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Recently, ganglioside complexes (GSCs) such as GD1a/GD1b, GD1a/GM1, GD1b/GT1b, GM1/GT1b, GQ1b/GM1 and GQ1b/GD1a have been shown as target antigens for serum antibodies in patients with Guillain–Barré syndrome (GBS)1 and Miller Fisher syndrome (MFS).2 Gangliosides may interact with each other to form a novel epitope, which serves as a target antigen for serum antibodies.1 In paticular, anti‐GD1a/GD1b IgG is reported to be associated with severe GBS and requirement of mechanical ventilation.1 However, there has been no previous case report describing GBS with anti‐GSC antibodies in detail. In this report, we present a patient with GBS having anti‐GD1a/GD1b antibody and investigated the clinical feature.
A 42‐year‐old man noticed weakness of the bilateral upper extremities 2 weeks after an episode of acute respiratory tract symptoms and diarrhoea. His symptoms further developed to dysarthria, dysphagia and tetraparesis, and he was admitted to the Department of Neurology, Ishikawa Prefecture Central Hospital, Kanazawa, Japan, 4 days after the onset of weakness. Neurological examination disclosed bilateral facial weakness, poor elevation of the soft palate, hoarseness, dysarthria, dysphagia, weakness of the tongue, flaccid tetraparesis (grade 4, Medical Research Council scale) and areflexia of deep tendon reflexes. He needed a wheelchair for transfer, and stomach tube for gastrogavage. Laboratory findings including cerebrospinal fluid (CSF) examination were normal, except for hypercapnia (PCO2 47.8 mm Hg) on blood gas analysis. Nerve conduction studies demonstrated a marked reduction of compound muscle action potentials (CMAP) with normal conduction velocity (CMAP was 2.87 mV and motor conduction velocity was 50.6 m/s in the right median nerve), but sensory nerves were normal. The MRI studies of the brain and spinal cord were normal. A diagnosis of GBS was made, and he was given intravenous immunoglobulin (IVIg; 400 mg/kg/day) and intravenous methylprednisolone (500 mg/day) for 5 days, according to the protocol used in the previously reported randomised trial.3 He underwent rehabilitation, and his symptoms gradually improved 1 week after admission. He could stand by himself 2 weeks after admission, and eat by himself without a stomach tube 1 month after admission. Nerve conduction studies still showed simple reduction of CMAPs 1 month after admission (CMAP was 1.21 mV and motor conduction velocity was 53.0 m/s in the right median nerve). At 2 months after admission, he could ambulate independently. He returned to work (English teacher at a high school) 3 months after admission.
The antibodies to gangliosides (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1b, GQ1b, GA1, Gal‐C, and GalNac‐GD1a) and GD1a/GD1b complex in the serum obtained on the first day of admission were examined by enzyme‐linked immunosorbent assay, as previously described.1,4 He was positive only to the antibody to GD1a/GD1b complex (anti‐GD1a/GD1b antibody).
Our patient showed acute progressive axonal motor polyneuropathy involving the cranial nerves 2 weeks after flu‐like symptoms. This condition fulfilled the established criteria of GBS, and the results of nerve conduction studies were classified as having acute motor axonal neuropathy (AMAN).5 Anti‐GD1a/GD1b antibody was detected in the acute‐phase serum; however, there were no antibodies to single gangliosides, including GD1a and GD1b.
In a recent report,1 8 of 100 patients with GBS had anti‐GD1a/GD1b antibodies, and three of these eight did not demonstrate any anti‐ganglioside antibodies. These eight patients with anti‐GD1a/GD1b antibody tended to have cranial nerve deficits and severe disabilities, and four of these patients required artificial ventilation.1 Of the three anti‐GD1a/GD1b antibody‐positive patients with available electrophysiological data, two showed an axonal neuropathy pattern, and the remaining one showed an equivocal pattern.1 Of the 12 patients with MFS, 7 had serum antibodies to some GSCs, and anti‐GSC antibodies might influence the clinical features, as sensory signs were infrequent in patients with anti‐GQ1b/GM1 antibody.2 These findings may support the theory that anti‐GSC antibodies correlate with a certain phenotype of GBS or MFS.
The clinical features of our patient were similar to those patients with anti‐GD1a/GD1b antibodies,1 such as AMAN‐type GBS with cranial nerve deficits and severe disability (the Hughes Functional Grading Scale at the peak of his disability was on grade 4). Although our patient did not require artificial ventilation, his hypercapnia suggested respiratory weakness. The patient received intravenous methylpredonisolone in addition to IVIg. This combination therapy might prevent his case from being aggravated to grade 5. However, a future large‐scale study will be needed to clarify this point.
Competing interests: None declared.
Informed consent was obtained for publication of the patient's details described in this report.