Looking back now on Edith’s descent into her own private amnestic world, Harold started to worry that he too was developing the dreaded Alzheimer’s. Yet, he reassured himself, he had no other memory problems. The bills were paid on time, he enjoyed (and remembered enjoying) meeting his old friends for bridge on Tuesday evenings, and, although he struggled to actually see some of the cards at times, he was perfectly able to follow or recall the gossipy conversations of the previous few games.
After a few days of spring-like weather, his street urchins and medieval knights had not turned up, so he reasoned he had simply been under the weather or that his new blood-pressure medication had caused these aberrations in his garden.
All was fine for a few weeks and, not one to bother doctors with trivialities, Harold decided to dismiss it from his mind. It was only when he awoke one night at about two o’clock in the morning to see Santa Claus in a helicopter ghosting over the wardrobe in his bedroom that Harold finally decided to seek medical help.
I saw Harold in the outpatient clinic in Charing Cross Hospital. He could not have been more gentle or kind to me. He tolerated my incessant questions with a smile. He was a very healthy 73-year-old retired schoolteacher. He never smoked, and rarely drank alcohol. There was no history of Alzheimer’s in his family, and from his eloquent but succinct responses Harold was patently in full control of his cognitive faculties.
Surprisingly, he seemed untroubled by most of the ‘visions’, as he referred to them. It was the arrival of Santa Claus that had frightened him into attending his GP, who sent him to our neurology clinic.
He cleaned his glasses as we spoke and asked whether I thought he might need a new prescription. He told me he had had cataract surgery a year or so before, and that he also used drops for early glaucoma.
Harold was presenting with a classic example of Charles Bonnet syndrome. This is usually caused by damage to the visual system such as by glaucoma, cataracts or macular degeneration. It can also be due to damage to the brain areas concerned with vision when the wiring connecting the eyes and the visual area of the brain – called the occipital cortex – is disrupted. It is thought that when the visual input to the brain becomes less than clear – literally because you can’t see well or when a part of the brain to do with sight is damaged – surrounding areas of brain take over from their ailing neighbour, but they don’t always succeed. In other words, parts of the brain begin to ‘make up’ images to replace the indistinct images it is receiving.
When they were asked directly, up to 15 per cent of people with impaired vision reported this astonishing phenomenon, which tends to occur in older people because their vision is more likely to be impaired, although it is not exclusive to the elderly. People are slow to report these visual hallucinations for fear their friends and relatives will think them mad or take it as a sign that they have started to develop dementia. Like Harold, they soon become accustomed to the bizarre visions, and some people even begin to find them comforting.
Apart from reassuring patients about what is taking place, and that they are not going mad, mostly treatment addresses the cause of the visual impairment – say, cataracts or glaucoma – and anti-epileptic medications can also help.
It is intriguing to listen to people describing hallucinations of any kind. I saw a retired nurse who had developed high blood pressure and kept seeing the cast of Fawlty Towers in a mosaic pattern across her living-room wall. Another man described intricate patterns moving in waves across his bedspread. And yet another began to see the world and the people around her in miniature form (micropsia). Sometimes the images are recognizable scenes from a person’s past, but more often they make no sense at all to the patient or anyone else. One cannot but wonder if there is not a vault of visual memories buried deep within all of us only to be released when our brains break down.
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FIRST IMPRESSIONS
While rare and colourful cases captivated me as a young doctor, before long I came to understand that the rhythm and shape of my working life would be formed by the commonplace as least as much as, if not more than, the exotic. Of course, in neurology the seemingly commonplace can be devastating. Conditions such as MS and Parkinson’s disease change people’s lives dramatically; others, like motor neuron disease are usually death sentences. So, while the daily business of being a neurologist might have less excitement than I anticipated, it has greater depth and richness.
Each week I meet at least twenty new patients, and up to 100 others who are attending for regular review. On Tuesday morning the neurology clinic receive more than thirty people for review. Appointments are booked every fifteen minutes with myself or a neurology registrar from 8 a.m. I will usually start before that for the early arrivals. The old ‘the doctor will see you now’ – the condescending call from a loyal secretary of many years to the next poor patient waiting in the cold and damp waiting room to see the great doctor – is rarely heard any more. No one would put up with such a lofty turn of phrase now, and rightly so. Instead, our receptionist will get the patient’s file ready and, if I do not know the patient already, I will look at the GP’s referral letter. Once I have the essence of why they’ve been referred, I will go out to the waiting room and call them in myself, always by their formal title.
I once called a middle-aged man, whom I had never met before. He was barking away into his mobile, upsetting everyone else. He turned nonchalantly towards me when I called his name.
‘How are ya, horse?’ he shouted over his shoulder as he stomped past me. Here was a confident man, and he wanted everyone to know it.
