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Abdullah Al-Ajmi, BMBCh, FRCPC
Consultant Neurologist
Jahra Hospital
Kuwait
Stick to allotted time!
Will not cover all aspects of immune-mediated NP
9%
Duration ≥2 months
Duration < 2 months
+ve family history
EDX
&
CSF criteria fulfilled;
other causes excluded
EDX
criteria fulfilled (may be –ve in 1st 2 wks)
Consider hereditary NP
Consider CIDP
Consider GBS
CSF
MRI
15.7 ms
3.35 mV
26.5 m/s
Diagnosis of GBS :
http://www.galenusrevista.com/
https://neuromuscular.wustl.edu
Effectiveness of PE was reported from France
Effectiveness of PE was reported from North America
MFS was reported
The term Guillain–Barre syndrome was first used
Axonal form of GBS was reported
Management of
Guillain-Barre ́ Syndrome
Disease-modifying Treatment
- It is of greatest benefit if instituted within 7 days, and four exchanges have been shown to be superior to two.
At present, the treatment strategies for AMAN and AIDP do not differ in clinical practice.
GBS variants no RCTs
mEGOS — modified Erasmus GBS Outcome Score
Captures:
• Age
• Preceding diarrhoea
• GBS disability score 2 weeks after hospitalization
EGRIS — Erasmus GBS Respiratory Insufficiency Score
Captures at time of hospital admission:
• Days between clinical onset of weakness
• Facial and/or bulbar weakness
• MRC sum score
Scores range from 1 to 7 and predict the clinical outcome after 6 months; a higher score corresponds to worse prognosis.
The scoring system ranges from 0 to 7, with corresponding chances of respiratory insufficiency within the first week after hospital admission from 1% to 91%.
ΔIgG
Larger increase is associated with a better clinical outcome.
1958
Austin described cases of probable CIDP.
1975
Dyck et al introduced the name chronic inflammatory polyradiculoneuropathy and thus defined CIDP as a separate disease entity.
Epidemiology
>50%
Around 18%
Clinical
Subacute onset; motor and sensory
Symmetric; proximal and distal
Chronic onset; motor and sensory
Symmetric; usually proximal rather
than distal
IVIG, corticosteroids, and PLEX effective
Treatment response
In March 2018 SCIG was approved by the FDA for maintenance treatment in adults diagnosed with CIDP
No data predict the best candidates for subcutaneous or intravenous immunoglobulin
Motor
predominant
Distal predominant
(if MAG
antibodies not present)
Sensory
predominant
(including CISP)
Focal
4–10%
Epidemiology
4–35%
Around 1%
2–10%
8–15%
Chronic onset; motor more than sensory
Symmetric; proximal and distal
Chronic onset; sensory more than motor
Symmetric; distal rather than
proximal
Clinical
Chronic onset; sensory more than motor; sensory ataxia (in CISP)
Symmetric; distal rather than proximal; upper rather than lower limbs
Chronic onset; slow disease
progression; motor and sensory
Brachial or lumbosacral plexus or one or more peripheral nerves in one limb; proximal and distal;
upper and lower limbs
Chronic onset; motor and sensory
Asymmetric; distal rather than proximal; upper rather than lower
limbs
Treatment response
IVIG,corticosteroids, and PLEX effective
IVIG effective;
patients’ condition
might deteriorate after
corticosteroids
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NF140
NF186
References