SYMPTOMS AND SIGNS

Taking a neurological history

M D OBrien

Case history 1
A 52-year-old, right-handed man complained of several blackouts. The first occurred 8 years ago and the last 3 months ago. He usually has about one every 6 months, but 3 or 4 years ago he had five or six in 1 year. The longest period free of attacks was 9 months, about 3 years ago. The last two attacks were 3 months ago and 5 months ago. The attacks have approximately similar patterns and can occur at any time of the day. He has had them both sitting and standing, but never while lying down or in bed. They are not related to a change of position and he is unaware of any immediate precipitating factors. The attack starts with a feeling of nausea and warmth, which he recognizes as the start of an event. He feels as if he is going to pass out, and does so within a few seconds of the onset of the aura. He is not aware of any change in his heart rate or breathing, nor is he aware of any change in vision or hearing. He is probably unconscious for about 1 minute, and always falls. He regains consciousness lying on the floor feeling weak and nauseated, but never vomits. When he feels a little better, he lies down and usually sleeps for about 2 hours. His wife, who has not accompanied him to the consultation, has reported that he is very pale and that no movements are observed, though he has been incontinent on two occasions. There is no other relevant history. His father died in his 50s following a stroke. Comment There was no abnormality on examination, except slightly elevated blood pressure (150/95 mm Hg). It can be difficult to distinguish between cardiac syncope and epilepsy. The history favoured epilepsy. ECG, 24-hour ECG, echocardiography and tilt-table testing were all normal. EEG showed a minor temporal lobe disturbance and subsequent sleep EEG showed an unequivocal left temporal lobe abnormality, but MRI showed no parenchymal lesions except a few small, subcortical white matter lesions, thought to be indicative of vascular disease. A diagnosis of epilepsy was made and, in view of the number of attacks that had occurred over the years, treatment with anti-epileptic medication was started.

The history is the most important and most productive part of neurological assessment, the purpose of which is to answer the following questions. Where is the lesion? What is it? Could all of this patients problems result from a single lesion at one location, or must there be more than one lesion? Is this a system disease? The history provides the most information about the nature of the lesion, gives a strong indication of its location and helps to determine the choice of appropriate physical signs. The precise location is usually more accurately defined by clinical examination. It has been shown that, in general medicine, the correct diagnosis can be achieved from the history alone in more than 80% of patients; the examination, which is traditionally given pride of place in neurological assessment, contributes only about 7%, and full investigation about 7%. Time spent taking the history is therefore likely to be rewarding, and failure to take an adequate history is a common reason for inappropriate investigations and incorrect diagnoses. If time is limited, it is better to be selective with the examination than with the history; this can be achieved by using the history to select the appropriate examination. The narrative history is most important it is unproductive to spend a disproportionate amount of time on a long list of complaints and a detailed systems review, as is currently taught in many medical schools. If a proper history has been taken, little of relevance will emerge from the systems review, though it may sometimes reveal information that could not have been anticipated. The history must be taken because it is seldom given in a useful form by the patient. It is often said that, if allowed to speak freely, patients usually give the diagnosis, but the time required is a luxury that few clinicians can afford. However, patients should be given the opportunity to add their comments at the end of the history.

Taking the history

Start the history by asking patients age and handedness. If they are right-handed, it is reasonable to assume that they have a dominant left hemisphere. If they say that they are left-handed, it is worth asking with which foot they would kick a ball, and whether they have any first-degree relatives who are also left-handed. At this stage, it is worth determining the main problem (headache, back pain, paraesthesiae in the hands). It is not particularly profitable to make a long list of all of the symptoms, unless the patient is polysymptomatic with several complaints that do not obviously cohere. In this situation, make a list to determine whether the problems indicate more than one condition and ask the patient to identify the most important symptom; the choice may surprise you. It is easy to get side-tracked by recent developments; the history may be much longer than this, and it is better to start at the beginning, not at a more recent, perhaps acute event.

