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.

• Authors: Sayuri Shima, Naoki Kawamura, Tomomasa Ishikawa, Hiromi Masuda, Chihiro Iwahara, Yoshiki Niimi, Akihiro Ueda, Kazuhiza Iwabuchi, Tatsuro Mutoh
• Journal: Neurology (Neurology) Vol. 82 Issue 2 Pg. 114-8 (Jan 14 2014) ISSN: 1526-632X [Electronic] United States
• PMID: 24319040 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antibodies
• Autoantibodies
• Glycosphingolipids
• Immunoglobulins, Intravenous

Topics

• Adult
• Aged
• Antibodies (analysis)
• Autoantibodies (analysis)
• Brain (diagnostic imaging, pathology)
• Evoked Potentials, Somatosensory (physiology)
• Facial Paralysis (etiology)
• Female
• Glycosphingolipids (immunology)
• Humans
• Immunoblotting
• Immunoglobulins, Intravenous (therapeutic use)
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Muscle Weakness (etiology)
• Neural Conduction
• Neurologic Examination
• Polyradiculoneuropathy, Chronic Inflammatory Demyelinating (diagnostic imaging, immunology, therapy)
• Spinal Cord (pathology)
• Surface Plasmon Resonance
• Tomography, Emission-Computed, Single-Photon
• Treatment Outcome