Headache, Neurology in Practice 2008

8/12/2019 Headache, Neurology in Practice 2008

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THE BARE ESSENTIALS

HeadacheRichard Davenport

Correspondence to:Richard Davenport, Departmentof Clinical Neurosciences,Western General Hospital,Crewe Road, Edinburgh EH42XU, UK; [email protected]

The patient with a headache often finds himself amedical orphan. He is fortunate indeed if hisheadache is transient, for otherwise he may findhimself on an excursion to the ophthalmologist,otolaryngologist, neurologist, dentist, psychiatrist,chiropracter, and the latest health spa. He isxrayed, fitted with glasses, analysed, massaged,relieved of his turbinates and teeth and too oftenemerges with his headache intact. RC Packard 1979

Other than the most dedicated of superspecialists,

all neurologists frequently see patients with head-ache. And yet headache has been, and perhaps isstill, somewhat of a Cinderella area, with manymyths perpetuated, and newer ones evolving.Faced with a patient with headache, the relevantquestions are simple to ask, if not necessarily toanswer (box 1). An accurate diagnosis almostalways depends only on the history, and for mostpatients further investigations are supremelyredundantalthough many are investigated.

What headache patients most want is a com-prehensible explanation of their symptom, whichis perhaps the most important therapeutic step.This is a tricky task, especially when so little isunderstood about many headache syndromes, andmany neurologists are not good at explainingconcepts such as migraine or chronic daily head-ache. Far too often it is assumed that patientswould like treatment and brain scans. Perhapscopying clinic letters to patients, and providingthem with up-to-date review articles or usefulwebsites, might help with our explanations.

To answer patients questions, one needs timeand energywhich are often in short supply; thereis a temptation to reserve those resources forpatients with proper neurological disorders suchas multiple sclerosis or stroke. Invisible symptoms,

such as pain, may lead to a degree of disbelief indoctors (and others)something patients areacutely aware of. Neurologists need to remind

themselves of this when faced with the healthy-looking chronic daily headache patient; an empa-thetic approach is as relevant here as anywhereelse, if not more so.

This article is written mainly in the context ofheadache presenting in the outpatient clinic. Butheadache is also the primary symptom in 12% ofpatients presenting acutely to an emergency depart-ment. Theskillsrequired in each setting areessentiallythe same, but as the differential diagnosis is ratherdifferent (albeit with considerable overlap), headache

in the emergency department is covered in a separatesection. Although facial and neck pain are alsocommon problems, this article will deal exclusivelywith pain and similar sensations felt in the head.

EPIDEMIOLOGYAlmost everyone experiences headache at somepoint. Fortunately, most do not seek medicalattention. But some information is pertinent:

c Headache affects 95% of people in their life-time.

c Headache affects 75% of people in any one

year.c One in 10 people have migraine.

c One in 30 people have headache more oftenthan not, for 6 months or more.

c At least 90% of patients seen in a neurologyoutpatient clinic with headache will havemigraine, tension type headache, or a chronicdaily headache syndrome.

c Sinister causes of headache are rare, perhaps0.1% of all headaches in primary care.

DIFFERENTIAL DIAGNOSIS AND CLASSIFICATION

The International Classification of HeadacheDisorders (ICHD) provides a framework on whichto base classification, although it is more suited toresearch than everyday clinical practice (box 2).Headache is divided into primary syndromes(predominantly migraine, and the trigeminal auto-nomic cephalalgias), but most patients and non-specialist doctors worry about the much rarerserious secondary headache syndromes (such asbrain tumours).

The ICHD gives no indication as to the relativeimportance of what one sees in the clinic. In reallife, the important headaches are:

c Chronic daily headache (most commonly med-ication overuse)

Box 1 Questions to be answered during the consultation with a

headache patient

c Can I classify this headache, and if so how?c Do I need to investigate?

c How can I best explain the diagnosis?

c What is the patient afterreassurance, explanation, treatment, brain scan,something else?

c Is treatment appropriate and if so, what is the most sensible approach?

