Distal Motor Axonal Neuropathies: "Distal Lower Motor Neuron Syndromes"
Immune/Antibody
W
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Overview
From WUSTL NeuromuscularClinical Features Onset Age: 16 to 62 years Distribution: Initial symptoms usually in hands Weakness Distal > Proximal Asymmetric Arms > Legs Bulbar: Normal ± Fasciculations Sensory: Normal Tendon reflexes: Normal; Occasionally reduced or increased Time course: Slowly progressive (years) Laboratory Electrophysiology NCV Axonal Loss (Asymmetric): Small CMAPs Occasional signs of demyelination: Mild; No conduction block Sensory: Normal EMG: Denervation Type: Acute & Chronic Distribution: Distal; Not paraspinous Serum Autoantibodies IgM vs GM1 (50%) IgM vs NS6S (45%) IgG vs GM1: Occasional patient
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
Demyelinating ataxic neuropathy with IgM binding to GalNAc-GD1a and GM2 gangliosides
GD1AACUTECHRONIC
External Resources
Data sourced from the Washington University Neuromuscular Disease Center. For clinical use, always refer to primary sources.