M D OBrien is Emeritus Physician for Nervous Diseases at Guys and St Thomas Hospital, London, UK. He qualified from Guys Hospital Medical School, and trained in neurology at the National Hospital for Neurology and Neurosurgery, London, the Regional Neurological Centre, Newcastle upon Tyne, and Guys Hospital.

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Many neurological conditions (epilepsy, transient ischaemic attacks (TIAs), migraine and other headaches, migrainous neuralgia, trigeminal neuralgia) cause a series of discrete events over time. In other conditions (carpal tunnel syndrome, multiple sclerosis), symptoms may be intermittent or fluctuate. The history of repeated events requires information additional to that of a single event, but the principles are the same. There are three aspects to the history of discrete events the distribution of events over time, the circumstances in which they occur, and the details of a typical event. It is better to obtain information about the pattern of events over time first, once it is established that all events are more or less the same. Obtain details about the frequency and distribution of attacks over time separately from details of the individual events. Patients often confuse these two aspects of the history; they may wish to describe the most recent event, which may have been the worst, and the reason for the consultation, though this event may be atypical of average attacks. Different types of event require separate histories. Pattern of events over time (Figure 1) To establish the pattern of events over time, it is important to determine when the first attack occurred by asking the following questions. When in your life did you first have one of these episodes? You never had anything like this before that first event? Asking the same question twice in different ways may elicit further information. If there have been only a few attacks (fewer than three or four), a detailed account of each is needed. If there have been many events, it is important to determine the date of the first; this may need to be only an approximate year, but the date of the last event should be as precise as possible because this may be relevant with regard to driving. It is then necessary to obtain information about the approximate frequency and whether events occur in clusters (migrainous neuralgia, trigeminal neuralgia). What is the maximum number of attacks you have had in a given time (days, months, years)?

Case history 2
A 67-year-old woman suffered a single episode of loss of consciousness on a Saturday 3 months ago. This was a unique event. She had walked about mile to a local shop at 11:00 a.m. and was standing in the shop when she seemed to have difficulty focusing. She realized that something was not right and called out for help, but immediately lost consciousness. She recovered consciousness in the casualty department of the local hospital about 1 hour later. She had bitten her tongue on the right side, but had not been incontinent. The shopkeeper subsequently told her that all four of her limbs were shaking and she was foaming at the corner of her mouth. She recovered fully by that evening and was discharged. Comment There was no abnormality on examination except her blood pressure (170/110 mm Hg). The history was clearly that of a single generalized tonic-clonic seizure; at the patients age, this was likely to have a vascular basis, but could be caused by a space-occupying lesion. EEG was normal, but MRI showed extensive abnormal signal in the white matter adjacent to the right trigone and some focal areas of abnormal signal in the subcortical white matter appearances consistent with vascular disease. Other than her blood pressure, no vascular risk factors were identified. After discussion, it was agreed that this single event did not justify anti-epileptic medication. The patient preferred to accept the risk of further attacks.

Pattern of events over time

Worst event

Maximum frequency of events

First event Longest symptom-free interval

Last event

What is the longest interval free of attacks and when did that occur? When were these attacks at their worst? Have you had any bad patches, with frequent or more severe attacks? How many of these events have you had in the recent past (1 month, 3 months, 6 months, 1 year)? When was the last event? Are you completely normal between attacks? If the history is very long, sometimes many years with epilepsy or migraine, the patient may not readily recall the frequency of early attacks. It is sometimes helpful to cue patients with major life events (onset of menstruation, school/college, leaving school, getting married, having children, changing jobs) and ask whether they had any time off during these events. This baseline information can be valuable in the future when trying to determine the effects of treatment. Circumstances in which the attacks occur, and aggravating and precipitating factors Before proceeding to a detailed account of an average event, a few attacks or a single event, it is useful to try to gain information about the circumstances in which these events might occur. Have they occurred while you are standing, sitting or lying down in bed, or in all of these positions? Are they related to a change of position, or exercise? Do they occur at any particular time of day or night, indoors or outdoors?

