NON-HEADACHE PRESENTATIONS OF MIGRAINE
DR NICHOLAS C SILVER
5TH JULY 2011
HOUSE OF COMMONS – COMBINED MEETING OF ALL PARTY PARLIAMENTARY GROUP ON PRIMARY HEADACHE DISORDERS (APPGPHD) AND ALL PARTY PARLIAMENTARY GROUP ON PAIN (APPGP)
In this talk to the combined All Party Parliamentary Groups on Primary Headache Disorders and Pain, I have outlined two main concepts related to migraine, namely
- That migraine is a significant but often relatively invisible cause of disability, and
- That migraine is primarily a disorder of the brain but is not just about headache – rather, it is a multi-system and multi-symptomatic disorder.
Migraine is known to be disabling. It is estimated that approximately 4% of adults consult their general practitioner annually because of headache. The World Health Organisation recognises migraine as one of the three most prevalent disorders in the world, affecting an estimated 324 million persons worldwide. The World Health Organisation have attempted to assess the disability caused by various conditions and they rank migraine as one of the four most disabling conditions known to man.
The hallmark of incapacity associated with this condition is reflected in that migraine is ranked equally alongside other conditions where persons cannot function, such as dementia, psychosis and quadriplegia. In the UK itself, it is estimated that 25 million school or working days are lost each year through migraine headache. In this talk however, I will discuss what essentially "goes below the radar" given that misdiagnosis rates for migraine headache may be as high as 60% to 80% and given that migraine may present with symptoms other than headache.
Migraine may be defined as a primary headache disorder characterised by central sensitisation and various combinations of neurological, systemic and autonomic features. In other words, migraine is a disorder of the brain but its symptoms may be throughout the body and its manifestations are primarily related to
- Amplification of normal bodily sensations (eg normal sensations or absence of awareness of sensation may become tingling, pain or throbbing, noise, light and smell may be amplified and become uncomfortable and balance input may be amplified asymmetrically so that dizziness arises because of vestibular imbalance).
- utonomic disturbance (involvement of the nerves that regulate automatic body functions, leading to symptoms such as red, runny or drooping eyes, facial swelling, blocked or runny nose, nausea, eructation (burping), diarrhoea or flushing, etc), and
- Brain dysfunction - this may manifest in the sense of aura symptoms in one in five persons whereby abnormal visual disturbances, dysfunction of speech or altered sensation occur; mood may change, leading to irritability, depression or even elevation of mood; there may be a prominent component of fatigue; memory may be subjectively poor and lead to confusion; in rare instances, patients may even describe symptoms of Alice In Wonderland Syndrome where they may note a distortion of reality and experience a feeling of body parts not belonging to them or being the wrong size, they may feel the wrong size compared with the environment or they may even hallucinate.
To understand chronic migraine, it is necessary to first look at what is known to occur in acute episodic attacks. Episodic migraine may occur in four separate phases but not every person has each phase and some persons will notice different attacks with different phases present. The four phases in order tend to be a prodromal phase, an aura phase, a headache phase and a postdromal phase. The prodrome may occur for some hours or even days and lead to symptoms such as depressed mood, irritability, feeling lightheaded or dissociated, yawning, food craving, neck ache, fatigue, passing a lot of urine or diarrhoea, etc. Many of these symptoms may also occur later on in the attack during the headache phase. As described above, aura symptoms may occur in up to one in five patients and may even occur without headache. During the headache phase, there are headache symptoms, including throbbing, sensitivity to movement where movement and exercise exacerbates the headache disturbance, patients may or may not be more sensitive to surrounding stimuli, including noise, light and/or smell and they may have other symptoms such as nausea and/or vomiting. Non-headache symptoms may also occur during the headache phase, including fatigue, confusion, loss of appetite, vertigo, etc. In the postdromal phase, patients may still have some residual tenderness of the scalp and they may feel somewhat fatigued and washed out.
We recognise that migraine pain is commonest in the head, face and neck, and indeed the pain may appear to start in the neck leading to a misperception that there is a neck disorder rather than a primary problem with the brain accounting for the cause of the attack. It has long been recognised, particularly in children, that pain may present in the abdomen during migraine and we term this abdominal migraine, where there may be associated abdominal symptoms, including nausea, vomiting or even diarrhoea. There are clear accounts of patients presenting with migraine where there are associated pains in the limbs, chest or back and occasionally patients may present with isolated pain in these areas without noticing much, if any, headache. In addition, many patients in acute episodes of
migraine may experience restless leg symptoms (when staying still they will have a discomfort and a feeling of need to move their limbs, most often affecting the legs but occasionally affecting the arms or trunk).
