Steve’s Story – From the outside Summer 2018.

Read Part One

The start

Looking back there were signs from as early as July 2018 (writes Lisa). Steve had completed a half marathon on a hot day and, in the weeks afterwards, he started to describe his calf muscles as ‘bags of snakes’ – they were constantly twitching (fasciculation).

He reported vivid dreams and problems sleeping. He was uncharacteristically stressed about his work as a trainer for Social Services. The first symptoms that sent him to his GP were dizziness and nausea. He then started complaining of a strange sensation in his chest which he found difficult to describe. He said it was quite a pleasant situation, as if something exciting was going to happen.

The GP did blood tests and checked out his heart which were all normal. He was prescribed propranolol for anxiety and a sleeping tablet.

October 2018 – Sudden memory loss

One Tuesday evening, I came home from work to two confused messages on my answerphone from Steve. I called him to see what the messages were about, but Steve couldn’t remember leaving them.

I went over to his house. He was about to eat his dinner uncharacteristically early. He wasn’t aware what time it was and couldn’t tell me what he had done that day. He didn’t know if he had been to work or not.

On the advice of 111, I took him to hospital. He underwent a CT scan of his head which was negative and blood tests which were all normal. He underwent a thorough physical examination and was seen by the on-call psych team. As a stroke and a brain tumour had been ruled out by the scan, the working diagnosis was anxiety.

The Doctor wanted to admit Steve for further tests but he refused to be admitted and so he was discharged home with an urgent referral to the local mental health team. Over the next few days, his behaviour varied from relatively normal to strange. His memory seemed back to normal.

Occasionally, he would stutter and turn his head to the right, usually if he was talking about work. I accompanied him to see his GP. A couple of times in the waiting room, he shouted out random words. His strange behaviour worried the GP and we agreed that I would take his car keys off him. She got on the phone and chased the referral to the mental health team.

A couple of days later, I was woken by Steve at my front door. It was 3am, dark outside and he was wanting his car keys to drive to work. Steve prides himself that he can guess the time correctly to within 15 minutes.

That day (and for about two months afterwards) he couldn’t tell the difference between 3am or 3pm. I took him back to his house and installed myself on his sofa which is where I spent the next few nights until he was admitted.

He was barely sleeping, and if he did, he physically acted out his dreams during which he was training people. Writing this it seems so obvious that there was something seriously wrong.

However, at the time his symptoms were transient and variable. The side effects of the medication he was on included paranoia, confusion, memory loss and peculiar mannerisms. Investigations had been normal.

Mid October – Admission to hospital

A week after his first visit to A&E, Steve was back there following strange behaviour at work. He underwent yet more tests. After several hours and more examinations, I was told that the most likely diagnosis was dementia.

Then there was a breakthrough – a positive test. His sodium levels were too low (hyponatremia). I was relieved to hear that this was treatable and could be the cause of his symptoms. He was admitted. Treatment involved restricting his fluids over a number of days. Steve underwent more scans to rule out a tumour (which can cause sodium ion imbalance).

At this stage, Steve was permanently confused. He knew that he was in hospital, but thought he was there assessing the staff. Initially there was some improvement as Steve’s sodium balance returned to normal but then his symptoms worsened again. He was restless, still not sleeping and would wander around the ward. He refused to put his things in the locker by his bed and he always had his bag packed ready to go.

In the first week he went missing twice from the ward, trying to make his way back home. After the second escape attempt, he was put under a DOLS (Deprivation of Liberty) for his own safety. This meant he had a staff member permanently assigned to him. In the first few days of having one-to-one nursing, his allocated staff member would be exhausted trying to keep up with Steve’s wanderings.

Now that his sodium levels were normal there was no obvious reason for the symptoms to continue. The working diagnosis returned to anxiety and I was told that the next day he would be sectioned.

However, the next day, nearly two weeks after being admitted, Steve had an EEG which showed that he was having seizures. The strange grimacing and arm movements turned out to be facial brachial seizures. This was followed by an MRI which showed inflammation on his brain. He had encephalitis.

At this point the most likely diagnosis was an autoimmune encephalitis (AE) as other types of encephalitis were ruled out with blood tests. We waited weeks for his autoimmune screen to come back from Oxford which was positive for the LGI1 antibody.

Meanwhile, Steve continued to deteriorate. I had to take his mobile phone off him as he was calling friends in the middle of the night confused. He became sleepier and weaker. He lost 11 kilos of muscle and could no longer stand for more than a couple of minutes.