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features occur, including acute confusionalstates and even coma.

c Frequency: median is about 1.5 attacks permonth, but at least 1 in 10 have weekly attacks

c Triggers: identifying reliable, reproducibletriggers for migraine is over-rated, but mostpatients are keen to discuss this aspect.Hunger, sleep deprivation and stress are all

recognised, and certainly an assessment of thepatients lifestyle is warranted. The relief(come down) of stress is probably a morecommon trigger than stress itself. Hormonalinfluences are important, and may open up adifferent therapeutic approach (see below), andgetting up later than usual at weekends andother work-free days (that is, changes in bodyclock) is not always recognised by patients.

c Hormones: migraine during periods, andmigraine emerging in pregnancy or withexogenous oestrogens (most commonly the

combined oral contraceptive pill) are all wellrecognised. The difficulty is that the relation-ship is often inconsistent, with paradoxicaleffects (for example, some women lose theirmigraine during pregnancy; others only experi-ence migraine during pregnancy). Patients whothink there is a hormonal link for theirmigraine must keep a daily diary of headache

and menstruation, and are often surprised tofind there is no definite link. This is importantbecause targeting a hormonal trigger is futile ifit is not really present.

Unusual migraine subtypes

Whether it is helpful to further categorise migraineis debateable, and the very existence of some of thesubtypes below is questioned. However, the termsare still used by some:

c Basilar-type migraine (previously basilarmigraine) is used for patients with migrainetype headache accompanied by symptoms refer-

able to the vertebrobasilar circulation, includingdysarthria, vertigo, tinnitus, deafness, diplopia,ataxia, decreased level of consciousness, andbilateral sensory symptoms.

c Hemiplegic migraine: weakness which maylast longer than 60 minutes (ie, much longerthan a typical aura). It may be sporadic, orfamilial with an autosomal dominant inheri-tance. Up to 20% of the familial casesexperience episodic cerebellar ataxia, and manydevelop more typical migraine (with and with-out aura).

c Retinal migraine: rare in practice, but identi-

fied as monocular visual aura (or blindness)followed by typical migraine headache. Othercauses of transient monocular symptoms includ-ing TIA, and ophthalmic structural disorders (forexample, retinal detachment) should beexcluded. Patients find it difficult to distinguishmonocular symptoms from the more typicalbinocular visual field symptoms.

c Childhood periodic syndromes (migraineprecursors): cyclical vomiting, abdominalmigraine and benign paroxysmal vertigo aremore common in children who subsequentlydevelop migraine than in those who do not.

c Ophthalmoplegic migraine: is now reclas-

sified as a cranial neuralgia (secondary head-ache syndrome), rather than a form of migraine. Typical migraine headache is asso-ciated with an external ophthalmoplegia (mostcommonly 3rd nerve palsy), but the headacheoften lasts a week or more, with a latencybetween headache onset and ophthalmic symp-toms of up to four days.

The complications of migraine

c Medication overuse headache(although moreaccurately this is a complication of the treatment).

c

Chronic migraine: a common cause of chronicdaily headache (see below).

Key questions in the headache history

c Periodicity: establish whether the headaches are episodic (at least a fewdays free from headache in between attacks), or present most of/all the time(at some point most/every day) (box 3)

c Associated features: any neurological symptoms and/or systemic problems:migrainous such as nausea, vomiting, dislike of noise, light, smell ormovement; red flags such as persisting cognitive problems; or constitutional

such as weight loss, malaise, fevers, etc? Autonomic features (lacrimation,redness, droopy eyelid) may need a witness.

c Behaviour during headache (from patient and witness): episodic migraine

usually leads the patient to a dark, quiet room, and the need to rest, and willstop a patient working. Patients typically look pale and unwell, contrasting

with the healthy appearance of most chronic daily headache patients. Agitatedbehaviour during cluster is typical, and quite different from migraine

behaviour.c Family history: migraine is often familial, although the family diagnosis may

be overlooked or erroneous and a family history is of no diagnostic value, butmay be an interesting discussion point. Cluster is occasionally familial.Patients commonly offer a family history of brain tumour or haemorrhage:these raise concern but do not raise risk, in respect of headache diagnosis.

c Current medication: are patients taking prescription drugs that maycontribute/cause headache (oral contraceptive, dipyridamole, etc)? Are theyusing/overusing analgesia (including over the counter drugs)? Are they usingrecreational drugs? People with medication overuse headache are typicallyreluctant to detail their medication use, and often under-report their usage.

c Social situation/stressors: building up a picture of the patients social/

psychological situation is important in any consultation, but my ownexperience is that most patients are stressed because they have a headache,not the other way round.

c What the patient thinks Always ask patients what their own thoughts are; sometimes the

answers are surprising, and many patients may suspect highly unlikelydiagnoses (for example, multiple sclerosis). In such cases, it is easy toreassure them, but unless one asks, one will not know.