Severity

Least event

2000

2001

2002

2003

2004

The timescale may be days, weeks, months or years 1

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Case history 3
A 62-year-old, right-handed man suffered three events over 4 months. The first two were 1 week apart and the third 10 days ago. They all occurred in the late afternoon while he was sitting reading. They were not associated with a change of position. He was unable to identify any clear precipitating factor. He suddenly realized that he could not understand what he was reading, and though he thought he could see the print clearly, he found that he had to use his index finger to track the line of print. This problem was maximal at onset and lasted about 25 minutes before resolving rapidly. He smoked 1015 cigarettes per day and was receiving treatment for high blood pressure. Comment The clinical diagnosis was transient ischaemic attacks in the dominant posterior parietal region. Examination showed slightly elevated blood pressure and a left carotid bruit. Subsequent investigation showed an 80% stenosis of the left carotid artery, which led to carotid endarterectomy. The patients account of these events clearly indicates dyslexia, almost certainly with a right visual field attention defect.

Shape of the history

Maximum deficit Residual deficit

Onset

Start of recovery

Now

Time to maximum deficit

Time at maximum deficit

Time of improvement

Stable

Do you know of anything that may trigger, precipitate or aggravate one of these episodes? Are you conscious of your heart beat or breathing? In women: Are they related to your periods? The event (Figure 2) It may be helpful to think of the history as having a shape comprising an onset followed by evolution to its worst, a period at maximum deficit, and then continuing progression or recovery to the present state. A detailed account of an individual event should be of an average attack if it is one of many; an estimate of the range from the most mild to the most severe can be obtained subsequently. It is important to determine the shape of the history by identifying: time of onset time to maximum deficit (rate of evolution) length of time at maximum deficit (which is seldom the duration of an attack) symptoms at their worst time of improvement (rate of recovery) length of time with stable deficit present symptoms, if any. Precise details of the onset of the disorder are particularly important, but most patients need cues if they are to give an accurate account. The circumstances in which the attack started can be determined by casting the patient mentally at the scene of the event. Although it is seldom relevant, asking patients the day of the week, the time, where they were and what they were doing often generates useful information. Patients commonly say that they were doing nothing, but this can never be the case; for example, it may be relevant that a patient with photosensitive epilepsy was watching television at the time. Brief auras may be missed because

of the patients desire to tell the clinician about subsequent, more dramatic events. What is the first thing that tells you something is about to happen? Can you recognize this as the start of an attack? Is there enough time for you to sit down? Patients often have difficulty with the timescale of attacks, which is of particular importance in determining the nature of the event and usually requires a cue (seconds, minutes, hours, days, weeks or months). After obtaining a detailed, step-by-step account of the way in which the event evolves or has progressed, it is often necessary and usually helpful to obtain an account from a witness, particularly if the events are associated with loss of consciousness. The author asks any relatives or friends who have accompanied the patient to join the consultation, and arranges interviews with witnesses or speaks to them by telephone. It should be remembered that the only objectively verifiable part of the history is the current state of the patient, which may be entirely normal at the time of the consultation. A sequential history with a clear account of the precise onset, rate of evolution and resolution usually indicates the nature of the underlying pathology. For example, hemiplegia could result from a TIA, tumour, stroke, migrainous aura, hypoglycaemia, Todds paralysis or multiple sclerosis; a good history should distinguish these conditions. A focal motor seizure might take seconds to develop, a migrainous aura evolves over a few minutes, a completed stroke starts abruptly, remains unchanged for a time and then improves over weeks or months, and a tumour starts insidiously and evolves steadily. Discrete similar events These can vary in three independent ways: frequency duration severity.

