Immune Sytem + CMT

[Guest guest]

Amelia,

Here's an article from last April on CMT and the Immune System. You

might also search through our archives for this information in

past posts. Ruth is one of our members, although I did not find

her mentioned in Paolo's book. ~ Gretchen

1: J Anat 2002 Apr;200(4):405-14

Role of immune cells in animal models for inherited neuropathies: facts

and visions.

Maurer M, Kobsar I, Berghoff M, Schmid CD, Carenini S, i R.

Department of Neurology, University of Wurzburg, Germany.

Mice heterozygously deficient in the peripheral myelin adhesion molecule

P0 (P0+/- mice) are models for some forms of Charcot-Marie-Tooth (CMT)

neuropathies. In addition to the characteristic hallmarks of

demyelination, elevated numbers of CD8-positive T-lymphocytes and

F4/80-positive macrophages are striking features in the nerves of these

mice. These immune cells increase in number with age and progress of

demyelination, suggesting that they might be functionally related to

myelin damage. In order to investigate the pathogenetic role of

lymphocytes, the myelin mutants were cross-bred with recombination

activating gene 1 (RAG-1)-deficient mice, which lack mature T- and

B-lymphocytes. The immunodeficient myelin mutants showed a less severe

myelin degeneration. The beneficial effect of lymphocyte-deficiency was

reversible, since demyelination worsened in immunodeficient

myelin-mutants when reconstituted with bone marrow from wild-type mice.

Ultrastructural analysis revealed macrophages in close apposition to

myelin and demyelinated axons. We therefore cross-bred the P0+/- mice

with spontaneous osteopetrotic (op) mutants deficient in the

macrophage colony-stimulating factor (M-CSF), hence displaying impaired

macrophage activation. In the corresponding double mutants the numbers

of macrophages were not elevated in the peripheral nerves, and the

demyelinating phenotype was less severe than in the genuine P0+/- mice,

demonstrating that macrophages are also functionally involved in the

pathogenesis of genetically mediated demyelination. We also

examined other models for inherited neuropathies for a possible

involvement of immune cells. We chose mice deficient in the gap junction

component connexin 32, a model for the X-linked form of CMT. Similar to

P0-deficient mice, T-lymphocytes and macrophages were elevated and

macrophages showed a close apposition to degenerating myelin. We

conclude that the involvement of T-lymphocytes and macrophages is a

common pathogenetic feature in various forms of slowly progressive

inherited neuropathies.

[Guest guest]

Gretchen,

Thank you so much for the article on CMT and the immune system. I will take

it with me to my doctor tomorrow. I found it very interesting on your posts

that you have been hospitalized for IV antibiotic therapy to knock out

infections; I have had 2 hospitalizations recently for infections requiring

IV antibiotics. Were your erythrocyte sedimentation rates (ESR) elevated?

Do you know what your WBC's were? Any information would be helpful for me.

Thank you so much.

Amelia

[Guest guest]

Amelia,

I'm glad that article helped. Yes, last year I scratched myself on the

leg on sharp coral while snorkeling. I put lime in it and it healed

fine. 2 weeks after I was home, I woke up with red streaks from my foot

to my thigh. It took the docs a few days to figure out what to give me

for it. The first antibiotics did nothing for 3 days. Then I had IV

Rocephin which knocked it out. I never asked about by ESR or my WBC. It

was pretty obvious I had some form of blood poisioning, and the

Infectious Disease doc said it was from the coral. Apparently, the live

coral had got in my system, then the slight wound healed - so that it

why it didn't happen right away. After that hospital stay I was well

rested, but weak from the strong antibiotics. Feel free to email me if

you want: liliwigg@...

~ Gretchen