as the lower/upper signs, it could also then be more clear cut on the next EMG.

What a pickle! 🙂
 

Neutrino

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Jul 16, 2021
Messages
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Learn about ALS
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UK
Have a simple ish question for a change :)

So part of this whole process I have had now quite extensive atrophy/wastage of arms, legs, shoulders, face muscles, and as of a GP check up this morning, the intercostal muscles.

But, these are almost complelty symmetrical left and right. Plus as many areas in upper limbs as lower limbs.

Can ALS be so widespread and symmetrical? I think earlier replies said it could be a difuse onset. But is it possible for ALS to be all limbs and all of those progress at the same rate?
 

Nikki J

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PALS
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Did the atrophy happen simultaneously on both sides? Or in multiple areas? That is not usual for ALS your emg should also have showed with such symptoms. when is the hospital happening?
 

Neutrino

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Best way i could describe it, is it was a staggered start to a certain level, then everything seems to have found an equilibrium and is now going at what seems the same pace (not so much the same speeds, just all are progressing at the same time). The early muscle groups where definitely not simultaneous. But most recent ones in upper legs, shoulders and intercostal, appear equal amount (left/right) of wastage at the same time.

Roughly:

  1. Left hand
  2. Left foot
  3. Right foot
  4. Left/right lower leg similar timing
  5. Right hand
  6. Bulk of face muscles
  7. Left/right lower arms (left a bit ahead of right)
  8. Left/right upper arms/shoulders
  9. Upper legs both sides
  10. Intercostal both sides
But, they all kind of fade into each other, as this is over just 4 months or so since the atrophy/weakness started. Cramps and fasciculations are still mostly in the legs, but im noting more in upper arms now. It is also definitely still the lower legs, knee down as the most wastage as a percentage, with both sides having similar amount of wastage.

I get admitted Tuesday, the repeat EMG is the Wednesday, then everything else the Thursday and Friday.
 

Nikki J

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PALS
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What happened with your hospitalization?
 

Neutrino

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Country
UK
Hello All,

Just dropping back to update, and say farewell to the forums.

Looks like they have pretty much honed in on the issue.

Until the DNA test comes back, its probable, but based on observed Myotonia, history, other symptoms, and increased CK enzyme levels. It is (and its a long one!): Adult onset Classic form type 1 Myotonic Muscular Dystrophy.

It is likely my mother was misdiagnosed with MS and COPD (local hospitals, general consultants, no imaging), and it was in fact the reason she was in a wheelchair by age 50, and passed away from respiratory failure at 62. It also explains all the heart arrhythmias i have been having.

Fingers crossed of my other 2 sisters and 1 brother, who are 10 years older than me, they didn't inherit it. It gets worse and appears earlier in each generation apparently.

Funny thing was, 4 days in hospital, PET scans, lumbar punctures, more bloods, repeat EMGs etc... and it was a reflex test on my palm, that showed my muscles contracting and then taking a prolonged time to relax, which was the Myotonia. Its possible in a couple of conditions. But the others where ruled out.

Thanks all for the time and help though. Whilst im still facing a wheelchair in not to distant future and possibly 10-20 years shorter life expectancy, i am indeed very grateful for the time i will have.

P.S Nikki - you replied just as i was writing this update!
 

Nikki J

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Joined
Mar 22, 2012
Messages
10,988
Reason
PALS
Diagnosis
04/2014
Country
US
State
MA
City
Boston
I am glad that you have an answer. I hope for slow progression for you. Also there is such an explosion of genetic research that I have hope there will be treatments to help you some day

thank you for letting us know
 
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