With this in mind, one can understand how chronic migraine might give rise to a number of symptoms in association with, or separate to, headache.
Chronic migraine with or without overuse of acute attack medications (eg painkillers and/or triptans) accounts for the vast majority of patients seen with headache by physicians or neurologists, either as inpatients or in their outpatient clinics. Generally speaking, approximately 90% to 95% of cases seen in specialised headache clinics relate to chronic migraine with or without medication overuse.
When patients evolve or transform from episodic to chronic migraine, we typically see a jumbling up of the headache and non-headache symptoms recognised in the various phases of episodic migraine.
As patients escalate from episodic to chronic migraine, they often mention that their attacks have become more frequent, the background has filled in with milder, less featureful headache, pressure or muzziness, and they typically reflect that some days may remain severe. However, some patients complain of little or no headache but have other prominent symptoms. With the escalation to chronic migraine, it is quite typical to see loss of efficacy of acute attack medications so that painkillers and triptans that previously worked now stop working. In my view, as soon as we see painkillers and other acute attack medications stop working, this usually implies that the patient is in chronic migraine and also usually we will note that the patient is over-using their acute attack medications, which in turn will perpetuate their condition. Preventative drugs that are taken on a regular daily basis may also lose their previous useful effect if medication overuse is present. As a patient escalates from episodic to chronic migraine, they will generally develop many other non-headache symptoms and feel generally very unwell.
The main cause of escalation from episodic to chronic migraine is generally recognised to be medication overuse (which may be witting or unwitting) and/or caffeine overuse. All known painkilling drugs, including Paracetamol, opiates, non-steroidal anti-inflammatories and triptans may lead to medication overuse headache. Caffeine, which may also work as an acute painkiller, may lead to such escalation from episodic to chronic migraine and caffeine is recognised in many substances, including tea (including green tea), coffee, cola, chocolate and a number of other fizzy drinks, energy drinks and alcopops. The International Headache Society reflects on medication overuse as "the interaction between a therapeutic agent and a susceptible patient" and recognises importantly that medication overuse is "usually motivated by a patient's desire to continue to function".
The concept of chronic migraine and caffeine overuse has long been recognised. In a household textbook for non-medical persons from the 1920's (Virtue's Household Physician, edited by a number of prominent neurologists of the day, including William Gowers, Frances Dercum, Charles Dana, etc), they comment on a phenomenon of "tea and coffee headaches" and suggest that "in the nervous and often the gouty and rheumatic person, the use of tea and coffee will cause violent headaches. These luxuries of life should be discontinued for at least one month. An extra strong cup of black coffee, to be sure, will stop the headache for the time being, but only adds fuel to the fire in the long run. We would strongly advise anyone that has constant or periodical headaches, if he uses either tea or coffee, and especially coffee, to leave them off entirely for three months. It may be the sole cause, and if caused by tea and coffee, there is no possibility of their cure by medicines while you continue their use". It is interesting that this record notes that persons with tea and coffee headaches will often appear nervous and often will describe a number of other pains (as suggested by the term "gouty and rheumatic") and it is not unusual to see persons with chronic migraine achieve diagnoses of stress-related or psychological disorder, anxiety, depression, or fibromyalgia when attending general practices and/or other medical or rheumatological clinics.
It is important to recognise chronic migraine, particularly where associated with medication and/or caffeine overuse, as once diagnosed, this condition is very treatable. It is thought that approximately 40% of patients will respond to lifestyle measures alone and these typically include eliminating all painkillers and other acute attack medications, eliminating all caffeine from the diet, ensuring very good hydration during the day, regular meals without missing food, and ensuring a regular time to go to bed and get up each day. As headache specialists, we normally advise patients that when they abruptly cut out painkillers and caffeine, they will typically experience significant worsening of their condition over a week to a few weeks period. If patients are over-using combined analgesics with codeine, they may experience more prolonged worsening over a few weeks but if coming off simple painkillers, triptans or caffeine, the period of worsening is usually less than a week. After that time, their condition may fluctuate and become more intermittent and then gradually settle over a further few weeks. As such, at least 40% of patients may manage to treat their condition with lifestyle measures alone that they need to keep in place thereafter. For the other 60% of patients who have not achieved significant benefit, the laying down of such a foundation of lifestyle will provide a very good background upon which we can build. In my practice, I would normally attend to any ongoing sleep disorders if still present after full detoxification and institution of lifestyle (particularly restless legs syndrome, as this is quite common in migraineurs – many patients however will find restless leg symptoms disappear when they cut out all painkillers and caffeine and they will then have improved quality sleep). If their sleep is of good quality and they wake refreshed in the morning and have a good lifestyle in place, then it will be reasonable to start a preventative drug. The most common reasons for preventative drugs failing are either that patients continue to use significant amounts of acute attack medication and/or caffeine, that they have not taken a high enough dose of the preventative medication or have not taken it for long enough (it may take up to four months to start working after reaching a useful maximum tolerated dose). Occasionally, preventative medications may be less successful when used at a dose too high for that patient, e.g. where it has caused significant side effects such as fatigue (which may limit its benefit), poor memory, or low mood. In that situation, the drug should be reduced until such side effects settle completely and then the effects should be monitored over the next three to four months to see if the drug becomes successful. Where a preventative is successful, it is often worth continuing the drug for six to twelve months and then slowly tailing off, keeping good lifestyle in place.
