als or mmn?

Hi,
My brother was diagnosis with ALS last July and I've been posting here since then – he's young, has a young family, and is the main caregiver/child rearer of the family – his wife works full time.
His little kids adore him. Its been very tragic seeing him unable to lift up his little girl with one arm. I know many here have progressed way beyond that. Sorry, I'm just new to it, and its terribly distressing to see.

Anyway, my brother was initially diagnosed with MND – he's in the UK, where MND is basically what in the US is called ALS.
Because he’s young, and because he has only LMN symptoms, the UK protocol is to give 6 months of IVIG to see if it has any beneficial effect and, if so, to consider changing diagnosis e.g. to Multifocal Motor Neuropathy.

He’s had 4 IVIG treatments so far. His treatments will be reviewed in April and then the consultants will decide whether to continue with IVIG or not.
He had a positive response to his first IVIG – he regained the use of a finger and partial use of a muscle in his arm, both had been out of action for a year. The consultant said this was objective improvement, and suggested a possible reclassification as MMN.
However, he had had no further improvements and last week developed new symptoms which I'm worried about – shaky legs and a feeling that he's about to fall/legs are about to give way.
This makes me think maybe clonus? Which is UMN so would rule out MMN?
I know people on here are having a tough enough time,and I'm sorry for questions and botherments. But my brother isn't asking anyone anything – not his neurologists, doctors, consultants, no one. He’s afraid to think too much about it and i get the impression he's still hoping it will all just go away.
Well I hope so too, but meanwhile, I’d like to be prepared and informed so that if and when the time comes that he needs to know what to do and what's going on, he’s got somewhere to start from.
Partly I'm just sharing here and partly I’m asking for input and feedback.
Thank you for listening, You are all amazing brave and strong, hats off to everyone living with this diagnosis.

Hi
Without him going back to his neurologist to have the new symptoms evaluated it's really difficult to give you any feedback. When I developed umn symptoms in my arm my neurologist said it pointed away from MMN and the MND diagnosis was confirmed. But and it's a big but! The only person who can properly tell what those symptom mean is his specialist, with my neuro we have three monthly appointments but if I feel I don't need them I can cancel however when I ring up with a fresh problem he will usually fit me in within a fortnight.

If your brother won't go or discuss his problems there is not much you can do except support him. This illness takes away so much of our control over our lives and being able to make our own choices is essential. You can tell him that you are worried and would like him to go, after that it's up to him I am afraid even if you don't agree with him.

I wish you and your brother luck and hope the ivig still works for him.

Wendy x

The leg shakiness you are describing/feeling of legs about to give way sounds less like clonus to me than simple leg weakness, which can very much be an LMN sign.

The US protocol is different, as you probably know. My husband displays exclusively LMN signs but still got a "ALS in all reasonable probability" diagnosis. Now he has slow rather than hyperactive reflexes so we will go into another EMG in February, even with the diagnosis of ALS in hand since June 30th. So, protocols or not we are a bit where you are.

I think that the best thing to do is to let the Doctors go through whatever the plan is, using the drug for MMN and not second guessing it until you hear a final word from them. Personally, it actually helps me to regard our situation as in no way ambiguous. Getting into a dream world where this actually turned out to be MMN after all, or something else non fatal would only break my heart if it were snatched away again.

Everyone's approach is different though. Your brother sounds very different from my husband in how he is dealing with this but if it works for him now good.

Thanks Wendy and Lenore. I know, after I posted this I thought what am I actually asking? I know there's no way of knowing at the moment and we just have to wait, and I know my brother has to make his own choices about how he deals with things. I'm sorry, I think I Just wanted to share and to hear back from other people, and I really appreciate your taking the time to respond to my post.
I'll keep updating with any new info. Meanwhile warm wishes to both of you.