Click here to watch Dr. Daniel Selchen discuss the case and the responses to the survey.

Kay, 39 years old, is sales clerk at a downtown department store. In 2009, at age 27 years, she was diagnosed with RRMS. Her initial presentation was optic neuritis, but she subsequently developed brainstem symptoms, including  mild vertigo and difficulty swallowing. There was no family history of demyelinating or autoimmune disorders. There was no significant medical history and no comorbidities.

Kay began treatment in 2010 with interferon-beta-1a 44 mcg tiw. She was switched to DMF 240 mg BID in 2016 due to ongoing clinical and MRI activity. At that time her EDSS score was 2.5 (pyramidal 2, cerebellar 2). She remained clinically stable for three years and continued her employment.

In 2019, Kay experienced a myelopathic episode that was treated with steroids. There was one Gd+ lesion on MRI brain and one Gd+ lesion on MRI spinal. During this episode, she developed new bladder symptoms. EDSS was 3.5 (pyramidal 2, sensory 2, bladder 2, cerebellar 2). Kay said she was satisfied with her current therapy, so treatment was not escalated.

During her appointment in 2021, Kay said she was experiencing increasing fatigue when walking. She said she found it difficult to walk 1 km, in part because of increasing stiffness in her legs. She reported that two years ago she could walk 2-3 km without difficulty. She was also experiencing some problems at work. Her employer had recently complained about the number of mistakes she was making, and she was feeling more mentally fatigued at the end of the day.

Repeat MRI of the brain and spinal cord showed no new lesions. EDSS was unchanged at 3.5.

The survey is now closed. We received 39 responses. See below for a summary of the answers you provided.

Question 1. A diagnosis of SPMS requires what criteria?
Few respondents said that a diagnosis requires an EDSS score of >4 (5%), a pyramidal system score >3 (0%) or evidence of atrophy (10%); 10% said all three were needed. Most (74%) said that a diagnosis was made according to none of the listed criteria.

Question 2. For Kay to be diagnosed with active SPMS would require what?
Respondents did not believe that MRI change or a relapse in the past two years was sufficient for a diagnosis of active SPMS; 13% thought that both findings would be sufficient. One-third (36%) said that progression independent of relapses (PIRA) would be required for the diagnosis. Most respondents (51%) required MRI changes, a recent relapse and PIRA before diagnosing active SPMS.

Question 3. Would you diagnose Kay with active SPMS?
A majority of respondents thought that Kay does have active SPMS because she has worsening symptoms and increasing disability (51%), or has demonstrated clear evidence of progression in the past two years (28%). 10% thought she does not have active SPMS since there is no evidence of disease activity (relapses or new lesions). 10% stated that Kay may have started the transition to SPMS.

Question 4. A diagnosis of SPMS may be delayed because of what?
Most respondents (64%) said that an SPMS diagnosis is delayed due to the lack of a clear definition of SPMS and the clinician’s reluctance to make the diagnosis. 10% that clinicians’ reluctance was the most common reason for diagnostic delay. 26% thought that the above-stated reasons, as well as difficulty getting an MRI and problems doing/interpreting the EDSS, explained the diagnostic delay.

Question 5. Kay is currently taking DMF 240 mg BID. What would be your treatment approach?
Most respondents said they would diagnose SPMS and switch to siponimod (74%). 10% said they would diagnosis SPMS and provide symptomatic therapy only. A minority said they would not diagnose SPMS; rather, they would keep their diagnosis as RRMS and switch either to a monoclonal antibody (10%) or to cladribine (5%).

View the video commentary by Dr. Daniel Selchen.