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Medically reviewed by Dr. Kimberly Langdon Cull, M.D. on Sept. 20, 2023

Late-onset neuromyelitis optica spectrum disorder may masquerade as a stroke. Early diagnosis and treatment can vastly improve outcomes for patients, underscoring the necessity for clinicians to be cognizant of this possible misdiagnosis.

  • Neuromyelitis optica spectrum disorder is a rare autoimmune disorder distinct from multiple sclerosis.
  • Atypical stroke-like presentations can be indicative of neuromyelitis optica spectrum disorder.
  • Early intervention with immune therapies may drastically improve patient outcomes.

Neuromyelitis optica spectrum disorder (NMOSD) is a unique autoimmune disorder that was once considered a variant of multiple sclerosis (MS). However, distinctions became clear, especially with the discovery of aquaporin-4 (AQP4-Ab) and myelin oligodendrocyte glycoprotein antibodies (MOG-Ab). Diagnosis can be complex, as demonstrated in one case involving a 58-year-old female patient who was initially treated for a pontine stroke. It was only upon further investigation and worsening symptoms that AQP4-Ab positivity was identified, leading to an NMOSD diagnosis.

Misdiagnosed NMOSD: A Closer Look

A systematic literature review and case report published in the journal Cureus, examined NMOSD cases initially mistaken as stroke. Following PRISMA guidelines, six cases of NMOSD were initially diagnosed as acute strokes. There was a consistent trend of progression and/or recurrence of symptoms, emphasizing the importance of supplemental investigations. These misdiagnosed cases tended to happen in patients who were older than the usual age group for NMOSD, further complicating diagnosis.

Treatment Approaches and Clinical Implications

NMOSD treatment typically consists of immune system suppression in the acute state and prevention of potential relapses. Steroids and plasma exchange are frequently used, with some clinicians recommending simultaneous usage for optimal outcomes. To prevent NMOSD relapses, medications such as azathioprine, mycophenolate, or rituximab were prescribed in cases included in the study. Certain AQP4-Ab positive patients could benefit from more aggressive immunosuppressive treatments, opening up new possibilities for managing the disease.

Relevance to Clinicians

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For healthcare providers, the findings from this review could be important. Atypical stroke-like syndromes, especially those with progressive or recurrent symptoms related to posterior circulation and unexplained gastrointestinal problems, should prompt consideration of NMOSD. In addition, stroke-like presentations without typical ischemic changes on initial brain MRI and vascular imaging should raise suspicions. For optimal patient outcomes, clinicians should aim for early diagnosis, timely treatment, and diligent monitoring for potential relapses.


Huang, B. K., Arora, R., McFarlane, S., Diamond, J. A., & Najjar, S. (2023). Neuromyelitis Optica Spectrum Disorder Mimicking Pontine Stroke: A case report and Systematic Literature review. Cureus, 15(6), e41099. https://doi.org/10.7759/cureus.41099