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3541 A case of a seronegative fulminant neuropathy responsive to antibody-depleting therapy

bmjno · 2025-10-23 · canonical JSON source

9 visible annotations · policy: published · automated confidence ≥ 75.00%

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Case A 53-year-old male presented with a 10-day history of progressive ascending weakness and sensory loss following COVID-19 infection. Initial MRI and CSF examination were unremarkable, and mild proteinuria was noted. A provisional diagnosis of Guillain-Barre Syndrome (GBS) was made, and he was treated with intravenous immunoglobulins (IVIG), resulting in recovery and discharge. He represented after 4 days with worsening sensorimotor deficits and had a poor response to plasma exchange, IVIG and intravenous methylprednisolone. Nerve conduction studies remained normal at 2 and 4 weeks after the onset of the symptoms. Mildly elevated protein was found in subsequent CSF examination. Nodo-paranodal antibodies were not detected. Proteinuria reached the nephrotic range. The patient progressively deteriorated, resulting in tetraplegia, oculobulbar palsy and respiratory compromise. Eight weeks after the onset of the symptoms, nerve conduction studies confirmed a demyelinating pattern, and MRI demonstrated subtle change in foraminal nerve roots. Induction of treatment with rituximab led to rapid improvement, achieving a modified Rankin scale of 2 from a nadir of 5.Conclusion This case highlights the need to recognise red flags when assessing neuropathies that resemble GBS. While neurologists excel at pattern recognition, they must stay vigilant for warning signs. Red flags that should prompt clinicians to reconsider the diagnosis of GBS include an early and severe relapse following an initial response to conventional therapies, the presence of proteinuria -especially in the nephrotic range, cranial nerve involvement and respiratory compromise. Furthermore, we have demonstrated the effectiveness of early treatment with Rituximab in these cases.