Barts-MS rose-tinted-odometer: zero-★s (black Monday; playing roulette with your patients’ brains)
I recently saw a patient for a second opinion. He was about to start dimethyl fumarate (DMF), but his wife who had spent a lot of time reading about MS wanted him to be treated with something more effective. She had read the MS-Blog (formerly the Barts-MS Blog) and wanted him to have a higher efficacy DMT.
He is only 43 years of age and has had MS for at least 12 years. He had an episode of transverse myelitis when he was 31. At the time his MRI of the brain had no MS-like lesions, but in retrospect, there was subtle brain volume loss; his so-called Sylvian fissures were much too large for a 31-year-old and he had largish lateral ventricles (the fluid-filled spaces in the brain). The clue to the diagnosis of MS was in the spinal fluid analysis that showed the local synthesis of oligoclonal IgG bands; these can now be used in the diagnosis of MS to indicate dissemination in time. This patient was sent away and told to come back if he developed any new symptoms. He did come back with an episode of vertigo and unsteadiness of gait 12 years later. His MRI of the brain was full of MS lesions and he had gross brain volume loss. He was told by his neurologist that he now had relapsing-remitting MS and was eligible for treatment and been offered DMF.
When taking a neurological history this patient had had numerous symptoms that indicated he had had several attacks in the last 12 years. An episode of sharp shooting pains in legs, a period of urinary frequency and urgency, an episode when he had noticed difficulty running with a partial dropping of his right foot. All of these were clearly relapses, which he ignored. The onus of reporting these symptoms had been put on the patient. Maybe things would have turned out differently if he had been seen annually and had regular monitoring MRIs.
On our video consultation, he said he was fully functional, working full-time and had no problems with activities of daily living. This was in keeping with his neurologist’s clinic letter that didn’t mention any abnormal neurological signs and the letter actually played down the MRI findings, by not even mentioning the gross brain atrophy that had been reported by the neuroradiologist on the current MRI.
As I was doing this consultation via a video platform something told me I need to examine this patient. I arranged a face-2-face consultation and when I saw this patient a few weeks later his neurological examination was far from normal. He has jerky eye movement with square-wave jerks indicative of cerebellar involvement. He had bilateral optic disc pallor indicative of optic nerve involvement. He was unable to walk heel-to-toe due to an unsteady gait and he had a positive Romberg’s test, i.e. his body swayed from side to side when he closed his eyes and he would have fallen if I had not told him to open his eyes. He had mild triparesis; i.e. weakness in three out of four limbs. He also had clear cerebellar signs with incoordination in both upper limbs with a mild intention tremor. Finally, he had impaired joint position sensation in the joints of the big toe.
On taking his history again he now volunteered mild to moderate urinary frequency and sexual dysfunction. His wife who attended with him told me that he had had to stop running a few years ago because of exercise-induced left foot drop, which had been getting worse and now was visible after 2-3 km of walking. She also mentioned limb jerks in bed at night, nocturia and that he had become very forgetful and was having difficulty at work.
It is clear this gentleman has advanced MS with severe end-organ damage affecting all functional systems. Sadly he has been let down by the system. He should have been told upfront it is likely he had MS. Just maybe cognitive testing and a set evoked potential 12 years ago would have shown dissemination in space and he would have been diagnosed with MS and treated. Instead, his diagnosis of MS has been delayed by 12 years. Based on his history and examination this paint has secondary progressive MS (SPMS).
Should I label him as having active SPMS and offer him siponimod? Should I say he has highly active MS or rapidly evolving severe MS and try and manipulate the NHS England treatment guidelines to offer him a choice of several high efficacy DMTs? Should I just take the path of least resistance and offer him ocrelizumab or ofatumumab the two very high-efficacy DMTs that can be used first-line in the NHS? Should I not offer him a licensed DMT and refer him for possible enrollment into the high-dose simvastatin trial?
What this pandemic has taught me is that my real skill, which won’t be replaced soon by a robot, is doing a neurological examination and eliciting signs of end-organ damage and then integrating this information with the history, MRI and other investigations. I wonder if the many MS self-monitoring applications that are emerging would have detected and interpreted the clinical examination the way I have done with this patient?
Interestingly, when I examined this patient he told me that his neurologist, apart from getting him to walk and test his eye movements, had not done a detailed neurological examination. This is not unusual in clinical practice; in fact, one of our colleagues at the Royal London Hospital has even argued for us not doing the neurological examination at all as it doesn’t add much to either the diagnosis and/or management of his patients. Do you agree with him?
Christopher H Hawkes. I’ve stopped examining patients! Pract Neurol. 2009 Aug;9(4):192-4.
If you are interested in finding out about what changes to our MS services from the pandemic will stick I suggest you log into the webinar that I am doing later this week with Trishna Bharadia when we will be discussing the impact and changes the pandemic has had om MS services and what I think will change. What is clearly not going to go away is the need for face-2-face consultations and neurological examinations. But then this is referring to my take on things and I may not be correct.
General Disclaimer: Please note that the opinions expressed here are those of Professor Giovannoni and do not necessarily reflect the positions of the Barts and The London School of Medicine and Dentistry nor Barts Health NHS Trust.