In addition to the above measures, we have some newer treatments for migraine which may be quite effective and these may include treatments such as botulinum toxin. This has recently been evaluated in good quality randomised controlled trials and found to have superiority over placebo. Patients may note up to two-thirds reduction in their days of migraine within six months of starting three monthly cycles of botulinum toxin injections into the scalp. For patients refractory to the above measures, we occasionally use greater occipital nerve blocks (injections of steroid and local anaesthetic around a nerve under the scalp at the back of the head) to provide temporary reduction in headache, but these are only thought to be helpful in about half of the patients with migraine and the benefits may last anywhere between days and months. Newer surgical techniques are being explored such as greater occipital nerve stimulation, where a wire is placed under the skin at the back of the head and this is attached to an implantable pacemaker device. We await further studies in this area to find out how effective such measures will be in those with chronic migraine refractory to other known treatments.
Coming on to non-headache presentations and manifestations of migraine, there is a constellation of symptoms that we typically see in the majority of patients who present with regular migraine headaches. In addition to the severe migraine headache days, the milder background headaches with milder migrainous features (milder sensitivities to noise, light, smell or movement, etc) and/or the occasional sudden stabbing headaches, we may see prominent pains in other distributions. It is quite common to see facial pain, particularly above, behind and below the eye, in the cheek, behind the bridge of the nose or down into the jaw or teeth. A "coathanger" distribution of neck pain with stiffness, tenderness and tightness may occur and there may be other focal points of muscle pain and/or tenderness in the trunk or limbs. Intermittent fluctuating sensory disturbance is common with symptoms such as pins and needles in hands, feet or face, or odd sensations like insects or water over the scalp. These are often quite fleeting, lasting seconds to minutes, but occasionally they may be more persistent. We may see up to four different types of imbalance or dizziness. Very commonly with chronic migraine, patients will describe what I term "migraine-related disequilibrium" symptoms where they start to feel lightheaded and spaced out. With this, they may feel somewhat detached or dissociated and as they start to feel these symptoms, they will typically note the pressure building in their head. Later in the phase of symptoms, they may start to feel anxious and may even develop other symptoms that are often misattributed to anxiety such as a lump in the throat (globus sensation) or a difficulty breathing in. Occasionally they will feel themselves start to shut down, their vision will go black, dark or spotty, their hearing may go quiet or buzzy and they may even faint (i.e. resulting in "migraine syncope"). Some patients during their acute attacks of severe migraine will notice true vertigo where they feel that either they or the room are moving. It is not unusual for chronic migraineurs to experience visual vertigo whereby visual stimuli such as patterned carpets, stripes, escalators, words on newspapers, etc, result in them feeling imbalanced. Patients may complain of veering to one side when walking. Symptoms of restless legs syndrome and/or periodic limb movements are relatively common in patients with chronic migraine and these symptoms will often be noted only at rest, will be quickly and fully relieved by movement and they will typically be diurnal in nature, most likely to be present towards the end of the day and evening. There may be periodic limb movements where involuntary twitching whilst awake or asleep occurs on a regular basis. The importance of this condition relates to the severe fragmentation of sleep patterns and architecture and as a result, poor quality sleep may potentially exacerbate and potentiate the overall migraine disturbance. It is very common for patients to experience fatigue which may fluctuate day to day. They may describe low mood, irritability and feeling more emotional and they may complain of short-term memory disturbances with difficulties with concentration, coming out with the wrong words when talking, getting stuck halfway through sentences and difficulties finding words or recalling recent calendar events. It is important to recognise that when patients have the occasional "brilliantly crystal clear" complete headache-free day, the above non-headache presentations and manifestations of chronic migraine will typically fully disappear as well. As such, if the above non-headache symptoms have been attributed to other disorders such as fibromyalgia, chronic fatigue syndrome, postnatal depression, cervical spondylosis, meniere's disease, panic disorder, etc, yet they fully disappear on headache-free days, this would suggest that the alternative diagnoses of these symptoms may well be incorrect and that they may actually be suffering chronic migraine disturbance to fully account for these manifestations.
