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

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

Caetano, André;Ladeira, Filipa;Fernandes, Marco;Pires, Pedro;Medeiros, Elmira;
journal of neuroimmunology 2019 Vol. 336 pp. 577026
265
caetano2019acuteonsetjournal

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.

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