Background And Objectives: Ganglioside antibodies can help diagnose distinct acute and chronic inflammatory neuropathies including axonal variants of Guillain-Barre syndrome, Miller-Fisher syndrome (MFS), multifocal motor neuropathy, and chronic sensory ataxic neuropathies. Because ganglioside antibody testing may be routinely ordered in patients with suspected inflammatory neuropathy, we sought to evaluate its yield and utilization in clinical practice.
Methods: We performed a retrospective chart review of all patients at London Health Sciences Centre who underwent ganglioside antibody testing between April 2019 and August 2023. The disease phenotype was determined for each patient, and the proportion of all tests that yielded a true-positive result was calculated. Ganglioside antibody positivity was classified as a true-positive result if the disease phenotype was robustly associated with the detected ganglioside antibody and there was no other more likely diagnosis.
Results: We identified 92 patients who underwent ganglioside antibody testing. One patient (1%) was classified as having a true-positive result; this patient had GQ1b-IgG positivity with MFS. Among 92 patients tested, 20 patients (22%) had a disease phenotype that was considered to be robustly associated with ganglioside antibody positivity.
Conclusions: The yield of ganglioside antibody testing in clinical practice is low. We found that this testing is frequently ordered in patients with disease phenotypes that are not robustly associated with ganglioside antibody positivity, indicating that suboptimal test utilization is a primary contributor to its low yield. Restricting ganglioside antibody testing to patients with characteristic disease phenotypes would be valuable to improving yield and utilization of this testing.
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http://dx.doi.org/10.1016/j.jns.2024.122903 | 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 PDFFront Immunol
January 2025
Department of Neurology, Chengdu Second People's Hospital, Affiliated Hospital of Chengdu Medical College, Chengdu, Sichuan, China.
Anti-GQ1b antibody syndrome is a spectrum of autoimmune disorders affecting nervous systems. We report a case of a 53-year-old woman presenting mydriasis with acute onset of periorbital pain, photophobia, and subsequently, diplopia. Despite weakly positive anti-GQ1b IgG antibody, the patient exhibited atypical features with isolated ophthalmoplegia and absence of classic Miller-Fisher syndrome triad.
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 PDFNat Rev Dis Primers
December 2024
Department of Neurology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Guillain-Barré syndrome (GBS) is a rare immune-mediated polyradiculoneuropathy. Patients typically develop rapidly progressive weakness and sensory deficits that can result in complete paralysis requiring mechanical ventilation. GBS is usually a monophasic disease in which an aberrant immune response to an infection or other trigger damages the peripheral nerves.
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.
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