title: More severe manifestations and poorer short-term prognosis of ganglioside-associated Guillain-Barré syndrome in Northeast China.
abstract: Ganglioside as a neurotrophic drug has been hitherto widely used in China, although Guillain-Barré syndrome (GBS) following intravenous ganglioside treatment was reported in Europe several decades ago. We identified 7 patients who developed GBS after intravenous use of gangliosides (ganglioside+ group) and compared their clinical data with those of 77 non-ganglioside-associated GBS patients (ganglioside- group) in 2013, aiming at gaining the distinct features of ganglioside-associated GBS. Although the mean age, protein levels in cerebrospinal fluid (CSF) and frequency of cranial nerve involvement were similar between the two groups, the Hughes Functional Grading Scale (HFGS) score and the Medical Research Council (MRC) sum score at nadir significantly differed (4.9 ± 0.4 vs 3.6 ± 1.0; 7.7 ± 5.5 vs 36.9 ± 14.5, both p<0.001), indicating a higher disease severity of ganglioside-associated GBS. A higher ratio of patients with ganglioside-associated GBS required mechanical ventilation (85.7% vs 15.6%, p<0.01). The short-term prognosis of ganglioside-associated GBS, as measured by the HFGS score and the MRC sum score at discharge, was poorer (4.3 ± 0.5 vs 2.8 ± 1.1; 17.3 ± 12.9 vs 46.0 ± 13.9, both p<0.001). All the patients in the ganglioside+ group presented an axonal form of GBS, namely acute motor axonal neuropathy (AMAN). When compared with the AMAN patients in the ganglioside- group, more severe functional deficits at nadir and poorer recovery after standard treatment were still prominent in ganglioside-associated GBS. Anti-GM1 and anti-GT1a antibodies were detectable in patients with AMAN while not in patients with the demyelinating subtype of GBS. The concentrations of these antibodies in patients with AMAN were insignificantly different between the ganglioside+ and ganglioside- groups. In sum, ganglioside-associated GBS may be a devastating side effect of intravenous use of gangliosides, which usually manifests a more severe clinical course and poorer outcome.
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Xiujuan
authors.initials: X
authors.lastname: Wu
authors.affiliation: Department of Neurosurgery, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Wu
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Zhengzheng
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Donghui
authors.initials: D
authors.lastname: Shen
authors.affiliation: School of Public Health, Jilin University, Changchun, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Pan
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Norman Bethune Health Science Center, Jilin University, Changchun, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Neuroprotection Research Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, United States of America.
authors.forename: Limin
authors.initials: L
authors.lastname: Wu
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Xiaokun
authors.initials: X
authors.lastname: Wu
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Jiachun
authors.initials: J
authors.lastname: Feng
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China.
authors.forename: Kangding
authors.initials: K
authors.lastname: Liu
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden.
authors.forename: Jie
authors.initials: J
authors.lastname: Zhu
authors.affiliation: Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden.
authors.forename: Hong-Liang
authors.initials: HL
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000906:Antibodies / Q000097:blood / Q000134:cerebrospinal fluid; D001369:Axons / Q000473:pathology; D002681:China; D003710:Demography; D055724:Electrophysiological Phenomena; D005260:Female; D005732:Gangliosides / Q000009:adverse effects* / Q000276:immunology; D020275:Guillain-Barre Syndrome / Q000097:blood / Q000134:cerebrospinal fluid / Q000139:chemically induced* / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013997:Time Factors; D055815:Young Adult
chemical_list: D000906:Antibodies; D005732:Gangliosides
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