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

Research output: Contribution to journalArticle

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

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Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Intravenous Immunoglobulins
Facial Nerve
Tremor
Paraparesis
Cauda Equina
Neurophysiology
Spinal Puncture
Polyneuropathies
Antibodies
Facial Paralysis
Upper Extremity
Antibody Formation
Steroids
Therapeutics

Keywords

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

Cite this

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title = "Acute-onset chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 antibodies and bilateral facial nerve enhancement",
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-Barr{\'e} 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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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.

In: Journal of Neuroimmunology, Vol. 336, 577026, 15.11.2019.

Research output: Contribution to journalArticle

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T1 - Acute-onset chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 antibodies and bilateral facial nerve enhancement

AU - Caetano, André

AU - Ladeira, Filipa

AU - Fernandes, Marco

AU - Pires, Pedro

AU - Medeiros, Elmira

PY - 2019/11/15

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AB - 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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KW - Nerve enhancement

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