t tMSN 1 l'NS-myclin lg(; I'BI. BM IgA PBI, BM IgM PBI. BM P, lg(; I'IH.
C E L L S 1N R t ' : S P O N S E T ( ) I ) I F F E R f ' N T Polyncuropathy
0.7 + O.g f 0.4 411.3 47 H I.g 4-
1.7 I.~'~ 0.7 0.5
(ontrols
2.5
() (l.8 1.0 0.6 0,3 0.5
+ + + + +
I.~ 3.0 {I.7 (l.5 0.7
nd nd nd nd lid nd nd
2.2 +
2.5
14.4 +
25.5
I~M
4.7 ±
~.8
1.6 +
2.1
nd
lgA
Pl{l.
1.2 ±
2.2
5.0 +
1(I.4
nd
IgM
I'}M I'BI, la;IM
I.B + (I.1 I1.3 +
1.7 0.3 1.7
1.3 + 0.6 + 0.5 +
1.5 II.g (l.9
nd nd nd
I'IH,
3.0+
5.7
13.1+
21.(}
BM
2.3 ±
2.5
1.6 +
2.1
MA(; lg(;
('SF lgA lgM MI'H' lg(; lgA lgM
~76
± 716
48")
+ 835
N[-R'V[:, A N T I ( i F , NS
1.4+
2.n
nd 31).l) ~ 37.7
I'BI.
0.9 +
2.11
3.4 +
7.``)
l}kl
25) ±
3.6
1.8 +
I.~
nd
0.g 4
1'1{I, BM
1.(I + 2.9 ±
1.1 3.3
0.7 + 0.7 +
1.2 1.3
(1,3 + (L7 nd
Pl'll.
1.4 4`
1.9
5.5 +
7.S
BM
5.1 4-
7.5
2.4 +
5.3
I'BL
().7 ±
0.7
3.2 +
~.0
BM
2.1 +
2.7
I).~ +
0.7
nd
PI'H, BM
11.4 + 3.6 ±
0.7 7.2
0.4 + 0.2 +
11.5 ().4
fl.-I ; nd
1.5
l.fl 4
1.9
nd n.3 ~
[LS I.!)
S i g n i t i c a n t h raisud ( > + 2 S D ) v k l h l e s ¢IFC t m d c r l i n e d .
spccific peripheral nerve antigens was more pronounccd m blood than in bone marrow due to the higher frequency of T-culls in blood.
Peripheral 0
30 ¸
Z
• [] []
HMSN I Po yneuropaPy Corqtro!s
Myelin anti,~en reactire B ('ells The number of cells secreting antibodies against the different antigens tested arc summarizcd in Tablc 3
blood
1400
o
f---t
B.
1200
1000
o
o
2O
800
• [] •
o
HMSN Polyneuropathy Controls
o
o
o Q_
6O0
i
g
g
fluid
z
g g
Cerebrospinal
10
m
i PNS
P2
MAG
i MBP
~o
400
o._
200
M A G
Fig I. A: lgG producing c u l l s / I H ~ mommuclcar cells (mean + SD) in peripheral blood m response to differem nerve amigcns. B: lg(i produchlg c u l l s / I l l " n l o n o n u c l u a r culls ( m e a n + S D ) in c e r e b r o s p i n a l fluid in r e s p o n s e to M A ( ] . S i g n i f i c a n t ditference~; a r e m a r k u d :+ P • li.(}5, * :+: f ' . 0 . 0 l , ~:** P < ().(i01. M N ( " m o n o n u c l e a r culls, P N S = p e r i p h e r a l nelwotls s y s t e m myelin, M A ( I - nlyelin a s s o c i a l e d giyc~,~plotchl, M B P myelin basic p r o t e i n .
Increased systemic B- and T-lymphocyte responses in hereditary motor and sensory neuropathy (HMSN I).
Immune mechanisms of possible importance for the development and maintenance of peripheral nerve myelin breakdown in HMSN I were analysed by measuring...