Anti-neutral glycolipid antibodies in encephalomyeloradiculoneuropathy.

Abstract:

OBJECTIVE:The aim of this study was to review 4 patients with encephalomyeloradiculoneuropathy (EMRN) and assess for autoantibodies against neutral glycolipids. METHODS:We studied the progression of clinical, radiologic, neurophysiologic, and CSF findings, as well as anti-neutral glycolipid antibodies in sera. RESULTS:All patients developed acute or subacute motor weakness and impaired consciousness. Their CSF showed pleocytosis and high immunoglobulin G concentrations. MRI revealed lesions in the brain and spinal cord. Neurophysiologic examinations indicated dysfunction of the spinal cord, nerve roots, and peripheral nerves. Steroid pulsed immunotherapy and/or high dose of IV immunoglobulin replacement therapy resulted in clear and often dramatic clinical improvements. Reactivity to anti-neutral glycolipid antibodies was positive in all patients with acute EMRN but not in the recovery phase. Forty-seven age-matched patients with other neurologic disorders and 28 age-matched healthy volunteers tested negative for reactivity to anti-neutral glycolipid antibodies. CONCLUSION:The resolution of radiologic and neurologic abnormalities and altered autoantibody titers against neutral glycolipids after immunotherapy suggest that EMRN is caused by an immune-mediated mechanism. These autoantibodies may be useful biomarkers for EMRN.

journal_name

Neurology

journal_title

Neurology

authors

Shima S,Kawamura N,Ishikawa T,Masuda H,Iwahara C,Niimi Y,Ueda A,Iwabuchi K,Mutoh T

doi

10.1212/WNL.0000000000000015

subject

Has Abstract

pub_date

2014-01-14 00:00:00

pages

114-8

issue

2

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000000015

journal_volume

82

pub_type

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