Most patients nod to their loved ones, who mouth ‘good luck’, and quietly make their way across the room. I shake hands and introduce myself. From this initial engagement I get a sense of what the appointment is going to be like. Whether it’s ‘How are ya, horse?’ or ‘Good morning, Doctor’, this moment can be very telling. There are as many kinds of first meeting as there are people in the waiting room. It’s a combination of what’s going on in patients’ heads and their baseline disposition. For some, a weak leg means they will be in a wheelchair and unable to care for their children. For others, they feel they are probably wasting my time fussing over an old rugby injury that has simply raised its head after many years. So, depending on their outlook, some will shake my hand with confidence – or, indeed, squeeze the life out of it to confirm their belief that they’re as hale and hearty as ever – while others can barely raise their gaze to meet mine. And, yet, to all appearances, the confident man may be just as fearful as the quiet one, so I try not to make assumptions based on either approach.
There are a few other things that I look out for when I call a patient from the waiting room. Do they faff about to pick up their things and come in (not keen on being here) or rush in (too keen on being here)? Many a follow-up patient in the afternoon clinic will have bags from the posh shops of Dublin, as their annual visit has become a daytrip to the big city; perhaps shopping distracts them from the business at hand. Have they missed previous appointments, and do they apologize for doing so? This may seem terribly judgemental, as I don’t know my patients personally, but that’s precisely why it’s important. Taking note of a patient’s little quirks helps to build a picture of their personality, and will help me figure out how best to attempt to relax them as they enter the consulting room. It also helps me to understand how afraid people are. The very concerned come hours before they are due to be seen, like myself going to the airport in fear of missing a flight. Furious gum-chewing might indicate a pre-doctor smoke to calm the nerves. The less concerned arrive, check in and go and get a coffee. The fresh cappuccino placed firmly on the doctor’s desk suggests an airy confidence that nothing is wrong as far as they are concerned; the truly worried rarely think of coffee when their long-awaited appointment has finally arrived. Some people are serially late, and nearly always the ones who take up the most time when you are at your lowest ebb at the end of a busy clinic
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How someone dresses when going to see the doctor can be instructive too. Missed bits of unshaved facial hair on a man’s chin may suggest a lack of feeling on one side of the face; a tremor might lead to multiple nicks while shaving, some fresh, some healed. A single scuffed shoe might point to a long-standing foot drop and an incompletely buttoned shirt suggests frustration at failing manual dexterity. All are potential indicators of neurological dysfunction – but, of course, they may also be nothing.
The next step is to direct the patient to the consulting room. ‘First on the left, please.’ I’ll hold out my hand to indicate the way, so that they will lead on, the better to see how they walk. Many a diagnosis of Parkinson’s is all but made by the time someone reaches the consulting-room door. In most cases an expressionless face on greeting, a shuffling gait and a reduced swing of one arm as they take the few steps towards the consulting room is more than enough information to know what is going on.
Other problems can reveal themselves in that hallway; some stroll right past the open door on the left and start opening doors to the right. True, some are just too nervous to take in an instruction as basic as ‘first on the left’, but if nerves don’t seem to be a problem, I immediately worry about cognitive function and dementia. An alarmed husband or wife will have become used to such mistakes over the preceding weeks or months and will know to guide their spouse to the room. When I see these behaviours, and we have yet to sit down, the possibilities are clear and the tenor of the conversation is set.
The next half an hour can be like a dance. How to tell them what they already most likely suspect? Patients rarely ask straight out, in the hope that they are wrong, so it’s a question of gauging their state of mind.
Sometimes it’s not the patient’s behaviour but who they’ve come with that helps the doctor. When young Irish women arrive with both parents I guess that MS is the big concern. Fear of MS is deep-rooted in the Irish psyche for reasons I have never been able to fully understand. It seems to be taken as read that any young woman with neurological symptoms has MS until proven otherwise. It’s true that multiple sclerosis is very common in Ireland, and that it is more common in women than in men. A lot of people I see will recall an aunt, a distant cousin or a grandmother who had the condition, and the stories of their original symptoms seem to enter into family lore for generations. This probably dates back to when treatments weren’t as advanced as they are now and we saw more young people in wheelchairs. Also, decades ago, in a time before MRI scans could provide more precise diagnoses, MS was often given as the most likely explanation for neurological problems of all kinds. But to this day non-medical folk regularly assume any neurological disability in young people, particularly women, to be MS.
I’m not sure at what age anyone should go to the doctor on their own, but it surprises me how often someone in their thirties (or even older) will be accompanied by their parents – but not their spouse. Is there a problem in the patient’s marital relationship that is manifesting as a psychological symptom – such as arm or leg weakness or persistent pins and needles? Or are they so scared that they have something serious – like MS – that they don’t want to tell their spouse?
And how much is there to read into a teenager arriving with one parent and not the other? Has there been a separation or divorce that has led to the teenager becoming stressed and presenting with neurological symptoms, or is the other parent just at work? As extreme – or over-analytical – as it may seem, I have to consider these questions, as not infrequently I will see young men and women with neurological symptoms but yet I can find nothing abnormal when I examine them. This is where it is critical to try to explore any other problems they might have in life – in work, at home, in their relationship – as external stress can lead to the development of physical symptoms. It is, of course, as easy to read too much into a situation as it is to miss some of these flags of family dynamics, so it’s a careful balancing act based on listening to and observing the patient and their loved ones.
