A question that often occurred to me when I was a GP was – Is this patient really ill?
Some people obviously weren’t because they came about something else – for general advice for instance. But of the ones who perceived themselves to be ill, were they? Usually the correct response for a doctor is to say – if the patient thinks they are ill, then they are. But it can be a very difficult question when you are in the consulting room.
Take the patient who is tired all the time, with very low energy levels who gradually becomes disabled, not able to walk far or has to give up work. All the tests are negative and ME is diagnosed. But that seems to be a slap in the face to the patient; though there is a diagnosis; there is no cure, no recommended management pathway and no prognosis. It isn’t a “real illness” and patients don’t get to be under the care of a hospital consultant or treatment plan. Is it all “in the head”?
Well, yes and no. No because the illness is real, something is going on which has real effects. But yes, because new ways of thinking about this have come to the conclusion that the symptoms are the result of unusual neuronal circuitry in the brain. Our eyes, ears, nose, and skin send data to the brain all the time, through the nervous system. But it seems that our conscious selves do not interpret this data in its “raw” state – it always goes through pathways in the brain that have internalized prior experiences and predictions, and this may alter the experience of the raw data. This can be beneficial, as we have memories of similar data sets in the past and these can colour the information that the brain now has to process. So with standard illnesses such as arthritis, different people can have very different reactions to objectively similar levels of illness, and the outcome is correspondingly better or worse, and this may be because of their prior expectations. It might even be an explanation of the well-known placebo theory, where just the expectation of benefit can result in improvement in symptoms.
But sometimes, very rarely, it goes wrong, and our past experiences override the raw data leading to unexpected symptoms, which may not be what the data is actually telling us. This leads to the brain “imposing” on the interpretation of the data so that the patient experiences symptoms such as weakness, paralysis and other neurological dysfunctions.
I’ll use an example from when I was practicing as a GP. A lad in his twenties, a slightly solitary, but very pleasant farm worker came to see me with symptoms in his left arm, below the elbow. He complained of pain in the skin and a dull ache inside his forearm together with some weakness in his hand. It had started he said when he had to do a lot of sheep dipping. (There was an epidemic of a parasite in the area at the time). His left arm was inside the sheep dip for long periods, and he felt the symptoms shortly afterwards. He had looked up the leaflet that came with the liquid, and there were lots of side effects, but none of them fitted his symptoms. He felt that the sheep dip had damaged his nerves, and was very worried about it, understandably. I knew him to be a bit of a worrier, and I took his symptoms seriously, examined his arm, his neurological system and did some blood tests. I found with pinprick testing that sensation was reduced below the elbow. I reassured him that it must be a superficial effect of immersion in a fluid on his skin for longish periods and asked him to come back if it didn’t settle.
But I didn’t think for one moment that his nerves were damaged. This is because nerve damage has to occur along the pathway of the nerve in question. In this case the nerves to the lower arm start in the neck and the fibres within them have crossed and merged several times. If the damage had been to the end fibres in the hand and fingers and had crept upwards there is no way that the cut off line would be the sharp demarcation that he had shown me. So I was surprised to see him back two weeks later with the symptoms having got worse. As his worry was specifically about the sheep dip, I gave him a note to say that he wasn’t to use it from now on. Fortunately, the programme for treating the sheep was coming to an end and it didn’t affect the farm work. But he came back several times, still worried, and asking for referral to a neurologist. I tried to demur, but eventually did so. Waiting lists for neurology were then, as now, very long, and six months later I saw him about something else. I asked him about his arm and he smiled and said the symptoms had all gone. I felt that the fact that he now had a new supportive girlfriend had a lot to do with it! The symptoms had been entirely real to him, but now that his brain’s activity was concentrating on something else more positive, the circuitry could bypass the expectations he had had and allow the true data through.
Another example, not from my practice, was a young woman who had a soft tissue lump on the left side of her head, which was removed by a dermatologist, and was benign. But she became convinced it was cancerous, and after several months she suddenly experienced weakness of her left leg, the same side as the lump. She was admitted to hospital, but shortly after her arm became weak as well, She was thoroughly investigated and no sign of a stroke was found. She was told that it could not be a stroke related to the lump on her head because if it were, the stroke would have affected the right side – the nerves cross over after leaving the brain, so that a right-sided lesion would cause left sided paralysis. She was discharged with intensive physiotherapy, but recovery took years even though she seemed to accept that it could not be cancer due to the lump on her head.
These are examples of what we now call “functional” illnesses, because they cause problems with functionality with no anatomic basis at all. They have been known for centuries; such illnesses were common in the Victorian era and were often considered “hysterical”. But they were very real to the patient and doctors have taken a long time to really appreciate what such patients are suffering.