The inevitable brain tumour concerns emerge, sometimes with goodcause (for example, family history or recent friend with the diagnosis;previous cancer elsewhere), although more often this is a concern of thereferring doctor rather than the patient (not uncommonly it transpiresthe patient was unconcerned until such a diagnosis was raised by theirdoctor or other healthcare worker).


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c Status migrainosus: a debilitating migraineattack lasting .72 hours.

c Persistent aura without infarction: typicalaura symptoms, usually experienced previouslyas a normal migraine attack for that patient,lasting .1 week (and often months to years,but with normal brain imaging).

c Migrainous infarction: typical migraine aura

symptoms persisting .60 minutes in someonewith previous migraine with aura, with radi-ological evidence of infarction in the relevantterritory, not explained by other disease. This isnot the same as an ischaemic stroke in someonewho has (or has had in the past) migraine.

c Migraine triggered seizure: epileptic seizureoccurring during or within 60 minutes of atypical migraine aura in someone with estab-lished migraine aura. Termed migralepsy, ithighlights the similarities between migraineand epilepsy. It can be difficult to distinguishaura from focal seizures in these circumstances.

Tension-type headache

Although this diagnosis exists within the ICHD,and review articles on the subject continue to bepublished, the increasingly held view is that it ismild/moderate migraine. The headache is usuallybilateral, non-pulsatile, and mild to moderate;although patients may describe it as severe, itrarely incapacitates them. In other words, it is aless disabling form of migraine.

Trigeminal autonomic cephalalgias

These involve activation of the trigeminal and

parasympathetic systems and are characterised byshort-lasting headaches with variable autonomicfeatures, cluster headache being the most commonandmostrecognisable;the othersare rare,or very rare.

Cluster headache (previously called migrainous neuralgia)

c The patient is more often male than female,age 2050, and a smoker.

c He describes recurrent attacks of very severeunilateral pain, usually centred around theorbit, with ipsilateral autonomic featuresincluding conjunctival injection, tearing, mio-sis/ptosis, and nasal stuffiness.

c Patients are typically agitated during attacks(contrasting with standard migraine behaviour).

c The attacks peak rapidly (within minutes), andlast 30180 minutes (typically about an hour).The offset is usually quite abrupt.

c During a cluster, attacks occur at least onceevery 24 hours, and often more frequently, andusually wake patients from sleep at the sametime (alarm clock headache).

c Most cluster headache is episodic, clusterslasting weeks to months, with months to yearsremission, but 1015% have a more chronicvariant, with clusters lasting longer than a year

without remission, or with remission lastingless than a month.

Paroxysmal hemicrania

c These patients describe frequent (.5 attacks/day) attacks of unilateral pain lasting 230 minutes, with autonomic features as incluster headache, with a definite response toindometacin.

c As with cluster headache, the attacks may beepisodic (periods of remission lasting at least

1 month) or chronic, and trigeminal neuralgiahas been described in association with them.

c It is not the same as hemicrania continua (seebelow), though both are responsive to indome-tacin

Short-lasting unilateral neuralgiform headache attacks

with conjunctival injection and tearing (SUNCT)

c One of the shortest lasting headache syndromes(but with the longest name), it is very rare.

c Patients complain of frequent (up to 200/day)attacks of unilateral pain lasting 5240 sec-

onds, with ipsilateral autonomic features.

Other primary headache syndromes

While these may not be common, it is important torecognise them to avoid unnecessary investigationsand to reassure the sufferer (and their family).

c Hemicrania continua: a continuous (asopposed to paroxysmal hemicrania) unilateralheadache usually without any other features,of moderate intensity, which responds toindometacin.

c Primary stabbing headache: previously called

icepick or jabs and jolts, refers to brief(seconds) attacks of stabbing pain, usuallywithin the distribution of the ophthalmicdivision of the trigeminal nerve, but withoutautonomic features. It often accompanies otherforms of headache, especially migraine.

c Primary cough headache: typically short-lasting, triggered by straining or Valsalvamanoeuvre. It usually leads to imaging of theposterior fossa, especially to exclude Chiarimalformation but what one should do if one isidentified is far from clear.

c Primary exertional headache: headache is

very similar to migraine, and lasts minutes to48 hours.

c Primary headache associated with sexualactivity: previously called benign orgasmiccephalalgia or benign sex headache, there aretwo varieties; pre-orgasmic which sounds mostlike tension-type headache and evolves as theact progresses, and orgasmic which may mimicsubarachnoid haemorrhage, with an abruptonset at orgasm. This kind of headache seemsless common in women.

c Hypnic headache: the patients are elderly,and describe headache awakening them fromsleep, usually at the same time of the night,

and lasting typically 30 minutes, of mild tomoderate severity.


