Click here to watch Dr. Daniel Selchen discuss the case and the responses to the survey.
Ken, a 53-year-old man, was initially seen in 2017 following onset of painful spasms in R leg and probable clonus while driving his car. He developed R leg weakness and band-like discomfort across his chest. He was worked up for stroke and cardiac issues and all investigations were negative. He had a prior history of asthma with nasal polyps and NSAID-associated dyspnea.
He was referred a few months later with worsening L leg weakness and urinary urgency. O/E asymmetric spastic paraparesis. There was decreased vibration in the arms (better in the legs). The MRI was poor quality. There were no definite lesions evident in the thoracic spine, but some irregular enhancement (isolated small lesion) in the lower cervical spine. There were a few pericallosal and juxtacortical lesions on brain imaging, but findings were generally nonspecific. CSF results: 9 cells (lymphocytes), normal protein and glucose, matched bands CSF and blood.
Ken was lost to follow-up for two years and no tests were performed. He was referred back by a different neurologist. Repeat MRI-spine revealed patchy non-enhancing lesions in the cervical and thoracic cord consistent with demyelinating disease. There were no long lesions. CT chest was compatible with mild sarcoid. CSF was atypical with bands in CSF and blood but more in CSF. He was negative for myelin oligodendrocyte glycoprotein (MOG) and neuromyelitis optica (NMO) antibodies.
Ken reported significant fatiguability and spasticity. His gait had worsened but he was still able to walk 1 km with no aids. O/E mild clumsiness but no weakness in the upper extremities, decreased vibration. Asymmetric spastic paraparesis, 4+ R, 3+ L. EDSS 4.0 (motor 3, sensory 3, bladder 2). Lung biopsy was positive for sarcoid. He was started on steroids with no improvement in neurological symptoms or signs.
Repeat MRI (brain, cervical/thoracic spine) was stable. Ken experienced gradual worsening of mobility and spasticity but no change in EDSS. Steroids were tapered off by Respirology with no change.
The survey is now closed. There were 32 responses. See below for a summary of the answers you provided.
Question 1: What is your diagnosis?
Respondents were divided between neurosarcoidosis (63%) and multiple sclerosis (37%). No one voted for the other options (transverse myelitis, cerebral small-vessel disease, NMOSD).
Question 2: Would you order further tests to rule out infection (e.g. Lyme disease, TB, syphilis)?
A majority (66%) said Yes, 25% said No and 9% were Unsure.
Question 3: Does this patient meet the diagnostic criteria for multiple sclerosis?
The largest proportion of respondents was unsure (41%). One-third thought that the patient met the MS diagnostic criteria of dissemination in space and time. Six percent said he met criteria for DIS but not DIT and 19% said the clinical/radiological findings were generally not considtent with an MS diagnosis.
Question 4: Would you initiate a disease-modifying therapy in this patient?
A majority (59%) would not initiate a DMT but would await further testing. Twenty-eight percent said they would start treatment immediately and 13% said that a DMT would be inappropriate for this patient.
Question 5: If you were to initiate therapy, which DMT would you use?
The most common response was an anti-CD20 agent (48%). A few respondents opted for a beta-interferon (16%), DMF or teriflunomide (12%), siponimod (12%) and oral cladribine (12%).
View the video commentary from Dr. Daniel Selchen.