| 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 |