ABSTRACT
Coronavirus disease 2019 (COVID-19) is due to the infection of the upper and lower airways by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). COVID-19 is characterized by different clinical manifestations ranging from paucisymptomatic conditions to life-threatening acute respiratory distress syndrome and may present multisystem involvement. A hyperinflammatory response to the virus and the associated prothrombotic state (immunothrombosis) are the major causes of tissue/organ damage. Several humoral mediators have been described to mediate the immunothrombosis in COVID-19;among them, a lot of attention has been paid to the synthesis of nonorgan specific procoagulant autoantibodies, the hyperproduction of proinflammatory cytokines, and to the activation of the complement cascade. All the above-mentioned pathogenic pathways are affecting the endothelium as one of the main targets of the disease and contribute to the clinical manifestations. © 2023 Elsevier Inc. All rights reserved.
ABSTRACT
Nodal and paranodal antibody-associated neuropathies have been recently classified as auto-immune nodopathies. Their clinical presentation can be easily mistaken for acute or chronic idiopathic demyelinating polyneuropathy (AIDP/CIDP). We present a case of pan-neurofascin antibody associated paranodal neuropathy with serial electrodiagnostic findings. A 61-year-old man presented with acute onset generalized weakness and tiredness one day after the Covid-19 booster vaccination. He had progressive sensory disturbance and weakness in his upper limbs spreading to the lower limbs. He was treated with IVIg for Guillain-Barre syndrome (GBS) and discharged with some improvement. Five days later, he developed rapidly progressive predominantly motor symptoms becoming quadriplegic and bedbound within 9 days with no cranial or respiratory involvement. He was found to have paranodal antibodies (neurofascin-155), consistent with a pan-neurofascin antibody positive neuropathy. Although the patient responded poorly to conventional immunomodulatory therapies, he showed a rapid remarkable recovery with rituximab. He was able to walk independently again within 3 weeks after the second dose of rituximab. Electrodiagnostic studies showed only subtle proximal demyelinating features with the initial presentation. The repeat study after his relapse showed a significant deterioration with features of a generalized, non-length-dependent, primarily demyelinating, sensorimotor polyneuropathy with conduction block, but the sural nerve biopsy showed axonal neuropathy. Electrodiagnostic findings nearly normalized two months after rituximab treatment with the resolution of the all the demyelinating features. This case presentation highlights the importance of considering nodal/paranodal neuropathy as a differential for AIDP/CIDP in an early stage of the disease, especially in patients with atypical presentation. In addition, it supports the currently available evidence that more targeted treatment such as rituximab can have an excellent outcome. Copyright © 2022