Pan-neurofascin autoimmune nodoparanodopathy: A case report and literature review

Auteurs

Krim E, Masri A, Delmont E, Le Masson G, Boucraut J, Mathis S

Les auteurs en lien sont membres de l'INS.

Résumé

Rationale: Locked-in syndrome (and its variant, completely locked-in state) generally has a high mortality rate in the acute setting; however, when induced by conditions such as acute inflammatory polyradiculoneuropathy, it may well be curable such that an attempt at cure should be systematically sought by clinicians. Patient concerns: A 52-year-old man presented with acute tetraparesia and areflexia, initially diagnosed as Guillain–Barré syndrome. Despite appropriate treatment, his condition deteriorated, evolving into a completely locked-in state. Diagnoses: The detection of anti-pan-neurofascin antibodies led to the correct diagnosis, acute pan-neurofascin autoimmune nodoparanodopathy. Interventions: Immunosuppressive treatment (rituximab) and plasma exchanges were performed. Outcomes: After several months, the patient’s neurological symptoms almost completely subsided, without any major sequelae. Lessons: In patients with locked-in syndrome (or its variant), neurologists and intensive care physicians must be aware of, and look for, the main etiologies (including pan-neurofascin autoimmune nodoparanodopathy), to allow the prompt initiation of treatment and thus a rapid recovery

Rationale: Locked-in syndrome (and its variant, completely locked-in state) generally has a high mortality rate in the acute setting; however, when induced by conditions such as acute inflammatory polyradiculoneuropathy, it may well be curable such that an attempt at cure should be systematically sought by clinicians. Patient concerns: A 52-year-old man presented with acute tetraparesia and areflexia, initially diagnosed as Guillain–Barré syndrome. Despite appropriate treatment, his condition deteriorated, evolving into a completely locked-in state. Diagnoses: The detection of anti-pan-neurofascin antibodies led to the correct diagnosis, acute pan-neurofascin autoimmune nodoparanodopathy. Interventions: Immunosuppressive treatment (rituximab) and plasma exchanges were performed. Outcomes: After several months, the patient’s neurological symptoms almost completely subsided, without any major sequelae. Lessons: In patients with locked-in syndrome (or its variant), neurologists and intensive care physicians must be aware of, and look for, the main etiologies (including pan-neurofascin autoimmune nodoparanodopathy), to allow the prompt initiation of treatment and thus a rapid recovery

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