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c Primary thunderclap headache: this diagno-sis can only reliably be made in secondary care,after exclusion of subarachnoid haemorrhage,as the clinical presentation is identical.Symptoms may last from hours to days, andmay recur, but are benign.

New daily persistent headache

The symptoms are similar to tension-type head-ache, but evolve de novo, without previousepisodic headache. It responds poorly (if at all) totreatment, and is often a source of considerableconcern to patients and their doctors, yet rarelyindicates a sinister cause. Nevertheless, secondarycauses usually require exclusion:

c Raised intracranial pressure: a space-occu-pying cause is unlikely if the only symptom isheadache of several months, but anyone with asuggestive history requires brain imaging. Morelikely is idiopathic intracranial hypertension(almost only affects obese women); the mostcommon sign is papilloedema, although avariety of cranial nerve palsies have also beendescribed, secondary causes such intracranialvenous thrombosis should be excluded.

c Low cerebrospinal fluid volume (or pres-sure) headache: previously called spontaneousintracranial hypotension, the clue is in thehistory, with a story typical of post lumbarpuncture headache (ie, headache worse on sittingor standing), but without the lumbar puncture.This postural relationship becomes less clearwith time and patients may forget their initialsymptoms, so the physician must enquire care-fully about this aspect. Only about 10% ofpatients have the typical MR appearances of asagging brain (meningeal enhancement andsometimes subdural hygromas/haematomas); itis sometimes possible to identify the source ofthe cerebrospinal fluid leak.

c Chronic meningoencephalitis: infectiousand non-infectious causes of meningoencepha-litis may cause persistent headache, but theseare rare, and usually have other features as wellas headache.

c Post brain insult headache: patients whohave an index brain insult (trauma, menin-goencephalitis, subarachnoid haemorrhage, etc)may subsequently develop new daily persistent

headache. There is great uncertainty abouthow the index event leads to the subsequentheadache (if it really does), and in many casesthere is retrospective recall bias.

CHRONIC DAILY HEADACHE SYNDROMESChronic daily headache is a more useful term fordoctors than patients. It is defined as headachelasting .4 hours on .15 days per month, for.3 months, but in practice means someone whohas a headache more often than not. Patientstypically describe symptoms over many weeks,months, years and sometimes decades.

It is important to establish whether the headachearose de novo, or began as an episodic syndrome, as

this helps in classification; unfortunately, this is notalways easy to establish, especially when symptomshave been present for a long time. There are seldomany systemic or focal neurological featuresit issimply headache, although adverse effects of analge-sia are common and may confuse the picture, anddepression may also be present.

It is best divided into primary and secondary

forms; primary may be further divided into:

c transformed migraine

c chronic tension type headache

c new daily persistent headache

c hemicrania continua.

There is confusion over the use of the termschronic and transformed migraine:

c Chronic migraine requires typical migrainewithout aura occurring on more than 15 daysof the month.

c Transformed migraine has a rather looser

definition and only requires one of: priorhistory of migraine, a period of escalatingheadache frequency, and/or concurrent andsuperimposed attacks of migraine.

What is not in any doubt is the relevance ofestablishing whether there is medication overuse.

All analgesics may contribute (including triptans),and a careful history is required; like for alcohol,patients may either deliberately or unwittinglyunderestimate their intake. They may also dismissover-the-counter medications as irrelevant. Askinghow long a packet of paracetamol might last, andhow many separate stores of analgesia they keep, is

one way of getting a more accurate picture, and theanswer may surprise the patient, if not thephysician.

HEADACHE IN THE EMERGENCY DEPARTMENTThe emergency department environment is notconducive to a carefully considered history, and yetnowhere is it more vital. Patients and their familiesare often distressed and frightened, and may notappreciate that talking can be more important thanimmediate brain scans, so particular sensitivity onthe physicians part is required.

The key issue is how the headache evolved. One

which reaches its maximum intensity immediatelyor within minutes always suggests subarachnoidhaemorrhage (SAH), even though only about 1025% of such patients will prove to have this.Unfortunately, there are no accurate discrimina-tors in the history, and although other neurologicalsymptoms, neck stiffness, vomiting, seizures ortransient disturbances of consciousness all indicatean increased likelihood of SAH, their absence doesnot exclude it. Other important secondary causesof abrupt onset headache include:

c ischaemic stroke, especially due to arterialdissection

c intracerebral haemorrhage, sometimes with nofocal localising signs


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c intracranial venous thrombosis

c intermittent hydrocephalus

c meningoencephalitis.

