Acute-onset chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 antibodies and bilateral facial nerve enhancement

André Caetano, Filipa Ladeira, Marco Fernandes, Pedro Pires, Elmira Medeiros

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1 Citation (Scopus)

Abstract

A 26-year-old female presented with acute onset distal paraparesis, upper limb tremor and bilateral facial palsy. Neurophysiology revealed a sensorimotor demyelinating polyneuropathy and lumbar puncture revealed an albuminocytologic dissociation. Neuroaxis MRI revealed bilateral facial nerve and cauda equina enhancement. Initially diagnosed as Guillain-Barré Syndrome, poor response to intravenous immunoglobulin, persistent deterioration, anti-neurofascin-155 antibodies and clinical response to steroid therapy led to diagnosis of acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP). CIDP patients with anti-neurofascin-155 antibodies are younger, with distal predominant weakness, tremor, and poor response to intravenous immunoglobulin. Up to 16% can present acutely, however bilateral facial weakness is rare.

Original languageEnglish
Article number577026
JournalJournal of Neuroimmunology
Volume336
DOIs
Publication statusPublished - 15 Nov 2019

Keywords

  • CIDP
  • Facial nerve
  • Nerve enhancement
  • Neurofascin-155
  • Polyneuropathy

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