Background: Whether or not antiganglioside antibodies are related to axonal or demyelinating Guillain-Barré syndrome (GBS) is still a matter of controversy, as detailed in previous studies conducted in Western and Asian countries.
Objective: To clarify whether antiganglioside antibodies are associated with axonal dysfunction in Japanese and Italian GBS patient cohorts.
Methods: Clinical and electrophysiological profiles were reviewed for 156 GBS patients collected from Japan (n=103) and Italy (n=53). Serum IgG antibodies against GM1, GM1b, GD1a and GalNAc-GD1a were measured by ELISA in the same laboratory. Electrodiagnostic criteria and results of serial electrophysiological studies were used for classification of GBS subtypes: acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN).
Results: In both Japanese and Italian cohorts, any of the antibodies were positive in 36% of the patients, and antibody positivity had a significant association with the AMAN electrodiagnosis. Approximately 30% of Japanese and Italian antiganglioside positive patients showed the AIDP pattern at the first examination whereas sequential studies showed that most finally showed the AMAN pattern. Clinically, seropositive patients more frequently had preceding diarrhoea and pure motor neuropathy in both Japanese and Italian cohorts; vibratory sensation was normal in 97% of Japanese and in 94% of Italian seropositive patients.
Conclusions: In GBS, clinical and electrophysiological features appear to be determined by antiganglioside antibodies, and the antibodies are associated with motor axonal GBS in both Japan and Italy. Classification of the GBS subtypes as a disease entity should be made, combining the results of antiganglioside assays and serial electrodiagnostic studies.
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http://dx.doi.org/10.1136/jnnp-2011-300309 | DOI Listing |
Cureus
December 2024
Internal Medicine, Leicester Royal Infirmary, Leicester, GBR.
Miller-Fisher syndrome (MFS) is characterized by the three major components of ophthalmoplegia, ataxia, and areflexia. The occurrence of MFS is relatively uncommon because of its monophasic nature, while recurrent Guillain-Barré syndrome (GBS) is a well-known condition. The pharyngeal-cervical-brachial (PCB) variant is a scarce variant of GBS (3%), which presents with muscle weakness initially involving the neck, oropharynx, and upper extremities.
View Article and Find Full Text PDFCureus
November 2024
Hematology and Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, JPN.
A 40-year-old man presented to our hospital with subacute progressive muscle weakness in the limbs and leukocytosis. Subsequently, the patient was diagnosed with chronic lymphocytic leukemia (CLL) complicated by peripheral motor neuron neuropathy (axonopathy). Serology test for anti-ganglioside GM2 IgG antibody was positive, whereas paraneoplastic syndrome-related and anti-myelin-associated glycoprotein antibodies were not detected.
View Article and Find Full Text PDFRinsho Shinkeigaku
December 2024
Division of Neurology, Anti-aging, and Vascular Medicine, Department of Internal Medicine, National Defense Medical College.
A 56-year-old woman who presented with left drop foot and low back pain a week after the onset of diarrhea. Neurological symptoms progressed for a week and gradually improved thereafter. No weakness was observed in upper limbs and clearly asymmetrical muscle weakness was observed in left lower limbs during the course of the disease.
View Article and Find Full Text PDFInt J Mol Sci
November 2024
Department of Biomedical Sciences, Chubu University, Matsumoto 1200, Kasugai 487-8501, Aichi, Japan.
Extracellular vesicles (EVs) play important roles in intercellular communication in various biological events. In particular, EVs released from cancer cells have attracted special attention. Although it has been reported that cancer-associated glycosphingolipids play important roles in the enhancement of malignant properties of cancer cells, the presence, behavior, and roles of glycosphingolipids in EVs have not been elucidated.
View Article and Find Full Text PDFIntern Med
November 2024
Department of Neurology, Daido Hospital, Japan.
Up to one-third of lymphoma cases involve the nervous system. Miller-Fisher syndrome (MFS) associated with lymphoma is extremely rare. We herein report a case of primary central nervous system lymphoma initially mimicking MFS in a 70-year-old man who presented with subacute unsteady gait and diplopia.
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