Headaches which evolve more slowly (overhours) are less likely to be sinister, but meningoen-cephalitis must always be considered, even in theabsence of fever, rash (which must be looked for

carefully, involving full inspection of the skin),neck stiffness or photophobia.

It remains the case that most emergencyheadaches will prove to be benign (most com-monly migraine), but investigations are more oftennecessary than in the outpatient setting, and thetreatment approach is also different.

Investigations and treatment in the emergencydepartment

c All patients with abrupt onset headache need anunenhanced CT brain scan as soon as possible(ideally within an hour of presentation).

c On the other hand, patients with suspectedmeningitis without focal symptoms or signs,and no disturbance of consciousness should nothave their lumbar puncture delayed by a CTto exclude raised intracranial pressure, whichof course a CT never can.

c All patients should have baseline bloods testsearly, including blood cultures if bacterialmeningitis is suspected (and those patientsshould then immediately receive an appropriateantibiotic with corticosteroids).

c Pain reliefincluding triptans if indicatedisan obvious, but often neglected, part ofmanagement, and unwarranted concerns aboutmasking evolving symptoms or signs withanalgesia persist.

c A not uncommon mistake is to abandonsearching for a possible secondary cause if theCT is normal. If this result is surprising onthe basis of the story, review the films, andconsider other CT normal explanations (forexample, intracranial venous thrombosis,missed signs of SAH).

c If a lumbar puncture is required, ensure it isdone by someone with the appropriate skills tomeasure and record the opening pressure,request the correct investigations, and pursue

and interpret the results correctly.

RED FLAGS

During the history, symptoms suggestive of asecondary cause should become obvious, but onlyif the right questions are asked. Remember thatpatients with SAH may present late in the out-patient department, so be ready to consider this andother potentially sinister secondary causes, eventhough very few outpatients harbour such pathol-ogy. Ensure the following have been covered:

c Other neurological symptoms: unless part of

typical migraine aura or autonomic features,these should always suggest a secondary cause.

c Systemic features: weight loss, fever andother systemic features should stimulate con-cern. Patients with benign headache are notpersistently unwell.

SECONDARY HEADACHE SYNDROMES NOT TOMISS

Giant cell (temporal) arteritis:This tends to be overdiagnosed, which is under-standable given the potential consequences of miss-ing it, but the considerable adverse effects ofprolonged steroid treatment mean that strict diag-nostic rigour should be applied. It almost never occursunder the age of 55, and one should have biopsyevidence to support the diagnosis in anyone younger.It is a disease of the elderly who frequently, but notalways, have systemic features (such as malaise,polymyalgia), with a very high ESR (.75 mm in firsthour), and whose headache disappears within48 hours (often less) of adequate (6080 mg pre-dnisolone/day) steroid treatment. However, basingthe diagnosis purely on steroid response is inadvi-sable, as steroids (or simply time) can lead toimprovement in many other headache syndromes.

It is important to strive as hard as possible toconfirm the diagnosis with temporal artery biopsy,if necessary bilateral, accepting that a negativeresult does not exclude it, and only persist withsteroids if there is an unequivocal, immediate andsustained therapeutic response in both the symp-toms and the ESR.

DepressionHeadache is a not uncommon symptom of depres-

sion, although clearly there is overlap with poly-myalgia and giant cell arteritis.

Cervicogenic headacheThis is usually obvious from the history andexamination, although neck pain is common inmigraine and other primary headache syndromes,so the presence of neck pain should not auto-matically lead to this diagnosis. The pain oftenradiates frontally, behind the orbit(s). Examiningneck movements is an important part of theclinical assessment, and finding a stiff and painfulneck with reduction of movement opens up an

obvious (if not evidence-based) therapeutic avenue(neck manipulation/massage), and identifying occi-pital neuralgia is useful, as this suggests a specifictherapy (local anaesthesia of the occipital nerve).

INVESTIGATIONSBy definition, only patients who are suspected ofhaving a secondary cause of headache requireinvestigation, which underlines the essentialrequirement of an accurate clinical diagnosis.

