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Demyelinating ataxic neuropathy with IgM binding to GalNAc-GD1a and GM2 gangliosides

Immune/Antibody
W
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antibody/pnimdem.html#Demyelinating ataxic neuropathy with IgM binding to GalNAc-G

Overview

From WUSTL Neuromuscular

Onset Adult Gait: Ataxia Sensory loss: Distal Clinical Sensory loss: Pan-modal; Symmetric; Distal; Romberg + Weakness: Mild; Distal; Symmetric or Asymmetric Cerebellar: Dysmetria; Wide based gait Tendon reflexes: Absent Course: Slowly progressive over years Laboratory Electrodiagnostic testing: Demyelinating neuropathy NCV: Slow; Usually 20 to 30 M/s Conduction block Serum M-protein: IgM κ Serum IgM binding: GalNAc-GD1a & GM2 gangliosides Treatment IVIg: Frequent dosing No benefit: Corticosteroids, Cyclophosphamide Also see Acute neuropathy with IgM binding to GalNAc-GD1a Acute motor neuropath

Related Conditions

Shared genes
"Classic" Guillain-Barré Syndrome
H131GD1A
Acute Immune Polyneuropathies
GD1APATHOLOGY
Acute Motor (Axonal) Neuropathy (AMAN)
IIGQ1BGM1
ACUTE NEUROPATHIES
GD1A
Antibodies vs Glycolipids & Glycoproteins
GM1MAGGQ1B
Chronic Immune Demyelinating Neuropathies: Variants
GD1AMOTORGM1
Classification of Acute Immune Neuropathies
GD1A
Distal Motor Axonal Neuropathies: "Distal Lower Motor Neuron Syndromes"
GD1A

External Resources

WUSTL Neuromuscular
Washington University Disease Center
PubMed
Biomedical Literature
GeneReviews
NCBI Expert-Authored Reviews
Orphanet
Portal for Rare Diseases
NORD
National Organization for Rare Disorders

Data sourced from the Washington University Neuromuscular Disease Center. For clinical use, always refer to primary sources.