CHRONIC IMMUNE-MEDIATED NEUROMUSCULAR DISORDERS

    General Treatment Strategies:  Based on diagnostic test results

Features of Diagnostic Testing Disease
Examples
Treatments
Commonly effective
Nerve
  conduction
Conduction  Block MMN
CIDP
IVIg
EMG Thoracic paraspinous denervation Motor neuropathy None
Antibodies IgM monoclonal Target antigen: Carbohydrate
Antibody location: Serum
MMN
MAG neuropathy
Sulfatide neuropathy
GALOP syndrome
Cyclophosphamide
Rituxan
NOT corticosteroids
IgG polyclonal Target antigen: Membrane protein
Antibody location: Serum
Myasthenia gravis
Lambert-Eaton
Neuromyotonia
? CIDP
Corticosteroids
Immunomodulation
  T-cell immunosuppression
Plasma Exchange
Intravenous immunoglobulin
Target antigen: Intracellular protein
Antibody location: Serum + CSF
Anti-Hu ganglionopathy None
Target antigen: Carbohydrate
Antibody location: Serum
Acute motor neuropathy
Miller-Fisher
IVIg
IgG monoclonal Target antigen: Unknown
Antibody location: Serum
POEMS Uncertain
General principle: Intermittent treatment is often as effective as daily therapy but usually has fewer side effects

SPECIFIC MEDICATIONS


Alan Pestronk MD
3/3/2003