Blood testsOther than an ESR or CRP in older patients with

new headache, blood tests are rarely helpful,although any suggestion of a systemic component


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to the history should stimulate at least basichaematological and biochemical analyses (as wellas a proper physical examination of more than justthe nervous system).

Brain imaging

Only a tiny proportion of patients with non-acuteheadache have a structural explanation. On theother hand, up to 10% of asymptomatic people mayhave an incidental abnormality of some sort on MRbrain imaging, which can raise very difficult treat-ment dilemmas (for example, unruptured aneurysm,meningioma). Thus brain imaging in non-acuteheadache patients should be approached withconsiderable caution. Unfortunately, brain imagingis becoming ever more available, and patients (andsome doctors) have misplaced faith in doing ascan. Furthermore, organising a scan is oftenquicker and more attractive to the harassed doctorthan carefully explaining what the diagnosis is, andwhy brain imaging is not appropriate. The end result

is that far too many patients with headache areimaged unnecessarilysome with damaging con-sequences if a worrying incidentaloma is revealed.

Therefore, avoid imaging unless there is a verypersuasive reason, and do not image patients with arobust diagnosis of a primary headache syndrome.Situations where imaging might be required are:

c headache syndromes where a structural expla-nation is plausible

c localising neurological symptoms or signs notexplained by migrainous aura

c constitutional or systemic symptoms or signs

c recent diagnosis/treatment of cancer, especiallywhere cerebral metastasis plausible

c unclassifiable headaches.

Sufficient time with patients will allow them todescribe their story fully, and for neurologists toprovide an understandable explanation of thediagnosis and why tests will not helpful. Warnpatients that the risk of an incidental finding on MRimaging is up to 1 in 10. MR imaging carries no riskof radiation (or indeed any known harm), thedisadvantage being in its very sensitivity,increasing the risk of identifying non-relevantabnormalities, so one might argue that CT is a moreappropriate choice. However, patients are increas-ingly aware that MR is more detailed than CT, andthus may still request MR even after a normal CT.

Other tests

These depend entirely on the clinical scenario, andmay include examination of the CSF, more specia-lised imaging such as CT/MR venography, etc.

TREATMENT

Only the treatment of primary headache syn-dromes will be discussed; of these, only migraine

and trigeminal autonomic cephalalgias are effec-tively helped by drugs. Always assess whether any

treatment at all is requiredan explanation andreassurance may be sufficient.

Migraine

c The few patients with avoidable triggers willmostly have recognised them long before theysee a neurologist, but cyclical/menstrualmigraine in women deserves special mention.

c Providing simple housekeeping tips, such asnot skipping meals, adequate sleep and so on isappropriate, and providing more information(most easily by referring the patient to apatient support organisation) is often appre-ciated.

c Discuss alternative treatments such as acu-puncture because it is remarkable how manypatients have already tried these, which theywere disinclined to mention until raised by thedoctor; tell them there is no robust evidence tosupport or refute most alternative treatments.

c Explain that drug treatment is one avenue.

c Before adding drugs, look at the patients currentprescription, and consider whether drug with-drawal is appropriate (particularly women withmigraine who are taking the combined oralcontraceptive pill where withdrawal for at least6 months is a potential treatment).

c Explain how treatments should be used, parti-cularly symptomatic versus preventative treat-ment, so often confused by patients (box 4).

c Symptomatic treatment is only effective forthe headache/nausea elements; there is nosymptomatic treatment for aura.

c The decision whether to use the oral or

parenteral route is important, but often notconsidered; early nausea and vomiting arelikely to reduce the absorption of oral medica-tion, and the parenteral route might be better.

c A stepped approach, using simple analgesiafirst, is appropriate, as this is highly effectivefor many patients.

Preventative treatments

The decision to use preventative therapy as well assymptomatic drugs is for the patient, depending onthe frequency and severity of attacks, their con-sequences for their life, as well as the relative

efficacy of the chosen symptomatic therapy. Anumber of drugs are available, and the choice ismainly governed by potential and actually experi-enced adverse effects (box 5).

Other treatments for migraine

c Acupunture: the only alternative treatmentfor migraine for which there is any evidence, itshould be considered as a non-drug option,although limited availability.

c Psychological intervention: no scientific evidenceto support its use, but a pain managementapproach may be helpful in patients with severe,

drug resistant migraine provided medicationoveruse headache is kept in mind.


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c Botulinum toxin: not effective in at least onerandomised trial, not recommended.

c Closure of patent foramen ovale (migrainewith aura): only published randomised trial todate did not achieve primary endpoint, notrecommended on current evidence.

