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Pan-neurofascin autoimmune nodoparanodopathy: A case report and literature review
Elsa Krim1, Alexandre Masri2, Emilien Delmont3
1Department of Neurology, Pau Hospital, Pau, France.
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.