But many acute neurological events that turn out not to be due to physical illness, come on extremely rapidly when the patient has no expectation at all that something may be not quite right, unlike in the two examples above. The acute symptoms, which could be exactly like an epileptic fit, or a stroke or paralysis, are a great shock to the patient who may have been very healthy previously. And unfortunately the symptoms may not go away and may be completely life-changing, with people having to give up their job, becoming dependent on others and having real problems, including with accessing benefits in order to live. So whatever previous experiences or thoughts that were altering the mind’s interpretation of the symptoms must be buried pretty deep, or be caused by a faulty series of neurological connections which weren’t conscious at all.
Research has been done into what sorts of people were prone to this syndrome. It is common, comprising about 16 % of patients attending neurological clinics; so that neurologists get very expert in deciding which patients have it. It is easier to be sure that there is no anatomical basis for their symptoms in neurology than in other disciplines because neurology pathways are very specific and not often what patients expect, as in the two cases above. Women are more often affected than men – nearly 70% of cases. However, women’s problems have historically been taken far less seriously than men’s so this may not be a true proportion. It can be triggered by physical illness or disease – a car crash for instance where pain or weakness lasts much longer than the injuries themselves. Sometimes stress can cause it. Sigmund Freud believed that it was always due to a past psychological trauma. (Well he would, wouldn’t he)? But a recent meta-analysis revealed that 24 per cent of people with a functional neurological disorder had been abused in childhood, against 10 per cent in a comparison group, so though it may be a factor it isn’t a common or necessary trigger.
The first challenge is to make a positive diagnosis of FND – functional neurological disease. In a case of paralysis or tremor, the neurologist has to rule out diseases such as stroke or MS, both clinically and by doing the relevant tests. But they also have to look for positive signs that might indicate FND. For instance, in a case of weakness or paralysis of one leg, you see a characteristic gait where the patient drags the leg behind the body as a single unit with the foot pointing inwards or outwards. Or the patient may walk with excessive slowness, especially with difficulty starting a movement. These are different patterns from those seen in MS or Parkinson’s. With severe tremor, a key positive sign would be change in the tremor when the patient’s attention is distracted. This is shown by the “entrainment test”, entrainment being a technical term for when the tremor frequency switches to match exactly the frequency of a voluntary rhythmical movement performed by the unaffected limb. If you take someone with a “functional” tremor in their right hand and ask them to hold out the hand, the tremor is more pronounced than when they are resting. Ask them to also hold out their left hand and tap it on the table to a beat and, with attention focused away from their right hand, it may either stop shaking, or it could pick up the same rhythm as their left. If you ask a patient with a Parkinson’s tremor, or benign essential tremor, to do this, it stays the same. There are many such examples where the responses of a patient with FND will differ from that of other neurological diseases, and the neurologists will be certain that this is indeed FND. It isn’t just a matter of excluding all other diseases. So it is definitely not a matter of a patient making it up – if it were there would be no consistency in what the symptoms the patient would get.
But sometimes there may actually be more than one process going on – a patient can sometimes have a functional disorder at the same time as an organic disease. For example a 38-year-old woman came to a clinic with a whispering hoarse voice that doctors decided was definitely a functional symptom. She then developed progressive mobility symptoms with unsteadiness, slowness and a change in personality. Doctors were not at all sure what was going on, and thought it was all functional. Then she developed drooling, unsteadiness, a parkinsonian tremor and finally became completely mute. Eventually doctors discovered she had Wilson’s disease, a genetic disease where copper builds up in the body causing neurological symptoms and liver disease. The original hoarse voice was indeed functional, but everything else was organic. Only then was she able to get the correct treatment.
So what about treatment? There is hope. Many neurologists are now referring their patients to physiotherapy departments, which are trying new methods of re-educating or re-training muscles to work properly and to overcome the faulty messages that the brain is sending out. Some of them are based on that entrainment test, which indicates that functional symptoms tend to decrease when people are distracted. It is possible to show people how their symptoms abate under distraction – sometimes you can film them as they moved their leg, for instance, to help convince them the muscles were working normally. Sometimes people can use their own distraction techniques if symptoms began. One example was of a patient with functional seizures, simply running her fingers over the holes in her walking stick, which provided enough distraction to seem to prevent symptoms and return of seizures. It made her brain thinks of something else, she said.
The inference was that distraction was enabling the “real” data to come through the circuitry in which expectation had become dominant.
So it has become more routine to refer patients with FND to speciality physiotherapy clinics, where patients get one-to-one therapy. There is a U-tube video which shows this being done very movingly.
There is some evidence that it works.
In one study, 60 patients with various functional disorders were enrolled into an experiment where the intervention was done according to a carefully constructed 5-day programme, delivered by a neurophysiotherapist who had undertaken additional specific training.