The association of sleep disorders with chronic migraine is increasingly recognised, particularly the association with restless legs syndrome and periodic limb movements. As a clinical neurologist, I have recognised an association between these disorders and treated them on a regular basis for six or seven years but it is only recently that the literature has established a clear link between these conditions. It is of interest as dopamine in the brain is increasingly recognised to be related both to the cause of migraine and to the cause of restless legs. It is also of interest that for a long time, it has been recognised that restless legs syndrome may be exacerbated and perpetuated by caffeine use. In my experience, when patients detox from caffeine and painkillers, they often note their restless legs initially worsen and then a significant number of patients will find that the restless leg symptoms disappear, only to come back when they subsequently go back into an individual attack of episodic migraine. It is, in my opinion, very important to recognise where restless legs and periodic limb movements are present as these symptoms may be made much worse by certain drugs that are conventionally used to treat migraine, including tricyclic antidepressants and SSRI antidepressants. Both of these types of antidepressant medication are often said to be useful in patients who have comorbid pain/headache and difficulty with sleep because they may induce sleep but the difficulty with these drugs is that they tend to induce a poor quality sleep such that the actual benefit from sleep may be actually reduced by taking these medications. In other words, the patient may feel they have slept well but will wake unrefreshed and appear more fatigued as a result of disrupted sleep patterns and architecture.
It is interesting to recognise associations between chronic migraine and fibromyalgia and to reflect that the hallmark feature of both conditions relates to an amplification of sensations mediated by the mechanisms of central sensitisation within the brain. More than a third of patients with primary headache syndromes are known to have fibromyalgia symptoms and those patients who have fibromyalgia typically have worse migraine and poor sleep. There have been studies linking the intensity of diffuse pain and fatigue with headache in fibromyalgia.
Many patients who present to headache clinics have been separately diagnosed as suffering from chronic fatigue syndrome. However, chronic migraine in its own right is recognised as a significant cause of fatigue and more than 80% of patients will have significant abnormal levels of fatigue on measurement of a Fatigue Severity Scale and 67% of patients will meet recognised criteria for diagnosis of chronic fatigue syndrome. However, the diagnosis of chronic fatigue syndrome or myalgic encephalomyelitis (ME) relies on the initial exclusion of any other known underlying cause and it is my opinion that ME or chronic fatigue syndrome should not be diagnosed without first excluding the presence of chronic migraine and/or sleep disorder.
It is not unusual to see a patient who presents with chronic migraine who brings a long list of symptoms. It is often regarded that patients with long lists of symptoms have anxiety disorders, stress-related disorders or functional illness where their medical symptoms can be explained on a psychological basis. However, with increasing knowledge, we recognise a number of conditions that have been described as hysterical being reclaimed by physical diagnoses. Indeed, when one looks in the old family reference medical book that I mentioned previously from the 1920's (Virtue's Household Physician), they comment on hysteria that "bright sparks are seen before the eyes ... at one time the person will feel as large as a barrel, at other times not larger than a whip stop, the head will feel light or heavy, large or small. The smell becomes perverted; the hypochondriac will smell odours where there are none ... the persons are subject to fainting turns ... they are irritable, fretful, peevish and fickle". One could argue that many of these phenomena noted could suggest migraine and/or Alice In Wonderland Syndrome associated with that condition.
I myself have issues about using stress as a diagnosis. Patients with chronic migraine often acquire the diagnosis of stress-related psychological or psychiatric disorder and stress is all too easily blamed for symptoms that we see in chronic migraine, yet we know that stress may be a trigger rather than a cause. Indeed, it may actually be the relief of stress that triggers a worsening of migraine. If one is to diagnose a psychological disorder (eg a hysteria or conversion disorder), then the physician needs good medical knowledge, experience and clinical skills to exclude pathology, as well as good psychiatric skills to ensure the presence of positive markers of a psychiatric disorder. It is all too easy in practice to reach a psychological diagnosis without implementing such skills. The wrong diagnosis in this particular area will often strain doctor-patient relationships, may cause stigma and may deny patients available appropriate therapy.
