CIDP

1
CIDP

• 2001? ????

2
CIDP

• ??????????????
• ????-?????
• ???????????

3
CIDP
?????
1
?????MRI
2
?????????
3
???????
4
4
????
DW3
B7
HLAA
CIDP
DRw3
DR2
5
????
?????
CIDP
6
????

• ?????????????

• Schwann????,????????

7
(No Transcript)
8
(No Transcript)
9
(No Transcript)
10
????
T 5,000 ? 22,000?
2-7.7/100,000.
CIDP
1.44.7
5060?????
?????
????
?????????
11
????
????????????
?????
??????
??????????????? ????????????????
?????????????? ??????,????? ????????????
MRI?????????? ?????????? ??
???? ?? ????? ???????
12
???? Dyck

• ?????15
• ?????15
• ?????34
• ??????34

13
MRI

• CIDP is a chronic progressive polyradiculoneuropat
  hy. On MRI
• the cranial nerves and nerve roots can be
  enhancing and prominently enlarged. In some
  cases, involvement of cranial nerves VII and VIII
  can simulate bilateral acoustic neuromas seen in
  NF2.
• Enlargement of the spinal nerve roots can
  simulate multiple neurofibromas seen in NF1.
• Patients with CIDP tend to have a slow
  progressive clinical course that is often
  misdiagnosed initially.

14
MRI

• 41-year-old man with leg weakness, decreased
  dexterity in hands, and numbness in umbilical and
  perineal regions.

15
MRI
16
MRI
17
??
CIDP
??
18
????
Nicolas ?Thaisetthawatkul
??/????
INCAT ?????????? ????????
AAN ????????
19
ANN

• ? AAN ???, CIDP ???????,??????,????????????,?????
  ???,??????????

20
ANN

• A. ???CIDP????
• 1. ????(mandatory)(1)????????????????????????
  ?-????(???????????),???2??????(2)????????(?????)
  2. ????(exclusive)(1)???????(mutilation),???????
  ????,?????????????????,?????????????(2)??(???)??
  ??(sensory level)(3)????????

21
ANN

• B. ???CIDP?????1. ????CIDP?????2.
  ?????(????)(1)???lt10/mm3(??HIV???)lt50/mm3(??HIV?
  ??)(2)????(VDRL)??C. ??CIDP?????1.
  ?????CIDP?????2. ????(1)??????????????????????,
  ?????(5??????)??????(50??,???4????????????gt12)
  (2)???????(???????????)???????Schmann????????????
  ???(?????????,????????????????????)????????????

22
ANN

• ???????1. ?????????????????????,??????????,????4
  ????3?(1)2?????????????1)?????????80????????
  ??????80??2)?????????80????????????70??(2)??
  ?????????????????????????(temporal
  dispersion)???????????,??????????,???????????1
  )??????(partial conduction block)??????????????
  lt15,????????????????????gt20?2)???????????(abnor
  mal temporal dispersion)???????(possible
  conduction block)??????????????gt15,??????????
  ?????????gt20,??????????????(3)2?????????????1)?
  ?????????80???????????????1252)??????????80?
  ??????????????150(4)2???????F??????F??????(101
  5???)1)??????????80,?F??????????????1202)?????
  ?????80,?F??????????????150

23
ANA???

• ?????,???1/3-2/3?CIDP???,?????CIDP??????

24
??????????? ???????? INCAT
????????????? ??????????????
INCAT
???????2????
?????????
25
INCAT
26
INCAT

• ??????????48?CIDP???????????????,? 21
  ????????? CIDP ? ??????????????????????????
  ?????????,????????? CIDP?????? ???????????????????
  ??? ,??????????

27
CIDP?????(?)
28
CIDP????(?)
29
(No Transcript)
30
CIDP????(?)
AIDP 3A?
????? 1?
???????? 2?
????? 4?
??? 3?
CIDP
??????? 2?
???? 1?
31
(No Transcript)
32
??????
33
??????
34
CIDP????
???
????????????
CIDP
??
Sjogren ???
????
AIDS
???
35
?????CIDP????CIDP
???????,I- CIDP ???DM - CIDP ????????
36
CIDP????
MASAM
37
???CIDP

• CIDP ????????????????? CIDP ????????,??????????,
  ?????????

38
????????(MMN)

• MMN ????CIDP ?????????????? ? CIDP ?????,MMN
  ?????????????????????, ?????????? ??, MMN
  ????IVIg ??????????
• ?????,???-??????MMN???? IVIg ?????,????????
  IVIg???????? ???, Van den Berg?40???MMN??,??????,?
  ?,???-??? ?????,Federico ?41???,?IVIg
  ?????,16????? 11?(69)????????????????????????????
  ??????? ?????????MMN?????????????????,
  ???????CIDP?????

39
????????????????? (MADSAM)

• MADSAM ??????, ?????Lewis-Sumner??????????????????
  ???MADSAM ??? ?????????????????, ??????
  ,????,????????? , ??????, F- ??????

40
??
?????100mg/d,24????
????40mg/d,????4d??20mg/d,?12d10mg/d,?12d,28d???
??

????IVIG??0.4g/kg,??5d
????(PE)??????????
41
IVIGMPPT

• ????????IVIGMPPT????CIDP?,???????????????MPPT?
• MPPT?????????????????,?????????????
• IVIG?IG?75,????????????,???????????????,?????????
  ????????????????,????????????????,??????????????

42
.

• high-dose intermittent intravenous
  methylprednisolone.
• ???????????????

43
high-dose intermittent intravenous
methylprednisolone.

• A retrospective medical record review between
  1992 and 2003 of outcomes in CIDP, comparing
  patients in 3 cohorts depending on whether their
  primary treatment was IVMP, IVIg, or oral
  immunosuppression with prednisone or
  cyclosporine. SETTING Washington University
  Neuromuscular Disease Center (St Louis, Mo),
  outpatient and inpatient records.
• PATIENTS Patients with clinical and
  electrophysiologic evidence of CIDP were
  identified. Of 57 patients, 39 had sufficient
  data for full analysis. MAIN OUTCOME MEASURES
  Quantitative muscle testing with a handheld
  dynamometer. Medication profiles and adverse
  effects were also recorded. RESULTS There was no
  significant difference in the mean improvement in
  quantitative muscle testing at 6 months or at the
  last clinic visit (an average of 4.5 years later)
  among the 3 groups. Fewer patients treated with
  oral immunosuppression improved at 6 months, but
  at the last visit, 81 to 88 improved in all 3
  groups. Less weight gain and fewer cushingoid
  features affected patients treated with IVMP
  (19) compared with patients treated with oral
  prednisone (58). CONCLUSIONS Treatment of
  patients with CIDP using high-dose intermittent
  IVMP results in improved strength equal to that
  with IVIg and oral prednisone. The frequency of
  occurrences of weight gain and cushingoid
  features with IVMP is less than that with oral
  prednisone. Intravenous methylprednisolone should
  be considered for initial and long-term therapy
  in CIDP when patients have disability due to
  weakness.

44
????
6???4.5??????
?????
IVMP
IVIG
?
????,????
????,????
8188?????????
45

• IVMP????CIDP???????

46
CIDP???(52?)

• ????

• ???????

???????
??????

• ???219??

47
Thank You !