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Case history 4
The patient was a 19-year-old man who was left-handed but right-footed and had a left-handed brother. His attacks started at the age of 14 years, and he left school at 16 years. He probably had four or five attacks during these 2 years. He had three attacks since then; the last was 2 weeks ago. Only two of all these events occurred during the day the one when he was 14 years old, and the most recent. The last event started at 10:30 a.m. when he was standing at work. He was aware of forced deviation of the eyes to the right and felt dizzy. He sat down on the floor, became aware of his head jerking to the right, and then lost consciousness. He was told that all four of his limbs started to jerk and he bit his tongue on both sides, but he was not incontinent. The movements lasted for about 10 seconds. When he recovered, he had a headache and felt tired. He sat down for half an hour, drank a cup of tea and was then able to return to work. In the attacks that occur while he is asleep, he is woken by forced deviation of the eyes to the right; he tries to call out, but is unable to do so. As in the daytime attacks, he is aware of his head jerking and then loses consciousness. He is not aware of generalized movements, but these have been reported by his girlfriend. Comment Neurological examination was normal and there was nothing to suggest a left frontal lesion. The clinical diagnosis was epilepsy, with the strong suggestion of a left frontal focus. This was confirmed on EEG, but MRI showed no underlying structural abnormality. In view of the frequency of attacks, treatment with anti-epileptic medication was started.

In patients with a neurological condition, it is mandatory to ask about sphincter disturbance, which is a common problem in spinal cord disease. This question should be expressed in terms that the patient understands. Sexual orientation and sexual function Many neurological conditions affect sexual function (erectile problems in spinal cord disease, adductor spasm in women, mobility problems in conditions such as Parkinsons disease). Patients may not mention these difficulties, but often welcome the opportunity to do so. Sexual orientation may affect the choice of investigations. Sleep Do you normally sleep well? If the answer is no, the patient should be asked the following questions. Do you have difficulty going to sleep or staying asleep, or do you wake early? Do you snore? Persistent heavy snoring is a marker of sleep apnoea. Depressed patients often wake early, and many neurological conditions disturb sleep. A poor sleep pattern for any reason impairs patients ability to cope with chronic disease. Travel Have you been abroad recently? If so, where?

Case history 5
A 59-year-old, right-handed woman complained of headaches. She was never prone to headaches in the past, but tended to have them during her periods, which stopped 3 years ago. The headaches have become more of a problem since then. She has experienced months free of headaches; the longest duration was probably 8 months. The headaches are always worse in cold weather, which she thinks is a major aggravating factor. The last episode was 2 days ago. She is asymptomatic between headaches. The headaches always start during the day with a tight feeling in both temples; if this is the major symptom, it is quickly eased by Anadin. Sometimes, the headaches are associated with a band-like sensation around the occiput; Anadin then has little effect. The patient also suffers occasional sharp pains in the upper cervical spine, radiating to the vertex. The headaches are not associated with nausea or visual phenomena, though she has eye ache and is both photophobic and phonophobic. On further questioning, it transpires that the recent headaches were related to problems at work, where there had been a major reorganization that she found difficult to manage. Comment There was no neurological deficit on examination. On the basis of the history, she was thought to be suffering both migraine and tension headaches. The sharp pain in the back of her head radiating to the vertex was thought to arise in the upper cervical spine. A tricyclic drug was recommended for prophylaxis.

These three aspects are often modified independently by treatment; for example, the patient may suffer fewer attacks of the same duration but greater severity. It is important to establish whether there have been recent changes in each parameter consultations often occur because there has been a change.

Systems review
Every experienced physician develops a personal list of discrete questions. Each question should be phrased to suggest normality, so that the patient has to contradict this assumption. This is less likely to produce spurious information. Appetite and weight What is your appetite like? Do you normally eat well? Is your weight more or less constant? Patients may say that they have a very poor appetite but their weight is constant or increasing, which can imply an affective disorder. Weight loss with a good or poor appetite may be more significant. Sphincter disturbance Do you have any problem with your bowels or passing water?

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Case history 6
A 44-year-old man first suffered an episode of left facial pain 18 months ago, and this continued for about 1 month. He was woken by it on three or four nights per week, usually between 3:00 and 4:00 a.m.; it never occurred during the day. He was then pain-free until about 2 months ago, when the problem recurred. This time, it was more severe and more frequent, occurring on five or six nights per week, but the duration and time were the same. He knows of no aggravating or precipitating factors and is unaware of any effect from alcohol. The attacks have a similar pattern. He is woken by pain in the left eye, which he describes as like a severe toothache. It evolves to its maximum in about 10 minutes, remains severe for about 20 minutes and then fades away, the whole episode lasting about 40 minutes. There are no associated visual symptoms. He is not aware of any change in his facial appearance or in his eye, which does not water. Comment The examination was normal. A diagnosis of trigeminal neuralgia had been made elsewhere, but the patient had failed to respond to carbamazepine. The history clearly indicated a diagnosis of migrainous neuralgia (cluster headache).

