George was diagnosed with autoimmune encephalitis in April 2021 after 9 months of living with the condition. He writes about his illness, diagnosis, the impact of his encephalitis and his ongoing treatment.
Firstly, this story is not complete, perhaps as far as the Encephalitis is concerned, it might never be but I am approaching what seems to be the ‘beginning-of-the-end’ rather than the ‘end of the beginning’ (to borrow from Churchill). Certainly there is enough of a story so far and a range of treatments to write about in case it is helpful to others.
It is worth noting before the story that I am very fortunate to have the support of my company, both in terms of the continuity of business given my absence and the financial support. I have also been very fortunate to have the wonderful support of my grown-up son and daughter and especially my wife. Her expertise as a trained nurse was invaluable; someone who understands the terminology, can navigate the way the NHS and private health systems work (which is contrary to my engineering experience) and also manage my prescriptions and pill-taking. At one point I was asked by a medic; “Where would you be without your wife?” “Well, probably not around!” I replied, in all seriousness.
My diagnosis in April 2021 of Autoimmune Encephalitis was of an uncommon type- Anti DPPX. Unfortunately this relative rarity did create problems and delays in the diagnosis and the resulting treatment.
Only in hindsight can I understand that this DPPX must have begun some 9 months prior to diagnosis in the Summer of 2020 and in the depths of the Covid pandemic. It started with such broadly diverged symptoms that the early efforts of the medics involved were thwarted and inconclusive. Collectively these symptoms included:
- Visual- magenta blinds spots in the periphery of vision and later some left-right flicking of vision.
- Behavioral- anxiety, exaggerated startle response and a few uncharacteristic outbursts of anger.
- Cognitive- some short-term memory loss and confusion.
- Physical- balance problems and some numb patches on feet and back
- Gastric- excessively loose stools resulting in rapid and significant weight loss.
- Sleep- disturbed, with prolonged waking after 2- 4 hours.
It is not entirely surprising that these symptoms did not result in a speedy diagnosis since they appear to be unrelated. The Anti-DPPX condition is too uncommon for most GPs to know and make a connection between the symptoms. GPs encounter all these symptoms and individually they all have more common causes. Separately, the medical specialists operate in domains where they will only be familiar with one of these symptoms and its underlying causes. What medical practitioner is there to connect optical blind spots and diarrhea to diagnose a neurological condition? So the condition is too uncommon for General Practitioners and too multi-facetted for many specialist consultants.
Consequently I saw an optician and an ophthalmologist for visual problems and separately a gastric specialist for a colonoscopy and endoscopy. I also had several MRI and PET scans. All of these investigations returned confusing results or were clear of an underlying cause.
In Winter 2020, and now at home and away from work, the anxiety, sleep and irritability were put down to Covid and work pressures. This was treated in hospital under the diagnosis of General Anxiety Disorder and Depression. It was not successful because it was treating a consequence of the DPPX and not the root cause. The other symptoms continued alongside and could not be explained by the diagnosis. My confusion continued, as did a general reluctance to do anything for fear of messing-up (apathy) and the desire to lie down (lethargy) and try to think problems through.
I had lost some of the past two years of memories and so hours were spent trying to remember and rebuild these by looking at files, photos and possessions. I cleared out a lot of drawers, hoarding and files in the process! Throughout this period there was also a sense ‘of not feeling oneself’, quite literally a personality change where one seems to inhabit someone else’s life and own their possessions and wardrobe. These objects would be recognised but only partially, as though they were someone else’s. Reality felt part broken and almost dreamlike. Confusingly, many dreams took on a life-like sense of reality. It’s a confusing condition that dissuades you from taking any significant actions or making any significant decisions for fear of making large mistakes.
Eventually, I did receive a diagnosis in April 2021 by a neurology consultant at Parkside, a private hospital in Wimbledon. Until then I have spent some considerable time watching daytime TV (I can’t abide the repetitive nature of Homes Under the Hammer or Border Control, but I do like Ramsey’s, Ottolenghi and Stein’s cookery programmes). Unfortunately the diagnosis had been missed in January 2021 because a blood test had mistakenly looked for a protein instead of the intended antibody but a repeated test in the Spring confirmed Anti-DPPX and from this more appropriate treatments could begin. I understand only that DPPX interrupts the Potassium channel of the (chemical) brain. It is no surprise then that thinking and memories feel partial, fractured or incomplete with the brain’s wiring disturbed.