Every day his memory got worse. The doctor would ask him each day where he lived and Steve gave older and older addresses until he was telling him that he lived in Ruislip where he had lived decades ago. He took 20 years off his age when he was asked how old he was.

His cognitive function worsened – he didn’t know how to shower himself and couldn’t work out what to do with his deodorant, applying it to his hair when handed the bottle.

At times, some of his friends were not sure if he recognised them.

November – Treatment

I came in one day and the curtains were drawn around Steve’s bed. The Doctor was trying, without success, to rouse him. Later that day the team started him on intravenous steroids even though AE had still not been confirmed.

The steroids had an amazing effect, and I am certain that if he hadn’t been given them at that point he could only have been days away from dying. Following the steroids, Steve became more alert, physically stronger and his cognition improved.

He was still confused much of the time and spent hours trying to train the staff and patients around him. Occasionally he thought he was in New Zealand travelling and another time he was convinced he was in a restaurant.

He was then given intravenous immunoglobulin (IVIg) for 5 days. His arms were full of bruises and scabs from where he would pull out the cannulas delivering his treatment. The IVIg helped with his confusion but his memory was still impaired.

He could never remember meeting the Doctor, even though he saw him daily. He couldn’t retain for more than a few minutes what was wrong with him. He didn’t know what he had eaten an hour earlier, ordering coronation chicken sandwiches almost every meal.

As he showed an improvement in his confusion, the team decided to move him to a neuro rehab centre. He had a second round of IVIg before they transferred him. This helped less, and in fact he became more confused and sleepy before the transfer.

Christmas to March – Rehabilitation and further treatment

Not long after moving to the rehab centre (and two months after his original admission) the confusion resolved. His short-term memory remained an issue and I was advised to buy him a diary so he could write down what he did each day.

Steve was reluctant to engage with the rehab sessions. He was so frustrated by his lack of fitness that it was hard to get him to go walking or running in the park during his physiotherapy sessions. He hated being tested psychologically as he was aware that he wasn’t functioning at his normal level. He was angry that he was not allowed to leave the rehab centre without a friend or staff member accompanying him.

Gradually Steve’s memory improved. The staff were kind and patient. Not being able to remember his passwords, Steve was locked out of all his accounts and devices. The Occupational Therapist took him to the bank and mobile phone shop so that he could start to reclaim his former life.

One thing that wasn’t improving was his seizures. They were getting worse and more frequent. He had another EEG which was videoed and reviewed by the team at St George’s Hospital. It was decided to transfer him to St George’s for a plasma exchange. He had 2 rounds taking 5 days each.

He continued to improve – how much of a difference the plasma exchange made at this point I don’t know.

Mid-March 2019 - Home coming

Steve spent five months as a patient in three different hospitals. His treatment will have cost a six-figure sum. The staff in all three hospitals were amazing. In the middle of March, I picked Steve up from St George’s to drive him home. It was an emotional moment.

For the first months of his admission it was doubtful that he would be able to return to living alone. The fact that he was going back home, independently was incredible and is a testament to both the NHS and to Steve’s determination. In the first few weeks at home he had occasional outbursts of anger. It took another couple of months for Steve’s personality and sense of humour to return.

A year after admission

Steve’s seizures are still frequent but much less so than the 10-20 an hour he was having in St George’s. He is still on treatment and has just started mild chemotherapy as an outpatient.

The hope is that the chemotherapy will dampen down his immune system and the seizures will improve. Steve finds crowds difficult, he is more impulsive and less inhibited than before, being quite direct with people at times. These were all traits he had before but they are now more obvious.

Whether this is due to the encephalitis, or a reaction to a life-changing experience, is uncertain. He has returned to work part time. He has regained his fitness and just over a year after he was admitted he completed Beachy Head marathon. His memory of 2018 is virtually wiped (perhaps a blessing for the months of his acute illness) but his short-term memory is back to normal. He can once again correctly guess the time to within 15 minutes.

About autoimmune encephalitis

AE is extremely difficult to diagnose as it can masquerade as many different more common conditions. I have worked in health care for over 20 years and I had never heard of AE before Steve’s diagnosis. The first antibody specific AE was only identified in 2005 so research into the disease is in its infancy. The doctor treating your relative or friend may never have seen a case of AE before - they may not have heard about it. Learn as much as you can about AE and its treatment. Contact the Encephalitis Society for information and support if needed.

Read Part One - Steve's Story