62

Journal MONONUCLEAR

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 .

66 TABLE 4

p 0 00

n

IMMUNOGLOBULIN CLEAR CELLS

PRODUCING

CELLS/105

p

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...
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