My treatment progressed through the least invasive medications upwards. The first of these was in the form of oral Steroids, starting with a big hit and then reigning back to a moderately high dose. Sadly these are not of the body-building variety of steroids but thankfully they weren’t of the ‘moon-face’ variety either. However, they did seem to wipe-out any energy I might have had, or perhaps this was just the underlying DPPX. Who knows?
As I received treatment some of my symptoms changed or improved. My eyes and bowels were better and I had a passion for snacks and food, possibly as a result of the steroids. As a result my weight returned to its pre-encephaliits levels- although in a different manner. I joked with people that ‘I had regained all the right weight- but not necessarily in all the right places!’ (cf the great Eric Morcambe on the piano with Andre Previn) Sadly, muscle had been fat and a rather round tummy.
At around this time, Encephalitis International put my wife and I in contact with another DPPX sufferer they had heard of in the USA and we arranged several zoom calls. This couple were in touch with yet another survivor who was about two years ahead in recovery than the pair of us. This guy’s symptoms were even worse, being wheelchair-bound, but eventually he recovered and resumed his work as a personal fitness trainer. This story was tremendously encouraging and uplifting. It is far more reassuring and helpful to talk to fellow sufferers and especially to recovered sufferers than to read the medical papers about the symptoms and devastating effect of this condition.
The similarities between myself and the USA contact were bizarre and highly amusing. Not only are we both English, about the same age and from around the same place, we have worked for the same companies, both owned a boat we now couldn’t use and each had a son called Thomas. We wore the same glasses, had the same everyday watch and even had the same brand of water sports watch! I did start to think we were both cut from the same cloth – or we were the same software clone, albeit with a DPPX bug in it in the manner of the Matrix films. When the other US contact learned of these similarities, he asked “Have you two guys seen the Matrix?” I knew exactly what he meant! There may be something connecting DPPX to our similarities, Matrix apart. Who knows?
Steroids, I’m afraid, didn’t appear to offer much improvement in the condition, although they might have halted progression. I was thus referred into St Georges Hospital, London and into the care of the wonderful neurology and neuropsychiatry departments. The next planned treatment was Plasma Exchange. In this, the erroneous antibodies are washed out of your blood. This is taken from one arm, through a contraption crossed between a dialysis machine, dishwasher and water filter, and then pumped back into the other arm. It is not for the faint-hearted! I used to be quite squeamish beforehand but following 5 Plasma Exchange I am now cured – of the squeamishness that is – sadly not yet of the DPPX.
The next level of treatment I progressed to was Rituxomab, given as two IV infusions. Alongside these I continued to take a reduced dose of Steroids and the ongoing medication for Anxiety. I believe the Rituximab might have helped because it did improve my thinking, memory and most of all levels of energy and just the plain inclination to do things, albeit not significant ones just yet. Given this, I did decide to approach my To Do list from the bottom up. I even painted the front door and plated its furniture – an item I’d not got around to for over 20 years! Sadly I did need a further stay in hospital at this point to readjust my medication. There were patients with a variety of neurological conditions and injuries on the ward, some encephalitis, some stroke victims and more than one who had a tree fall on them!
I likened the shape (or profile) of my condition over time to that of a Bathtub with a rapid descent, a long and gradual improvement and then a rapid and short improvement. (The Bathtub Curve is an engineering principle meant to characterise the unreliability of machines, such as cars, through their lifetime). And so it is with rapid change that recovery can overshoot and I needed management of my drugs to avoid exceeding normal behaviour. (This is also an engineering term called under-damping when things overshoot their target, whereas critical damping is when systems reach their target in the shortest time and don’t overshoot.)
Many of my symptoms have subsided in the time since the Rituximab infusions, although the short-term memory loss and left-right visual flicking problems still partly remain. I said at the beginning of this story, that I haven’t yet reached the end. The next treatment planned is a series of six Cyclophosphamide infusions over three months. This is a rather powerful chemotherapy drug with some unsavoury side effects I’m not looking forward to, but, those apart, I am looking forward to any improvement it can provide and a return to as normal a life as possible.
