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
Neurologyjournal_title
Neurologyauthors
Shima S,Kawamura N,Ishikawa T,Masuda H,Iwahara C,Niimi Y,Ueda A,Iwabuchi K,Mutoh Tdoi
10.1212/WNL.0000000000000015subject
Has Abstractpub_date
2014-01-14 00:00:00pages
114-8issue
2eissn
0028-3878issn
1526-632Xpii
WNL.0000000000000015journal_volume
82pub_type
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