CHRONIC IMMUNE DEMYELINATING NEUROPATHIES:
COMPARATIVE FEATURES
Neuropathy Clinical Features Electrophysiology Antibody M-Protein Treatment
CIDP Motor > Sensory
Weakness:
  Proximal & Distal
  Symmetric
Onset: 1 to 80 yrs
Chronic/Relapsing
Motor + Sensory Δ
NCV: Slow
Conduction Block
Distal Latency: Long
F-waves: slow
Targets
  β-tubulin
  Heparan sulfate

Class: IgM or IgG
Frequency: 10%
15% T-cell immunosuppression
  Prednisone
  Cyclosporine A
  Methotrexate
HIG
Plasma Exchange
Multifocal
  CIDP
Chronic
Motor > Sensory
Weakness:
  Distal > Proximal
  Asymmetric
  Arms > Legs
Onset: 15 to 75 yrs
Motor + Sensory Δ
Slow NCV
Conduction Block
Distal Latency: Long
F-waves: Slow
? ? T-cell immunosuppression
  Prednisone
HIG
MMN Motor only
  Distal > Proximal
  Arms > Legs
  Asymmetric
Onset: 25 to 60 yrs
Slowly progressive
Motor only
  Conduction Block
  Axon Loss

EMG: Denervation with
  disease progression
Targets
  Co-GM1 or NP-9

Class: IgM

Frequency: 80%
20% HIG
B-cell immunosuppression
  Plasma Exchange +
    Cyclophosphamide

  Rituximab
Anti-MAG Sensory > Motor
Distal; Symmetric
Gait disorder
Tremor
Onset: > 50 yrs
Slowly progressive
Motor + Sensory Δ
Distal Latency: Long
Slow NCV
No conduction block
Axon Loss
Target: MAG

Class: IgM

Frequency: 100%
85% B-cell immunosuppression
  Plasma Exchange +
    Cyclophosphamide

  Rituximab
  ? Fludarabine
GALOP Gait Disorder
Sensory > Motor
Distal; Symmetric
Onset: > 50 yrs
Motor + Sensory Δ
Distal Latency: Long
NCV: Slow
No conduction block
Target
  Sulfatide in
    lipid membrane

Class: IgM
80% HIG
Plasma Exchange +
  Cyclophosphamide
Anti-Sulfatide Slowly progressive
Sensory > Motor
Distal; Symmetric
Onset: > 45 yrs
Motor + Sensory Δ
Distal Latency: Long
NCV: Slow
Axon Loss
Target
  Sulfatide

Class: IgM
90% HIG
Plasma Exchange +
  Cyclophosphamide
Anti-GM2 &
  GalNAc-GD1a
Sensory > Motor
Ataxia: Limb; Gait
Distal
Symmetric or
  Asymmetric
Onset: Adult
Slowly progressive
NCV: Slow Targets
  GM2
  GalNAc-GD1a

Class: IgM
Common HIG
Anti-
  GalNAc-GD1a
Motor
Distal
Asymmetric
Onset: Adult
Slowly progressive
Minor changes
Axon loss: Distal
Targets
  GalNAc-GD1a

Class: IgM
Rare HIG
Polyneuropathy
Organomegaly
Endocrinopathy
M-protein
Skin changes
Sensory & Motor
Symmetric
Onset: 25 to 60 yrs
Slow NCV
Axonal Loss
Target: ?

Class: IgA or IgG
90% ?
Neurofascin Sensory & Motor
Distal
Tremor
Onset: Adult
Progressive
NCV: Slow
Target
  Neurofascin

Class: IgG4
No
?
Contactin-1 Sensory & Motor
Distal or Diffuse
Onset: Adult, late
Progressive
Distal latency: Long
Conduction block
Target
  Contactin-1

Class: IgG
No Prednisone
Not HIG
Alan Pestronk M.D.
Box 8111 Neurology
660 South Euclid Avenue   
St. Louis, MO 63110

E-mail: pestronka@neuro.wustl.edu
Phone: 314-362-6981
Fax: 314-362-3752
References & More detail: http://www.neuro.wustl.edu/neuromuscular/antibody/pnimdem.html