trisialoganglioside-gt1 and Muscle-Weakness

We report a 78-year-old man with Guillain-Barré syndrome (GBS) who showed upper limb dominant muscle weakness following an upper respiratory infection. He had no weakness in extraocular, oropharyngeal and neck muscles. Tendon reflexes were absent in his upper limbs. Electrophysiological studies suggested demyelination of motor nerves in his upper and lower extremities. He had serum IgG antibodies to GM1 and GT1a but not to GQ1b. Anti-GT1a antibodies did not cross-react to GM1 by means of the absorption test. Titers of the antibodies decreased after recovering from muscle weakness of upper limbs. Since the presence of serum antibodies to GT1a but not to GQ1b were reported in patients with pharyngeal-cervical-brachial weakness of Guillain-Barré syndrome, it has been suggested that anti-GT1a antibodies play a role in acute oropharyngeal neuropathy. This is the first report of a patient with GBS lacking oropharyngeal palsy who had serum IgG antibodies to GT1a but not to GQ1b. Our case suggests that anti-GT1a antibodies are related not only with acute oropharyngeal neuropathy but also with upper limb dominant motor neuropathy.

Topics: Aged; Arm; Autoantibodies; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Muscle Weakness

A 15-year-old Japanese girl developed bulbar palsy and upper limb-dominant muscle weakness 2 weeks after the onset of an upper respiratory tract infection due to cytomegalovirus (CMV). Her symptoms resembled that seen in the pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome (GBS). Although bulbar palsy usually continues for several months in PCB, her bulbar palsy was very mild and improved rapidly before intravenous immunoglobulin therapy was instituted. Serum anti-GT1a IgG antibody titer was elevated at the acute phase of the disease and gradually decreased. The bulbar palsy-dominant GBS is thought to relate to anti-GT1a antibody and Campylobacter jejuni infection in adult patients. Our Case report suggests that CMV can also induce the production of anti-GT1a antibody, thereby resulting in PCB. When one sees acute onset bulbar palsy and limb muscle weakness, the possibility of PCB, even in children, should be considered, thus compelling the need for serum anti-ganglioside antibody measurement.

Topics: Adolescent; Arm; Bulbar Palsy, Progressive; Cytomegalovirus Infections; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Muscle Weakness; Muscle, Skeletal; Neck Muscles; Pharyngeal Diseases; Predictive Value of Tests; Treatment Outcome