Seronegative neuromyelitis optica presenting with life-threatening respiratory failure

The Journal of Spinal Cord Medicine, 2016 · DOI: 10.1080/10790268.2015.1101984 · Published: January 1, 2016

Simple Explanation

This case report describes a woman with neuromyelitis optica (NMO) who experienced a severe relapse with a life-threatening complication: respiratory failure. The patient, who had a history of optic neuritis, presented with rapidly worsening shortness of breath and was found to have spinal cord involvement on MRI. The doctors considered and excluded other potential diagnoses, such as infections, other autoimmune disorders and tumors. Prompt recognition and aggressive treatment are crucial in such cases.

Study Duration

Not specified

Participants

One 58-year-old woman

Evidence Level

Case Report

Key Findings

1
The patient presented with acute respiratory failure as the primary symptom of NMO relapse, which is an atypical presentation.
2
MRI revealed extensive abnormal signal in the cervical spine involving the medulla oblongata, indicating brainstem involvement.
3
Standard tests for NMO antibodies were negative (seronegative), highlighting the diagnostic challenges in such cases.

Research Summary

This case report describes an unusual presentation of seronegative NMO, where the patient's initial symptom of relapse was acute respiratory failure requiring mechanical ventilation. The patient's cervical spine MRI showed extensive abnormalities with involvement of the medulla oblongata, suggesting brainstem involvement as a cause of respiratory failure. The case emphasizes the importance of considering NMO in patients presenting with unexplained respiratory failure, even in the absence of typical antibody markers.

Practical Implications

Diagnostic Awareness

Clinicians should consider NMO in cases of unexplained respiratory failure, even if antibody tests are negative.

Prompt Treatment

Early recognition and aggressive treatment are crucial to managing life-threatening complications of NMO.

Imaging Importance

MRI of the cervical spine and brainstem is essential to identify lesions that may contribute to respiratory failure.

Study Limitations

1
Phrenic nerve conduction studies and needle EMG were not performed.
2
Fluoroscopic examination of the diaphragm with phrenic nerve stimulation was not conducted.
3
A cell-based assay for serum aquaporin-4 antibodies, the current reference standard for NMOSD, had not been performed.

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