Seronegative neuromyelitis optica presenting with life-threatening respiratory failure

J Spinal Cord Med. 2016 Nov;39(6):734-736. doi: 10.1080/10790268.2015.1101984. Epub 2015 Dec 18.

Abstract

Context: Dyspnea has rarely been reported as a presenting symptom in patients with neuromyelitis optica (NMO). We report an unusual case of NMO relapse presenting with rapidly progressive respiratory failure and briefly discuss the possible pathophysiological mechanisms of this potential life-threatening complication of NMO.

Findings: The 58-year-old woman with a history of bilateral optic neuritis presented to the emergency department with rapidly worsening dyspnea. Cervical spine magnetic resonance imaging showed extensive abnormal signal with involvement of the medulla oblongata. Since in our patient chest radiography failed to disclose a diaphragmatic palsy that is commonly observed in patients with phrenic nerve involvement, this acute manifestation of the disease may be attributed to brainstem involvement instead of cervical myelitis.

Conclusion/clinical relevance: Clinicians should be aware of this atypical presentation of NMO, which needs to be promptly recognized and aggressively treated.

Keywords: Anti-aquaporin-4 antibodies; Brainstem; Cervical spine; Neuromyelitis optica; Respiratory failure.

Publication types

  • Case Reports

MeSH terms

  • Brain Stem / diagnostic imaging
  • Brain Stem / pathology
  • Female
  • Humans
  • Middle Aged
  • Neuromyelitis Optica / blood
  • Neuromyelitis Optica / complications
  • Neuromyelitis Optica / diagnosis*
  • Respiratory Insufficiency / diagnosis*
  • Respiratory Insufficiency / etiology
  • Serologic Tests
  • Spinal Cord / diagnostic imaging
  • Spinal Cord / pathology