9. Diagnostic criteria
Mandatory inclusion criteria:
• Progressive or relapsing muscle weakness
for 2 months or longer
• Symmetrical proximal and distal weakness
in upper and lower extremities
• Hyporeflexia or areflexia
5/24/2017 9
10. Mandatory exclusion criteria:
• Evidence of relevant systemic disease or toxic
exposure
• Family history of neuropathy
• Nerve biopsy findings incompatible with
diagnosis
Diagnostic criteria……cont.
5/24/2017 10
11. Diagnostic criteria……cont.
Mandatory laboratory criteria:
• Nerve conduction studies with features of
demyelination (motor nerve conduction <70% of
lower limit of normal)
• Cerebrospinal fluid protein level>45 mg/dl,
cell count<10/µl
• Sural nerve biopsy with features of demyelination
and remyelination including myelinated fiber loss
and perivascular inflammation5/24/2017 11
12. Diagnostic categories
Definite: Mandatory inclusion and exclusion
criteria and laboratory criteria
Probable: Mandatory inclusion and exclusion
criteria and two of the three laboratory criteria
Possible: Mandatory inclusion and exclusion
criteria and one of three laboratory criteria
5/24/2017 12
13. Associations
CIDP is a syndrome presented in many diseases
HIV infections
SLE
DM
MGUS
Plasma cell dyscrasia
Chronic active hepatitis
IBD
Hodgkin lymphoma
Hashimoto’s thyroiditis
Drugs
5/24/2017 13
14. Differentials
• AIDP
• Sub-acute GBS
• Multifocal motor neuropathy
• Amyotrophic lateral sclerosis
• Hereditary motor and sensory neuropathy
5/24/2017 14
15. Features CIDP MMN ALS
Weakness Proximal=distal
Usually
Symmetrical
Distal>proximal
Asymmetrical
Distal>Proximal
UML sign --- --- +++
Sensory loss +++ --- ----
Motor conduction
block
Frequent Frequent Absent
Sensory Low SNAPs Normal Normal
CSF protein Elevated Normal >70% Normal
5/24/2017 15
16. Investigations
Nerve conduction study:
a) Reduction in motor conduction velocities in at least
two motor nerves
b) Partial conduction block or abnormal temporal
dispersion in at least one motor nerve at non
entrapment sites
c) Prolong distal latencies in at least two motor nerves
d) Absent F waves or prolonged F wave latencies in at
least two motor nerves
5/24/2017 16
18. Figure. Sagittal (A through C) and parasagittal MR slices of the lumbar spine in a patient with
chronic inflammatory demyelinating polyradiculoneuropathy.
Gerd Diederichs et al. Neurology 2007;68:701
5/24/2017 18
MRI:
19. Nerve biopsy:
1) Moderate reduction in
myelinated fibres
2) Endoneural and
subperineural edema
3) Segmental demyelination and
remyelination
demyeli
48%
axonal
21%
mixed
13%
Normal
18%
5/24/2017 19
20. Others investigations
CBC with ESR
FBS,HbA1C
HIV antibody
CXR
Hepatitis profiles
Thyroid function test
Serum and urine immunoelectrophoresis and immunofixation
Skeletal bone survey
ACE
ANA
LFT
RFT
Vasculitic screening
5/24/2017 20
21. Treatment
When to initiate treatment?
Progression is rapid or walking is compromised
(significant functional deficit)
Treatment options:
1) IV Ig
2) Plasma exchange
3) Corticosteroid
4) Other Immunosuppressive agents
5/24/2017 21
22. IVIg:
• 2gm/kg body wt given in divided doses over 2-5
days;
• three monthly courses are recommended
• Gradually reduce dose and increase gap between
the doses………….. interval according to response
Plasma exchange:
Initiated two to three treatments per week for 6
weeks
5/24/2017 22
Treatment….cont.