It took into account events that seemed to trigger symptoms, other concurrent diseases, psychological factors, attention focused too much on the self and unhelpful reinforcement of symptoms. Movement retraining was developed aimed at restoring normal movement by redirecting the focus of motor attention. There was a control group which received more usual physiotherapy; and the results were analyzed after various periods of time. It was found that the intervention group did significantly better than the control group and continued to improve after the programme was stopped.
These all seemed to be sensible methods of helping people to re-programme their muscle activity towards more normal activity.
However the take home point for me was the use of targeted physiotherapy early and to focus on re-training of muscle movement. We all know that lack of use itself is the cause of severe disability. If you don’t use it you lose it and for patients with paralysis it is essential to continue movement as much as possible. The intervention should be done early so that the least damage is done by inactivity. If a patient has been in a wheelchair for years, recovery is going to be very difficult whatever the initial reason for the disability.
These new thoughts about FND fitted with what happened to the patients I saw in my surgery. Most of their symptoms were relatively mild; if there had been symptoms of epilepsy or paralysis they would obviously have to go to hospital. They tended be slightly anxious and to worry about their health. But if they became convinced that they did not have an on-going progressive disease, and believed the results of negative tests, then they did tend to improve, especially if investigated early and thoroughly. However quite a few patients were never convinced that their symptoms were functional. In particular I had patients who were convinced that the real cause was some pollutant in the environment or medications that they had been taking which had hitherto unrecognised side effects. Although none of my patients were diagnosed with this, in some cases neurological side effects of medications were found and published in the literature. Obviously this would be a great tragedy if it had been missed, and presumably compensation would have to be paid. I am sure there are examples of this sort of thing especially with young people and children who can be extra sensitive to pollutants.
I think it is extremely important to distinguish between the group of patients who are likely to have functional illness, which is very real to them and is undoubtedly in my mind caused by a real malfunction even if it cannot be exactly pin pointed by neurologists, and those who are actually faking it in order to get a benefit for themselves, be it financial, extra attention, or true malice. To reassure those who are not faking it that their symptoms are real, as well as to maximise their changes of recovery, they deserve as much attention from doctors, nurses and physiotherapists as if they had a more standard neurological problem. After all it is quite clear that the prognosis without help and treatment for those with FND is even worse in many cases than for those with MS, Parkinson’s and so on. There are an awful lot of such patients– there are many milder cases who remain in general practice and see their GPs on an on-going basis, and the 17% of all neurological referrals who are diagnosed as having a functional neurological disorder. Slowly, the health service in many areas is coming to realise that they deserve better than the treatment they have had in the past.
Neurologists do actually have to get over their own preconceptions. Comparing MS and FND from a neurologist’s perspective, it was estimated that the “popularity” of MS with neurologists is about 90% while with FND it is about 5% – in other words neurologists don’t really like seeing patients with FND. They often think it is not their business. And public understanding of MS is 95%, while FND is about 2%. Very few people know about FND, let alone understand it.
Considering that new diagnoses of MS in a neurology clinic are about 6%, while FND is much commoner at 16%; and the disability level for both is the same at 7%, you can see that the FND patient does not usually get a fair crack of the whip. I do hope that attitudes in the medical profession, and in the public at large continue to change in the right direction so that such patients get a much better service in the future.
References
New Scientist: The illnesses caused by a disconnect between brain and mind
https://www.newscientist.com/article/mg24232240-100-the-illnesses-caused-by-a-disconnect-between-brain-and-mind/#ixzz63HVJkdZl
Health 3 April 2019 Clare Wilson
FUNCTIONAL SYMPTOMS AND SIGNS IN NEUROLOGY: ASSESSMENT AND DIAGNOSIS
J Stone, A Carson, M Sharpe J Neurol Neurosurg Psychiatry 2005;76(Suppl I):i2–i12. doi: 10.1136/jnnp.2004.061655
Functional symptoms in neurology: mimics and chameleons
Stone J, Reuber M, Carson A. Pract Neurol 2013;13:104–113.
Neuropsychiatry: Research paper
Randomised feasibility study of physiotherapy for patients with functional motor symptoms
G Nielsen1,2M Buszewicz3, F Stevenson3, R Hunter3, K Holt2,4, M Dudziec2, L Ricciardi1, J Marsden5, E Joyce1, MJ Edwards1,4
How to use the entrainment test in the diagnosis of functional tremor
http://dx.doi.org/10.1136/practneurol-2013-000549
Louise S Roper1, Tabish A Saifee2, Isabel Parees2, Hugh Rickards3, Mark J Edwards2
U tube video : My Functional Neurological Disorder (FND) Recovery Story
Jamie Lacelle

