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.
Highlights
Guillain-Barresyndrome (GBS) is an immune-mediated disorder in the peripheral nervous system with inflammatory infiltration, demyelination and/or axonal damage as the main pathological features [1]
Depending on the involvement of nerve fiber types and the predominant nature of nerve injury, GBS can be divided into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motorsensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS) and some other relatively rare subtypes [1]
Subjects were divided into the ganglioside+ group and the ganglioside2 group
Summary
Guillain-Barresyndrome (GBS) is an immune-mediated disorder in the peripheral nervous system with inflammatory infiltration, demyelination and/or axonal damage as the main pathological features [1]. Depending on the involvement of nerve fiber types (sensory, motor and autonomic) and the predominant nature of nerve injury (demyelination or axonal degeneration), GBS can be divided into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motorsensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS) and some other relatively rare subtypes [1]. Immunohistochemical studies on AMAN revealed antibodymediated damage to the motor axonal membrane suggestive of an immune response directed against the motor axolemma [3,4]. AMASN is another axonal subtype of GBS in which axonal degeneration involves both motor and sensory nerves [1].
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