Encephalitis: The signs of the life-threatening condition which sees the brain swell, the need for early diagnosis and what support is available

Wednesday, 22 February, marks the 10th anniversary of World Encephalitis Day. The global awareness day for those affected by encephalitis is set to be bigger than ever this year.  People around the world are walking, or wearing red, to shine a light on this condition. 

Even Niagara Falls is rumoured to be lighting up red this year as well as other key monuments from around the world going #Red4WED in support.

The importance of awareness of the condition cannot be understated. I’ve seen first-hand the devastating impact encephalitis has had on my clients and their loved ones. It can affect people at any age and from any walk of life. 

What always strikes me when I attend conferences and work with experts in this field, is that encephalitis is much more common than we might expect. There are reportedly up to 6,000 cases in the UK each year and hundreds of thousands worldwide. 

What is encephalitis?

Encephalitis is brain inflammation caused either by an infection invading the brain - infectious encephalitis - or through the immune system attacking the brain in error - post-infectious or autoimmune encephalitis. It can be life-threatening and requires urgent treatment in hospital.

What are the symptoms?

It isn’t something we can prevent as such, as any virus has the potential to produce encephalitis. Every person seems to be affected differently, but there are some key signs and symptoms to look out for. 

Infectious encephalitis usually begins with a headache or flu-like illness which deteriorates over hours, days and weeks.

The key symptom is then an alteration in the level of consciousness which can present as mild confusion or drowsiness.  In some patients, they can completely lose consciousness.  

Other symptoms include fever, seizures, aversion to bright lights, loss of control of speech or body movement, sensory changes and neck stiffness. 

Another symptom which I’ve heard from families time and again is that their loved one was presenting with uncharacteristic behaviour such as aggressions and lack of co-operation.

Autoimmune encephalitis can come on more gradually but symptoms may include confusion, altered personality or behaviour, psychosis, movement disorders, seizures, hallucinations, memory loss, or sleep disturbances.

The need to timely diagnosis

I’ve worked with families where there’s a suspected delayed diagnosis. One of the first difficulties faced by clients can be trying to get that initial diagnosis because the symptoms set out above can be misdiagnosed as something else.  

It can be incredibly frustrating when there’s a misdiagnosis at first because the loved ones know that person best and are best placed to say that something simply isn’t right. 

The cases I’ve seen have been where perhaps the alarm being raised by family members wasn’t taken seriously enough. I also see cases where the patient’s symptoms can be dismissed as something else. For example, I worked with a family who repeatedly took their daughter to A&E because her behaviours were completely out of the normal for her; however, they were sent away each time with different explanations. 

A common misdiagnosis is to attribute the problems to mental illness. For many of these families, one of the most distressing things about the diagnosis is that they felt ignored at the start.

What treatment is available? 

Patients can only do so much by reacting to the symptoms and seeking help. Hospital tests are needed to confirm the diagnosis including lumbar puncture, brain scans and blood tests. It’s important that investigations are carried out as soon as possible because the sooner treatment is started, the better the outcome.

Treatment depends on the cause of encephalitis: antivirals are prescribed for viral types of encephalitis, antibiotics for bacterial types and immunomodulatory drugs for autoimmune encephalitis (such as steroids and plasma exchange).

The devastating impact of delayed diagnosis

Nerve cells may be damaged leading to an acquired brain injury (ABI) from which no two people will have the same outcome. The brain recovery from an illness or injury can be a long and slow process.

Therefore the impact of living with and rehabilitating from encephalitis is devastating and completely life-changing for the patient and those around them. It can cause physical and cognitive disabilities which affect movement, requiring care and assistance and preventing that person returning to work or study. It impacts their ability to communicate and function as they did before. 

The treatment with steroids appears so varied.  I’ve seen clients improve through rehabilitation at such differing rates – with steroidal treatment seeming to have an effect but also getting in those vital rehabilitation services early such as speech and language, physiotherapy, psychiatry and occupational therapy.  The main aim of rehabilitation in to help the person affected by encephalitis develop new skills, habits and strategies for coping with their remaining difficulties.  

For the clients I work with, another hard part of their journey is not knowing the future. Compared to other injuries or illnesses where there might be a clear timescale for recovery, there is so much uncertainty around the recovery process which is in itself unsettling.

Helping establish answers

If a patient or their family suspect there has been a delay in diagnosis, or inadequate treatment given, the first thing they usually want is answers and an explanation. 

This is where we become involved. We can undertake an investigation outside of court, working with independent experts in fields such as neurology, to establish what happened, whether anything could have been done differently and if there were failings whether these amount to clinical negligence. 

The injured person will then know whether they have a legal case to pursue, and at the very least they come away with answers.

If we prove a legal case, then we as medical negligence lawyers, we switch our focus to the impact on that person’s life. 

We’ll assess with clinical experts how different the outcome has been for that person, as a result of the negligence, what's the impact on that individual now and for the rest of their life and what care, support and therapies they may require.  

The Encephalitis Society 

The Encephalitis Society lead the way with funding research through its Scientific Advisory Panel, raising awareness and supporting those families affected. Whenever I have been to its conferences, there’s a real community feel despite the global reach. 

Recently I was delighted to attend the Annual Encephalitis Conference at the Royal College of Physicians in London.  It was fascinating to hear about the global research under way with clinical trials in Autoimmune Encephalitis. 

I'm always fascinated to hear Professor Tom Solomon speak and his invited guest lecture was so important. A topic keenly followed by all the families I've worked with is what difference steroids make to improve patient outcomes. 

This is the question I've always focused on in my own cases where there is an alleged delay in administering the right treatment at the start (usually because of a delayed diagnosis). The data still needed to be analysed, showing there is still so much we don’t know, and more research is needed. 

We need to support the Encephalitis Society as best we can and continue to raise awareness both on Wednesday and throughout the year.

Find out more about Irwin Mitchell's expertise in supporting people and families affected by encephalitis and other care issues at our dedicated medical negligence section.