Worried grandma

[Absolutely 11]

Hi, I am a 71yr old female with history of mixed connective tissue disorder. R handed. Active and well till March 24. Saw GP with proximal weakness- most apparent in R leg and R arm. Presumed to have polymyalgia: inflammatory markers nad. Didn't improve. Trial of steroids- not a good response. Refer rheumatology. Bloods ++ ? for trial of methotrexate. In interim saw GP again- sensory changes in both lower legs. Referred nerve conduction studies- normal. Rheumatology review- weakness and muscle wasting R leg. Refer EMG. By time I went for EMG walking distance decreased and muscle fasciculation in legs. Neurophysiologist lovely: tested all 4 limbs. Told me I needed neuro referral; MRI brain and spine.
Saw neurology Feb 25. Conclusion : neurodegenerative disorder all 4 limbs. When pressed gave a differential of inclusion body myositis and mnd.
Plan: diagnosis by exclusion. Have had more bloods; baseline respiratory function; MRI next week; Repeat EMG early May to include parasitical muscles and tongue;neuro review July!
I saw a lovely OT- I'm struggling to manage stairs; walk on flat. Physio: not till July! Saw a friend who is physio- do my stretches and breathing exercises. I still drive my automatic car.
Home is 2 floors with hubby, cat and dog. Daughter nearby.
I'm trying to be zen and watch my clear deterioration calmly, but am concerned I now have neck muscle fasciculation; poor core and July seems a long way off!

 

[Nikki J]

I am sorry. Waiting is stressful I know. Early May is very soon though and if you don’t hear anything from neurology your gp should be able to look at the results

Please do whatever is necessary to avoid falls OT and unofficial pt input is great to have and it sounds like you have family support.

Wishing you the best and please let us know what happens

 

[Absolutely 11]

Thank you for taking time to read and reply. I have good support, but feel frustrated at my declining mobility and slow progress towards " label"

 

[lgelb]

If you have the EMG report, we could be more helpful. IBM should show up differently from MND so not sure what this differential is based on. And MTX is certainly not the only rx that can be tried. See IBM dx criteria here:

Inclusion Body Myositis - StatPearls - NCBI Bookshelf

 

[Absolutely 11]

Update: MRI done. EMG repeat in 2weeks. In interim mobility deteriorated. Truncal stability poor. Can walk with rollator that OT left " just in case".
I don't have 1st EMG report, just neurophysiologist report that caused rheumatology to write that I " had an anterior horn disease"

 

[Absolutely 11]

EMG this morning: v.detailed. Tested all 4 limbs; paraspinal muscles; chest wall muscles; trapezius; sternocleidomastoids, tongue. Then did NCS .
No report. Will go to neuro in about a week. Pointed out my next scheduled appt will be July and I need to plan my life.....Dr replied that on receipt of his report he expects my appt will be expedited! Exhausted, but necessary.
Physio and OT coming to visit this week.

 

[Absolutely 11]

Update: have not heard from hospital or GP. However digging deep into NHS I found a letter from neurophysiologist to neurologist stating his conclusion that the electrophysiological findings are suggestive of generalised anterior horn disorder. Does that = confirmation of ALS?

 

[lgelb]

It is the most common condition in that spectrum, though there are also other MNDs and rare/post-polio syndromes, to name two.

 

[Absolutely 11]

Thank you. Polio is not in the equation, so now I must wait till someone applies a name to my condition. Not what anyone wants to hear, but if you know what disorder is altering your life, you can discuss, plan and get on with it. Limbo is not a peaceful place.

 

[Absolutely 11]