Relationship between IgA PDN and anti-MAG PDN/MGUS associated demyelinating neuropath
AnonymousMarch 3, 2008 at 6:50 am
Following on from my thread about IVIg treatment after almost 15 years of my neuropathy I was struck by another thought as I was on the infusion pump.
My neurologist on ward rounds detailed the main clinical characterisitics of my PDN/MGUS associated neuropathy. “Mainly sensory, small fibre, mild or little weakness, symmetric, slowly progressive” he told them. He explained that I had read much about the neuropathy and monoclonal gammopathy!
Knowing that does not ease the present pain however.
The thought that struck me again was this.
Some IgA PDN/MGUS associated demyelinating neuropathy sufferers ‘can show features similar to anti-MAG IgM patients’. (Cocito et al 2003.)
A joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society reported in ‘Guidelines on management of paraproteinaemic demyelinating neuropathies’ that “a minority of patients with IgG or IgA PDN have the DADS clinical pheonotype (observable characterisitics) and associated electrophysiological features”.
Most patients with IgM PDN have DADS clinical phenotype most strongly associated with anti-MAG antibodies. The phenotype is distal (away from the centre point of attachment), acquired, demyelinating, symmetrical. It is chronic, slowly progressive, symmetric, predominantly sensory involvement, relatively mild or no weakness, ataxia and often tremor.
That description is very similar to my IgA PDN clinical and my experienced characteristics.
Mainly sensory, mild or little weakness, symmetric, slowly progressive. No tremor initially but I have some now, but that could be age related – now 80+ compared with 65 at onset.
Any comments please from experienced ant-MAG IgM folk who post, for example about Rituxan treatment etcetera.
AnonymousMarch 4, 2008 at 12:04 pm
Ken, this description for anti-MAG IgM neuropathy you mention and which I find in the literature fits me exactly: DADS. It affects feet and legs, hands and arms, is mostly sensory, progresses very slowly. Some motor nerves are also affected as is evident when I’m trying to walk up and down steps or push a shopping cart.
IGA PDN is still a mystery to me. As you well know there is not much to be found in the literature. By the way, did you ever read the book excerpt about it I posted some time ago? Did you know that there are more IgA antibodies being produced by our immune system than any other combined, most of them of the excretory type (IgA2) found on the mucuous membranes. The second type, IgA1, stays in circulation and makes up only about 10% of all the antibodies. I assume it’s this second type that is giving you all your problems.
I hope the IVIG is giving the relief you need. I was surprised to find out that you have not received any immuno-modulating treatment all those years. If IVIG does not help perhaps you may want to consider Rituximab as an alternative treatment.
If you’ve followed my story you probably know that IVIG has not helped me an awful lot and that I received Rituxan in October now waiting for results. My tongue is almost back to normal and a few days ago I noticed that I can feel the keys on the keyboard again with my fingertips. This is very encouraging. It just takes time and a lot of patience. A friend of mine in Switzerland with antiMAG IgM received Rituxan at the same time. He was told by his neurologist that it might be June before he sees any improvements. I’m scheduled for another round of Rituxan in about six weeks.
I am sorry you have to endure so much pain. Again, I hope you see some improvement soon.
AnonymousMarch 4, 2008 at 2:34 pm
[QUOTE]Did you know that there are more IgA antibodies being produced by our immune system than any other combined, most of them of the excretory type (IgA2) found on the mucuous membranes. The second type, IgA1, stays in circulation and makes up only about 10% of all the antibodies. I assume it’s this second type that is giving you all your problems.[/QUOTE]
Yes, I knew that IgA was the most common antibody but also that IgA is the rarest of the PDN/MGUS associated neuropathy group. That’s why there is little in the literature, there being so relatively few cases reported that it is difficult to gather any clear case comparisons.
Way back in 1998 when at last there was a certain diagnosis I had to accept what my neurologist believed then that possible treatments had to be balanced between benefits and risks. It’s much like our friend Allaug’s long pain/problem history from the late eighties to 15 years later when a haematologist/oncologist finally put his diagnostic finger on anti-MAG IgM PDN. Then her beneficial treatment with Rituximab after almost everything else before that diagnosis.
I believe that I have mentioned before that we have a good friend of yours here in Halifax, UK with an idiopathic pn 2 years longer than mine but that behaves almost precisely the same as mine. She is also getting loaded with pain but her body is 25 years younger than mine. I am godfather of their younger son, now a policeman.
I am in e-mail contact with my neurologist, by his ‘instruction’. At the weekend he responded to my week 1 report post IVIg within 30 hours and on a Sunday evening. I am keeping Rituximab in mind but cannot find any cases of IgA PDN being so treated.
AnonymousMarch 5, 2008 at 4:33 am
Would you mind telling me where your tremors are? I have CIDP and I have a constant tremor in my jaw and do have intention tremors in my arms – if I overuse them. As you may recall, my sensory involvement (in particular a dorsal root ganglionopathy) is worse than my motor.
AnonymousMarch 5, 2008 at 5:38 am
The tremor is slight so far in my hands but it could be age related.
When a final diagnosis was made in April 1998 (it all began in 1993 – there is a link to my website on my other recent thread here) I was asked to meet a Neurophysiologist at Leeds GI. He checked me thoroughly for tremor and there was none then.
Way back in the mid-eighties he had written a paper about IgM neuropathy. I have it somewhere. So I was not sure whether the check was for his research benefit!;)
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