23. Treatment….cont.
Corticosteroid:
60 -80 mg Prednisolone PO daily for 1 -2 months
Continue high dose until a remission or plateau
gradual reduction of 10 mg per month …patient may
need 10 to 30 mg alternate day steroid for 2 to 3 years to
prevent relapse
Pulse steroid few side effect but equally effective (40dexa
40mg/day for 4 days…….then repeat 4 weekly
5/24/2017 23
24. IV Ig PE Steroid
Response rate 70 80 65
Response
speed
++++ +++ ++
Relapse +++ +++ _
Cost
Complication
(immediate
and late)
5/24/2017 24
29. Plasma exchange
40-50 ml/kg three times over a week
Adverse effects
Need for large bore venous access
Pneumothorax
Hypotension
Arrhythmia
Electrolyte imbalance
Potential exposure to blood products
Suppression of the patient's immune system
Autonomic instability
Hypocalcemia
Bleeding due to depletion of clotting factors
5/24/2017 29
30. Prognosis
50% severely disabled at some point of disease
10% patients remain disabled in spite of treatment
95% respond with improvement after immunosuppressive therapy
40% remained in partial or complete remission without any medication
62%
26%
12%
Good Failed to respond Died
Six years after
treatment
5/24/2017 30
31. References
• Harrison’s Neurology in clinical medicine – 4th edition
• Bradley’s Neurology in clinical practice – 6th edition
• Adams and Victor’s Principles of Neurology – 10th edition
• Davidson’s principles and practice of medicine – 22nd edition
• Medscape
• www.aan.com
• Google Image
5/24/2017 31
Incidence less than GBS but prevalence more than GBS………ten times more in DM both insu dep and non insu dep…….
Monophasic and progressive all….,,,steo wise and progressive…….33%
Manifestation extremely variable, proximal equal distal. Not length dependent, both upper and lower limb involved. Sensory…tingling and numbnesss most prominent but sometimes pain may occur…. Preceding infection (infrequent)
Initial limb weakness, both proximal and distal
Sensory symptoms (eg, tingling and numbness of hands and feet)
Motor symptoms (usually predominant)
In about 16% of patients, a relatively acute or subacute onset of symptoms
Symmetri, proxi equal distal….stocking glove
Multifocal acquired Demyelinating sensory and motor ……………..asymmetricall
Distal acquired symmetrical Demyelinating neuropathy…………..Ataxia may present in sensory
Associateion found about 25% cases………………………tacrolimus, macrolides, etaneercept, infiximab, adlimumab………. ………drug after 2 weeks to 16 weeks
Family history, bony abonormalities, pes cavas, hammer toe, proximal more than distal muscle weakness……MGUS….older, more protracted course, less response to immunotherapy…
Evidence of axonal loss due to prolong demyelination can be found in 50% case…..have a poor prognosis……….. At least three criteria are necessary to diagnosis CIDP
45---250…1000 papilloedema………….elevated gamma globulin and lymphocytic pleocytisis in 10% cases …………. and level above 100mg/dl are common
Pleocytosis if hiv
Figure. Sagittal (A through C) and parasagittal MR slices of the lumbar spine in a patient with chronic inflammatory demyelinating polyradiculoneuropathy. (A) T2-weighted image, showing lack of regular fluid-isointense signal, due to swollen cauda equina fibers (arrows). (B) Corresponding T1-weighted, fat-saturated image. Diffuse cauda equina enhancement (arrows) is depicted, indicating inflammation. (C) Parasagittal T1-weighted, fat-saturated image. Enlarged and enhancing root fibers are shown, exiting the neuroforamen (arrows).
Semithin section
Mild Disorder : Awating spontaneous remission
50% achieve plateau in 6 months….steroid side effect sodium not more2 gm/day, ppi , ca, vit d, cat, bisphosphonates
One third become resistant to ivig andd pe after repeated use in 1 or more years
For resistant cases, no RCT evidence………..high dose cyclo following GCSF shown promising result in resistant cases
