Click here to watch Dr. Dalia Rotstein discuss the case and your responses to the quick poll.

A 51-year-old woman presents with lower extremity weakness and numbness which began 24 hours ago and gradually ascended up to her mid-chest. She could not ambulate for the last 12 hours, and it is more difficult to pass urine. She has no history of recent infectious symptoms or travel.

She is evaluated by neurology in the Emergency Department. Her power is 2/5 in the right leg and 4/5 in the left leg in a pyramidal distribution. Plantar responses are upgoing. She has absent pinprick sensation up to a T6 sensory level on her torso. Vibration is absent in the lower extremities and intact in the distal fingers. She cannot weight-bear.

A diagnosis of transverse myelitis is suspected. An MRI of the cervical and thoracic spine with gadolinium reveals a longitudinally extensive T2-hyperintense and Gad-enhancing lesion from C2-C6. CSF studies reveal 50 WBC, 2 RBC, a mildly elevated protein of 0.7 g/L (normal: 0.15-0.45 g/L) and normal CSF glucose. Oligoclonal banding is negative.

The survey is now closed. There were 33 responses. See below for a summary of the answers you provided.

Question 1: The consultant neurologist plans to refer the patient to a demyelinating disease specialty clinic. Which of the following tests are the most important to do now in advance of the referral and treatment?
All respondents thought that MRI-Brain and serum aquaporin-4 (AQP4) and MOG antibody testing were the most important to obtain.

Question 2: If the AQP4-antibody test had not been sent and the patient was not started on long-term immunosuppressive therapy, which of the following is most likely?
Most respondents (81.8%) thought that the most likely outcome was a severe, disabling attack of optic neuritis within 2 years. About 9% of respondents said she would likely experience progressive disability with weakness gradually beginning to affect her arms; 6% thought she would experience a mild attack with area postrema symptoms (nausea, vomiting, hiccups) in 5-10 years; and 3% thought she would remain relapse-free.

Question 3: She receives 3 days of IV methylprednisolone 1 gram daily without improvement. Which factor has been shown to be most important in predicting how much she will recover from this relapse?
A majority (69.7%) said that early initiation of plasma exchange (within 5 days of relapse) was the most important factor in predicting recovery from the relapse. Another 18% that plasma exchange within 8 days of the relapse would predict recovery, and 12% said that the presence of an enhancing spinal cord lesion would be most predictive.

Question 4: Three weeks later she meets with a specialist in the MS/Demyelinating Disease Clinic. Which of the following drugs have shown efficacy in randomized clinical trials for treatment of AQP4+ NMOSD?
A total of 90.6% correctly stated that satralizumab, inebilizumab and ravulizumab have demonstrated efficacy in clinical trials. The remaining 9.4% opted for mycophenolate mofetil, satralizumab and inebilizumab. It should be noted that no randomized controlled trials have been completed for mycophenolate mofetil in NMOSD, although several studies have demonstrated efficacy (for a systematic review see Songwisit et al. Sci Rep 2020;10:16727).

Question 5: Which of the following drugs is matched with the correct potential side effect?
Most respondents (96.8%) correctly stated that there is a risk of meningitis with ravulizumab (and eculizumab). Patients must be vaccinated against meningococcal infections at least two weeks before initiating ravulizumab unless the risks of delaying treatment outweigh the risks of
developing a meningococcal infection. Patients who start treatment <2 weeks after vaccination must receive antibiotic prophylaxis until two weeks after vaccination.

View the video commentary from Dr. Dalia Rotstein.