So which medical practitioners will see patients with chronic migraine yet fail to recognise that this is the cause of the presentation. In my experience, there are a number of groups of clinicians and healthcare practitioners who will potentially fall into this trap. Examples of this include general practitioners that may diagnose stress, anxiety or depression, dentists may diagnose atypical facial pain, teeth grinding, temperomandibular joint (TMJ) dysfunction or even consider tooth extraction, orthopaedic specialists and physiotherapists will often treat the neck and assume cervical spondylosis1 (wear and tear in the neck vertebrae and discs) and this may lead to potentially unnecessary operations on minor disc bulges unrelated to the presentation. Ear, nose and throat (ENT) specialists will quite commonly see patients with dizziness or vertigo and increasingly recognise migraine as a prominent cause of vertigo in their clinics. However, some ENT specialists may still fail to recognise migraine causing facial pain, diagnose sinusitis and then operate on scan findings of sinus change2 that are so common in the general population and potentially unrelated to the presentation. Neurologists and cardiologists will sometimes fail to recognise migraine as a cause of blackouts and instead consider cardiac syncope or non-epileptic attacks. Immunologists and endocrinologists may not recognise chronic migraine masquerading as a chronic fatigue syndrome and gastroenterologists may fail to recognise migraine as a cause of abdominal pain or a potential cause of irritable bowel type symptoms. Obstetricians may see prominent vomiting in early pregnancy and diagnose hyperemesis gravidarum rather than recognising migraine as a potential cause. In other patients, they may attempt to treat premenstrual syndrome symptoms which actually may be part of a premonitory phase of migraine worsening. Psychiatrists may fail to recognise migraine as a potential cause of treatment-resistant depression, anxiety, panic attacks, premenstrual dysphoric disorder (isolated very low mood related to menstruation) or conversion disorder (a psychological disorder resulting in apparent physical symptoms and/or signs). Pain Clinics may not recognise the importance of migraine when seeing patients with chronic pain syndromes, neck and back pain, fibromyalgia or facial pain (particularly with regards to the need to eliminate medication overuse before considering preventative drugs). Paediatricians may see some rarer instances of migraine such as where the condition presents with cyclical vomiting or alternating paralysis down either side of the body.
Incorrect diagnosis may lead to unnecessary suffering, over-investigation, unnecessary operations, stigmatisation, waste of resources, increased casualty and hospital admissions, increased outpatient consultations with "patients doing the rounds", breakdown of GP-patient relationships and an overall waste of significant resource and increased NHS expenditure.
In the National Health Service, it is all too easy to see how difficult management for complex or multi-systemic conditions has become. With far fewer neurologists in the UK than other European countries, and with increasing specialisation, GPs and hospital doctors struggle to cope with neurological disorders, including chronic migraine. Patients may also complain that they do not see the same general practitioner for follow up each time and this may lead to fragmentation of care.
In hospital, it may be no better as doctors increasingly work shifts and the patients generally move between different wards and pass to new medical teams to improve bed use efficiency. The doctors who first see a patient on admission do not necessarily follow the patient through their management and may fail to learn by not seeing through the diagnosis, treatment and outcome of that patient. As patients pass between different teams, it is all too easy to replace good clinical assessments with scans and tests and diagnosis is not always achieved by the time of discharge, given that discharge planning may assume greater importance. Most importantly, it is difficult for one individual doctor to assume overall responsibility for ensuring that the patient embarks on the right path of treatment.
So instead of seeing patients with a list of symptoms as a "heart sink" patient, I would urge doctors and other health care practitioners to spend more time with that patient and try and find a unifying diagnosis. To quote one of the oldest texts in the world "Ask and it will be given to you, seek and you will find; knock and the door will be opened to you".
1Most people in early adult life and middle age will have some features of cervical spondylosis on x-ray or MRI scan of the neck, making it all too easy to blame the neck for many pain problems.
2Changes associated with sinusitis are extremely common on CT and MRI scans, including in the normal population. Sinus-related pain should really only occur over one sinus, should not swap sides or occur bilaterally, and should be associated with symptoms of a bloody or mucopurulent discharge from the nose on that side. When pain occurs above and below the eyes, particularly where it can occur on both sides, migraine is by far the commonest cause.
Dr Nicholas Silver is a consultant neurologist and honorary clinical lecturer at the Walton Centre for Neurosciences, Liverpool. He trained in London at the National Hospital for Neurology and Institute of Neurology, Queen Square and at Barts and the Royal London NHS Trust. He obtained a PhD after completing four years research into investigating magnetic resonance imaging techniques in inflammatory and toxic neurological conditions and part of his research also focussed on psychiatric and headache disorders. He has particular interests in headache and unexplained neurological symptoms and he runs a clinic for refractory and difficult to manage headache disorders at the Walton Centre.