Cyclical/menstrual migraine

c Should be diagnosed only after recording at

least 3 months menstrual and migraine diary.c Supplemental oestrogen in the premenstrual

phase, and tricycling the oral contraceptivepill have been recommended.

c The symptomatic use of mefenamic acid,aspirin/paracetomol/caffeine, or triptans haveClass A evidence to support them.

c Frovatriptan and naratriptan are effective aspreventative treatment when started 2 daysbefore menses, and continued for a few days,although are not licensed for such use; clearly apatient must be able to accurately predict herperiod to use this strategy effectively.

c Hormone replacement therapy to combatmenopausal symptoms is safe to use inmigraine patients, but there is no evidence

that it is effective in treating migraine, and cansometimes exacerbate it.

Treatment of trigeminal autonomic cephalalgias

c The only class A evidence for the treatment ofcluster headache is sumatriptan 6 mg subcuta-neous.

c High flow 100% oxygen (requiring a specifichigh flow oxygen regulator and face mask) orintranasal lidocaine 10% drops may be con-sidered for those patients who do not respondto sumatriptan.

c

The published evidence to support preventativetherapies is poor. One (non-evidence based)approach is to start prednisolone (60 mg/day)and verapamil (up to 240 mg/day, sometimeshigher) or methysergide (up to 12 mg/day)together at the beginning of a cluster, tailingthe steroids after 23 weeks, but continuingverapamil/methysergide until the cluster hasresolved. ECG monitoring is recommendedwhen using verapamil, especially in high doses.

c A positive response to indometacin (up to225 mg/day) in paroxysmal hemicrania is partof the diagnostic definition. Start at 25 mgthree times daily for first week, then 50 mg

three times daily for second week, and 75 mgthree times daily in third week, but continue

Box 4 Symptomatic treatments for migraine

Oral route

Parenteral route

Aspirin 900 mg plus anti-emetic (eg, domperidone 1030 mg) Supported by Class A evidence*

Ibuprofen 400 mg/ketoprofen 100 mg plus anti-emetic Supported by Class A evidence*

Other non-steroidal anti-inflammatory drugs licensed for migraine in UK: diclofenac, tolfenamic acid,flurbiprofen, naproxen

Triptans (eg, almotriptan 12.5 mg, r izatriptan 10 mg, eletr iptan 4080 mg) Supported by Class A evidence*;must be taken at onset of headache, notof prodrome/aura;

patients who do not respond to one triptan should be offered at leastone other

Ergotamine Less effective, more adverse effects

Opiates Ideally should be avoided

*Scottish Intercollegiate Guidelines Network, Class A: at least one meta-analysis, systematic review or high quality randomised trial.

Diclofenac 100 mg suppositories +/2 domperidone 30 mg suppositories or buccal prochlorperazine36 mg

Sumatriptan ( 1020 mg) o r zolmitr ip tan (5 mg) nasal spr ay Many f ind sub cutaneous easier to toler ate than nasal. Class A evidence

Sumatriptan 6 mg subcutaneous

Haloperidol 5 mg iv Effective, but adverse effects, and rarely used in UK outside hospital.Metoclopromide 10 mg iv Effective at relieving pain and nausea/vomiting (Class A evidence)

Box 5 migraine preventative treatment

Drug Comments

Prop ranolol 40240 mg/day Class A evidence

Beta-blockers should be avoided in asthmaOther beta-blockers (eg, metoprolol,atenolol, timolol, nadolol)

Probably as effective as propranolol

Sodium valproate (8002000 mg/day) Class A evidence

Teratogenicity and weight gain limit usefulness in women

Topirama te (100 m g/da y) Cla ss A e vidence

Gabapentin (up to 1800 mg/day) Evidence less robust than for valproate/topiramate

Anti-depressant drugs (start amitryptyline10 mg or dothiepin 25 mg and titrate upas necessary)

Tricyclics/venlafaxine appear effective, but SSRIs are notuseful

Pizotifen (1.53 mg/day) Appears less effective in adults than children, adverseeffects limit usefulness

Flunarizine (510 mg/day) As effective as propranolol, not l icensed in UK

Methysergide (up to 12 mg/day) Effective, but rare serious fibrotic adverse effects: shouldnot be used for longer than 6 months without at least1 month break


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only if positive response. If successful, continuefor at least a few weeks before titrating down.

c Lamotrigine has been suggested as an effectivetreatment for SUNCT.

