Entrapment in anti myelin-associated glycoprotein neuropathy.
Anti-myelin associated glycoprotein (MAG) neuropathy is a chronic disorder in which IgM antibodies react with Schwann cell glycoproteins, including MAG and peripheral myelin protein 22 (PMP22). Nerve conduction studies show features of axon loss and predominantly distal slowing consistent with demyelination. Because a genetic loss of PMP22 function yields hereditary neuropathy with liability to pressure palsies (HNPP), loss of PMP22 function due to anti- MAG antibodies may result in increased sensitivity to entrapment. We investigated this by performing standardized electrophysiological studies in 16 patients with anti-MAG neuropathy and 16 disease controls with genetically confirmed HNPP. Disproportionate slowing relative to adjacent segments occurred in similar proportions of patients with anti-MAG neuropathy and HNPP, and was of the same magnitude in each group. Affected were the elbow, carpal tunnel and the wrist-hand segments of the median and ulnar nerves. However, in anti-MAG neuropathy as compared to HNPP, absolute values of distal motor latencies and conduction velocities outside entrapment sites were slower and amplitudes were lower. In conclusion, increased sensitivity for entrapment may occur in anti-MAG neuropathy and contribute to part of the nerve damage.
Faber
Catharina G
CG
Dept. of Neurology, University Hospital Maastricht, 5800, 6202 AZ, Maastricht, The Netherlands. c.faber@mumc.nl
Notermans
Nicolette C
NC
Wokke
John H J
JH
Franssen
Hessel
H
D000328:Adult; D001132:Arm / Q000294:innervation; D001323:Autoantibodies / Q000378:metabolism*; D004576:Electromyography; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D007866:Leg / Q000294:innervation; D008875:Middle Aged; D009185:Myelin Proteins / Q000378:metabolism; D018992:Myelin-Associated Glycoprotein / Q000378:metabolism*; D009408:Nerve Compression Syndromes / Q000150:complications*; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000276:immunology; D011129:Polyradiculoneuropathy / Q000150:complications* / Q000276:immunology; D012583:Schwann Cells / Q000276:immunology
D001323:Autoantibodies; D007075:Immunoglobulin M; D009185:Myelin Proteins; D018992:Myelin-Associated Glycoprotein; C073687:PMP22 protein, human