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 or   Relapsing	Motor + Sensory Δ Slow NCV Conduction Block Distal Latency: Long Slow F-waves	Targets   β-tubulin   Heparan sulfate Class: IgM or IgG Frequency: 20%	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 Slow F-waves	?	?	T-cell immunosuppression   Prednisone HIG
MMN	Motor only   Distal > Proximal   Arms > Legs   Asymmetric Onset: 25 to 60 yrs Slowly progressive	Motor only   Conduction Block   Axonal Loss EMG: No paraspinous   denervation	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 Axonal 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 Slow NCV 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 Slow NCV Axonal 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	Slow NCV	Targets   GM2   GalNAc-GD1a Class: IgM	Common	HIG
Anti-   GalNAc-GD1a	Motor Distal Asymmetric Onset: Adult Slowly progressive	Minor changes Mostly axonal 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%	?

* Frequency based on testing by immunofixation methodology.

Alan Pestronk M.D. Box 8111 Neurology 660 South Euclid Avenue    St. Louis, MO 63110 9/11/2007

E-mail: pestronk@kids.wustl.edu Phone: 314-362-6981 Fax: 314-362-3752 References & More detail: http://www.neuro.wustl.edu/neuromuscular/