Case history 7
A woman, now 37 years old, experienced an episode of pins and needles in both feet at the age of 25 years; it lasted for about 6 weeks and then resolved. There were no other symptoms and no sequelae. She developed paraesthesiae in her right leg 5 years later; she described this as like water running down the leg, and after about 1 week she felt tingling in her right hand. All of these symptoms resolved after about 2 months. She developed tingling and increased sensitivity on the anterior lateral aspect of her left thigh 2 months ago; this has largely resolved, leaving a patch of slightly altered sensation. Comment: The only deficit was impairment of pinprick appreciation on the anterior lateral aspect of the left thigh above the knee, corresponding to the distribution of the lateral cutaneous nerve of the thigh. The history was strongly suggestive of multiple sclerosis, and this diagnosis was confirmed by MRI. The recent history was clearly a consequence of meralgia paraesthetica and not related to multiple sclerosis, which appears to have been quiescent for 5 years.

Allergies Are you allergic to anything? Have you ever had a problem with a drug or medicine? Have you ever had asthma? This question is important because the information obtained may preclude use of -blockers, non-steroidal anti-inflammatory drugs or aspirin, all of which are widely prescribed in neurological practice. Drugs Are you taking any medicines, pills or drugs? A full list should be made of all drugs being taken, their doses, the timing of the medication and the duration of the treatment. It is unwise to prescribe medication if this list is in any way incomplete. Many patients do not include homeopathic or herbal remedies unless asked, and many women forget to mention the contraceptive pill. Many patients with a long history of uncontrolled epilepsy or migraine state that they have tried all available drugs without success, but a proper trial of a drug requires an adequate dose taken for a sufficient length of time. For example, an effect is unlikely to occur in epilepsy with phenytoin, 100 mg daily for 6 months, because the dose is too small, or in migraine with amitriptyline, 100 mg daily for 1 week, because the duration of treatment is too short. Menses Are your periods normal and regular? Are you on the pill? Have you ever taken the pill? If so, when? Were there any problems? In younger women: When did your periods start? In older women: When did your periods stop?

Tobacco, alcohol and substance abuse Do you smoke? If the answer is yes, ask: How many? and: What is the maximum number that you have ever smoked in a day? If the answer is no, ask: Have you ever smoked? If the answer to this is yes, ask: When did you stop? If the patient smokes, try to ascertain how many cigarettes over how many years to obtain an estimate of pack-years. It is useful to obtain an estimate of the number of units of alcohol drunk each week, and of the maximum number of units drunk in 1 week or in a given time in binge-drinkers. Many patients are reluctant to admit to smoking more than 20 cigarettes per day or drinking more than 4 units per day. Honesty can be encouraged by making a suggestion that is well in excess of the likely consumption. Evasive patients, when asked: Less than one bottle of whisky a day? might say: Yes, about that, or might strongly deny that it is anything like as much because they drink only half a bottle each day. Similarly, the suggestion that a patient smokes fifty or sixty cigarettes each day may be denied vigorously if he or she smokes only forty. Have you ever used recreational drugs? Patients seldom volunteer this information, which may be relevant in certain circumstances. Previous diseases What accidents, operations and illnesses have you had? A complete list of conditions, with dates, is necessary. Patients may require cues; they often say that they have had no serious accidents, operations or illnesses, but produce a long list when asked whether they have ever been in hospital or consulted a doctor. The question may need to be repeated three times, because the patient may hear and answer only the first point about accidents.