Treatment of other primary headache syndromesIndometacin should be considered as a treatment,often prophylactically, for many of the otherheadache syndromes (see dosage schedule above).No response should lead to rapid abandonment ofthis strategy.

Treatment of chronic daily headache syndromesThis is not a rewarding area for drug treatment,and indeed withdrawing drugs is probably moreimportant. Patients who are using any analgesiathree times or more each week are likely to havemedication overuse headache, and withdrawal isthe treatment. This strategy will never work unlessthe theory is adequately explained to a patient, andeven then frequently fails.

c Explain why complete withdrawal fromanalgesia is essential.

c Tell them they are likely to get worse beforethey get better, and that many weeks may passbefore they improve.

c Perhaps use low dose tricyclics to cover theperiod of withdrawal (amitryptyline 10 mg ordothiepin 25 mg), and occasionally topiramate

or propranolol if convinced they have chronic/transformed migraine.

c For patients with long standing, intractablechronic headache, a pain management pro-gramme may help.

What to do if treatment is not working

c Reconsider the diagnosis carefully. Have youmissed medication overuse headache?

c Is the patient taking the treatment? If not,why not (commonly because he or she does notunderstand the rationale for treatment, oradverse effects)?

c Is medication overuse complicating the picture?

c Is the treatment being taken correctly (forexample, triptans commonly fail because theyare taken at onset of aura/prodrome ratherthan onset of headache, or is preventativetreatment being taken in a symptomaticmanner)?

c Recognise that many headache syndromes donot respond well to medication (less commonin migraine, very common in chronic dailyheadache syndromes).

c Consider non-drug approaches.

CONCLUSIONS

c Most headaches are due to a primary headachesyndrome.

c Most patients who reach a neurologist haveeither migraine or a chronic daily headachesyndrome.

c Patients want an adequate hearing of theirsymptoms, followed by a diagnosis, and under-standable explanation.

c Most patients need reassurance, some willbenefit from treatment, and few requireinvestigations.

c Some headache syndromes are amenable tomedical treatment (e.g. migraine), but othersare much less so (chronic daily headache), andan honest explanation is usually appreciated.

c Above all, patients want someone who isinterested in their headache and who willlisten to their story.

Acknowledgements: This article was reviewed by Giles Elrington,London, UK.

Competing interests: Within the last 5 years, I have receivedhonoraria for speaking or chairing meetings from various companiesinvolved with headache treatments, including Merck Sharp & Dohme,Menarini, Janssen-Cilag, and Pfizer.

USEFUL WEBSITES

c British Association for the Study of Headache (BASH): For healthcare

professionals with interest in headache www.bash.org.ukc International Headache Society (download ICHD-2): www.i-h-s.orgc Migraine Action Association: www.migraine.org.ukc Migraine Trust: www.migrainetrust.org

c Organisation for the Understanding of Cluster Headache (OUCH UK): www.ouchuk.org/html/

FURTHER READING

c Davenport RJ. Sudden Headache in the Emergency Department. Pract Neuro2005;5:13243.

c EFNS guideline on the drug treatment of migraine report of an EFNS taskforce. Eur J Neurol2006;13:560572.

c Elrington G. Migraine: diagnosis and management J Neurol NeurosurgPsychiatry2002;72:1015.

c Frearson R, Cassidy T, Newton J. Polymyalgia rheumatica and temporalarteritis: evidence and guidelines for diagnosis and management in olderpeople. Age Ageing 2003;32:3704.

c Goadsby PJ. Recent advances in the diagnosis and management of migraine.BMJ2006;332:259.

c Lipton RB, Bigal ME, Steiner TJ, et al. Classification of primary headaches

Neurology2004;63:42735.

c MacGregor EA. Oestrogen and attacks of migraine with and without aura.Lancet Neurol 2004;3:35461

c Matharu MS, Goadsby PJ. Trigeminal autonomic cephalgias J NeurolNeurosurg Psychiatry2002;72:1926.

c Packard RC. What Does the Headache Patient Want?Headache1979;19:3704.c Schoenen J, Sandor PS. Headache with focal neurological signs or symptoms:

a complicated differential diagnosis. Lancet Neurol2004;3:23745.

c Stovnera LJ, Zwarta J, Hagena K,et al. Epidemiology of headache in Europe.

European Journal of Neurology2006;13:33345.

c Sudlow S. US guidelines on neuroimaging in patients with non-acuteheadache: a commentaryJ Neurol Neurosurg Psychiatry2002;72:1618.


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