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Medicine · 2025

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

СтатьяКлинический случайОбзорНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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Krim Elsa, Masri Alexandre, Delmont Emilien, Le Masson Gwendal, Boucraut Joseph, Mathis Stéphane

Аннотация

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 for these ultimately curable conditions. Despite causing major disability, pan-neurofascin autoimmune nodoparanodopathy is curable if the appropriate treatment is given.

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