In August 2011 I went into the National Hospital for Neurology and Neurosurgery for a few days of investigations expecting to be diagnosed with Multiple Sclerosis. I was finally let out exactly a month later with a diagnosis of "presumptive CLIPPERS" after MS and lots of other conditions had been considered, investigated and rejected. This simplified account is based on memory and doesn't include the extensive discussions amongst the medical staff on my team which happened behind the scenes and included the detailed results of many more tests than I report here. I've never stayed in hospital before and my experience was that all the medical staff at the NHNN and especially the nurses on John Young Ward were professional, patient, kind and tolerant sometimes in difficult or unpleasant circumstances.
Week 1: Multiple Sclerosis?
An extensive history was taken. The main points of interest at this stage were whether I had hyper-tension risk factors, whether I could be diabetic, whether I had travelled anywhere exotic (and picked something up), whether I had been ill or whether I had been bitten by any tick-like insects. They also looked carefully for signs of muscle-wasting (I had to explain that I've always had thin arms!). Peripheral nerve conductance tests were done to see if the nerve pathways between my arms (legs) and hands (feet) were intact. They were fine which suggested any movement problems were likely to be brain related. MS was still possible but diagnosis is tricky as the relapsing-remitting form requires evidence of two distinct attacks and the primary progressive form requires evidence of a year of progression on top of characteristic MRI appearance, consistent lumbar-puncture and other test results. Then my MRI brain scan was done (12th August) and showed a curious and unexpected pattern of lesions (inflammatory spots); so the picture started to change. I was sent for lumbar-puncture (17th August) and a spinal MRI (18th August) which confirmed a couple of small additional lesions.
Week 2: Sarcoidosis?
Some kind of inflammatory / auto-immune disorder was still likely. Neurosarcoidosis was considered. It is most commonly found in the lungs but can affect the brain possibly while still silent elsewhere. I was sent for CT head and angiogram (22nd August). The angiogram looks at the difference in two CT scans before and after a contrast agent is injected to highlight the major arteries. This shows whether there is a problem with blood supply to parts of the brain. Fortunately nothing was found here as this can be bad news. I was then sent for a PET scan (25th August|) to look for abnormal metabolic signs in the head and chest which might indicate the presence of inflammatory processes. I also had a second lumbar puncture.
Week 3: Vasculitis?
Around this time vasculitis started being mentioned much more. It is another non-specific inflammatory condition which affects blood vessels and which can originate outside the brain. I had a minor blip in a urine protein test which prompted referral to kidney specialists to check for signs of kidney/metabolic disorders. Following a consultation and kidney ultrasound (30th August) they decided against biopsying my kidneys for which I was greatful. Following the report on my PET scan I had cardiac ultrasound to double check my heart function. I was offered a brain biopsy so that brain tissue can be examined directly under the microscope. This is particularly valuable where indirect tests are inconclusive but doesn't always provide definitive answers. I declined this procedure and will discuss why in a future post.
Week 4: CLIPPERS?
One of my medical team mentioned a recently characterised inflammatory brain disorder which had appeared in the research literature in 2010 called CLIPPERS. He said my symptoms and test results fit the pattern for CLIPPERS quite well (and certainly better than everything else that had been considered). There were still a few unusual possibilities other than CLIPPERS but nothing to make a strong case for them. So the decision was taken to treat my condition as CLIPPERS in the absence of any evidence against it. In the mean-time I was sent for chest CT to check again for any evidence of inflammatory processes outside the brain - none found. While in hospital some further gradual decline had occurred. I noticed my speech slowing to me as though I had trouble getting words out. Also my hand-writing was increasingly scratchy, my texting was slow and chaotic and tying shoe-laces was becoming a challenge.
Treatment
I was started on 5 days of intravenous steroids (1g prednisolone for an hour per day). After a few days my wife complained she couldn't get a word in as I was talking so much. No other side-effects though. My walking and balance started to improve noticeably but not dramatically. My speech improved as well. My facial numbness disappeared but my double-vision was unchanged. I developed "glitches" where my limbs would stiffen for a few seconds when starting a new activity (or sometimes randomly) so I would walk stiffly like a tin-soldier and then recover. My speech although mostly normal would occasionally stall in a similar manner. So there was overall improvement but not yet complete resolution. I had a final MRI to look for radiological improvement and the lesion presence was reported much reduced if not completely resolved. I was discharged on 9th September exactly a month after admission with a programme of oral steroids starting at 60mg day and reducing over 12 weeks.
Treatment
I was started on 5 days of intravenous steroids (1g prednisolone for an hour per day). After a few days my wife complained she couldn't get a word in as I was talking so much. No other side-effects though. My walking and balance started to improve noticeably but not dramatically. My speech improved as well. My facial numbness disappeared but my double-vision was unchanged. I developed "glitches" where my limbs would stiffen for a few seconds when starting a new activity (or sometimes randomly) so I would walk stiffly like a tin-soldier and then recover. My speech although mostly normal would occasionally stall in a similar manner. So there was overall improvement but not yet complete resolution. I had a final MRI to look for radiological improvement and the lesion presence was reported much reduced if not completely resolved. I was discharged on 9th September exactly a month after admission with a programme of oral steroids starting at 60mg day and reducing over 12 weeks.
So that was my summer in 2011.
Read other articles in this series at Living With CLIPPERS.
Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