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Family history Is there anybody else in your family with anything like this? The family history is often relevant in neurological disease, and may give additional information about the domestic situation that will not otherwise emerge. The author asks in detail about first-degree relatives (parents, siblings and children) and, if any of them have died, about the cause of death. If a familial condition is suspected, a detailed family tree should be constructed and the possibility of consanguinity raised. Are your parents alive? How old is your father/mother? Is he/she well? Do you have any brothers or sisters? Ask about their ages and health. Do you have any children? Ask about their gender, age and health. Personal history Details of patients social, business and domestic background is often relevant in determining the management of many conditions. It is necessary to know their occupation, how they get to work, who lives at home with them, what degree of support is available (psychological and physical) and their housing conditions. Sometimes, these questions reveal unexpected information; for example, the recent arrival of a demented mother-in-law, the stress of caring for a disabled child, redundancy of the patient or the patients partner, social isolation following the death of the partner, stress related to illness in the partner, or the fact that the patient is a single mother without support, which would make admission to hospital difficult. This is a good opportunity to ask patients how their condition has affected their life. How much time have you missed from work because of this problem (in the last month, 6 months, year)? Does this problem ever prevent you from going out or limit your activities? Some patients describe severe blinding headaches, but are nevertheless capable of going out in the evening or continuing work. This information gives some independent measure of the severity. Patients with an increasing neurological deficit (e.g. those with multiple sclerosis or Parkinsons disease) often undertake fewer activities as their horizons shrink; this may occur as a result of increasing disability, or because of frequency and urgency of micturition. These matters should all be addressed, because effective management of such problems may improve the patients quality of life more than any attempt to treat the underlying condition.

Neurological eye problems

Masud Husain Christopher Kennard

Many neurological disorders present with visual dysfunction (e.g. loss of vision, diplopia). The history and examination (Figures 1 and 2) help to reduce the number of possible diagnoses.

Ocular vascular disease

Central retinal artery occlusion leads to sudden, usually painless monocular blindness. When the occlusion is temporary, the patient may complain of transient monocular visual loss (amaurosis fugax, Figure 3), with blindness described as coming down like a curtain. Permanent occlusion of the central retinal artery leads to retinal infarction and blindness. When only a branch retinal artery is occluded, an altitudinal field defect may occur, with visual loss in either the upper or the lower half of the visual field. Fundoscopy may demonstrate embolic material within blood vessels, retinal oedema and, later, a cherry-red spot where the vascular choroid at the macula stands out against a pale, infarcted retina. Insufficient blood flow in the central retinal artery (temporary or permanent) may result from thromboembolism, vasculitis, spasm or reduced perfusion (e.g. critical carotid stenosis). Examination and investigations are similar to those in transient ischaemic attacks or stroke, and the principles of treatment and prophylaxis are also the same. However, it is important to exclude raised intraocular pressure (e.g. glaucoma) as an underlying cause. Anterior ischaemic optic neuropathy results from infarction of the optic nerve head caused by occlusion of the posterior ciliary arteries. Patients typically complain of sudden, painless monocular visual loss that is usually altitudinal. The disc is swollen and commonly pale; there may be haemorrhages and cotton-wool spots. This condition is most commonly non-arteritic caused by atherosclerosis, or arteritic usually associated with temporal arteritis and raised ESR. Prompt administration of high-dose corticosteroids is important to prevent visual loss in the other eye when temporal arteritis is suspected (see page 11).

Concluding the history

Before proceeding with the examination, ask the following question. Is there anything else you would like to tell me? Failure to take a good history is a common cause of failure to reach the correct diagnosis. Failure to explain to the patient what you think and how you intend to proceed is the most common cause of complaint, so conclude the consultation with: Is there anything else you would like to ask or discuss?

Masud Husain is Reader in Neurology and Wellcome Trust Senior Fellow at Imperial College London and the West London Neurosciences Centre, Charing Cross Hospital, London, UK. He qualified from the University of Oxford. His research interest is disorders of visual perception in stroke and neurodegenerative conditions. Christopher Kennard d is Professor of Clinical Neurology and Deputy Principal of the Faculty of Medicine at Imperial College London and Honorary Consultant Neurologist at Charing Cross Hospital, London, UK. His principal research interest is neuro-ophthalmology, particularly disorders of eye movement in neurological disease.

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