Some patients come alone, which, if they are worried about dementia, most likely means we’ll both have an uplifting consultation. After all, if they have scheduled the appointment and made their way on their own, it’s pretty unlikely to be dementia, even if they have been losing their keys or forgetting people’s names more than usual. In clinics that can be full of sad stories and dire news, reassuring people that it’s natural to forget things as they head back home is a relief to doctor and patient alike.
Having got them into the examining room, I invite people to remove their coats and hats and to sit down. Addled patients will fuss over unbuttoning their jacket and where to hang it, or hesitate over which chair (of two) to sit in. I’ve seen really stressed patients sit in my chair, which lightens the mood when they realize what has happened. The jacket removed, it is often thrust into the hands of the patient’s partner. Some partners make a point to say that they are only there as support; the ones who promise that they are under strict instructions to keep quiet are inevitably the most loquacious. They can’t help themselves, but I’m generally glad, as stressed patients can find it hard to remember anything, as if they were in the Mastermind chair, so a chatty, open partner can help to fill in the blanks.
I always explain what I plan to do: ‘I’m going to ask you lots of questions, as doctors do, but the first few are just a checklist of things to make sure I don’t miss anything basic.’
I will routinely have a few medical students with me in the clinic, and always ask before we go into the room whether the patient or their family minds the students being there to give them the option of saying they would rather they were not. Most patients do not mind (‘we all have to learn’ is their understated and generous response) and some enjoy teaching the students. Others like the attention of a group of doctors (as they perceive it) and feel they are getting the benefit of more than one medical opinion.
And some put on a performance for the students. They see it as an opportunity to indirectly criticize either the doctor personally or the health service as a whole. ‘They couldn’t tell what was going on for years and it was only after a great effort on my part did I get them to listen.’ I just try to side-step the criticism by bringing the focus back to their health today. These uncomfortable moments are a way people have of asserting their authority when they feel they have been hard done by, by doctors in the past. In fairness, it can be the case that they are right and they have had less than good care so it is best just to listen and hope that if they get a chance to let off a bit of steam that will be the end of it, allowing us to get back to the problem at hand.
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THE BIG PICTURE
Shortly after his cancer diagnosis, the journalist Christopher Hitchens wrote of his moment of deportation ‘from the country of the well across the stark frontier that marks off the land of malady’. This struck a chord with me and I realized how useful that idea could be when questioning nervous and anxious patients at their first consultation. The sense of being there at the moment that a patient’s life changes utterly is a critical starting point in trying to deduce the subsequent diagnosis.
A good narrative is the key to getting and relaying a patient’s medical history, as it is with any story. The cadence of the illness is like a musical score. Did the change occur insidiously or abruptly? Has it been progressing or is it a single hit causing static disability? Is it recurring and what factors seem to either provoke it or help alleviate the symptoms?
In meeting patients for the first time, we start with what brought them to see a doctor. It can be something like a numb hand that does not resolve itself, or a weak leg that, after a few miles on a regular walk, refuses to obey their commands to continue as normal. A well-prepared patient will have pondered when that problem began and how it has evolved from the time they originally noticed it. Many will have written down a timeline and be able to tell me who else they have seen about it before coming to me. If they hav
e already had a scan they will bring a copy of it and any other results of tests performed by their GP. If they cannot remember all of the details they might bring someone with them to help clarify the development of their symptoms.
In contrast to all that, I meet some people who tell me they have no idea or can’t remember why they have been referred (some waiting times are so long these days this is sadly far more common than you might imagine). The memory plays tricks on us all and we do not recall precisely when we developed any sort of symptom. Yet it still strikes me as a little strange when people have so much difficulty recalling the onset of their neurological symptoms. After all, they had been perfectly well at some point, and then they were not. How can they not remember when they went from one state to the other? Is it a form of denial when we get sick that we say to ourselves ‘It’s nothing’ and plough on, hoping things will just resolve themselves? Or is it so intimidating sitting in front of a doctor that your mind goes blank (as so many patients tell me is the case)? Are they fearful that by revealing all of their symptoms their darkest worries about their illness might be realized?
The moment we consider ourselves sick has particular relevance in the neurology history as initially symptoms may be very subtle. Oftentimes people will have seen a range of specialists before being referred to a neurologist. A weak leg is blamed on a bad back and an orthopaedic opinion is sought (although in Ireland this can be after appointments with a chiropractor, an osteopath, an acupuncturist and even various ‘healers’ dotted around the country). Others will have had their eyes tested thinking that it is a case of needing new glasses to remedy blurred vision, or their ears examined in the hope that having the wax removed will cure persistent unsteadiness.
Just One More Question Page 3
