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Just a headache? Disentangling Headache and Facial Pain Dr Nicholas Silver Consultant Neurologist The Walton Centre for Neurology and Neurosurgery Liverpool.

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Presentation on theme: "Just a headache? Disentangling Headache and Facial Pain Dr Nicholas Silver Consultant Neurologist The Walton Centre for Neurology and Neurosurgery Liverpool."— Presentation transcript:

1 Just a headache? Disentangling Headache and Facial Pain Dr Nicholas Silver Consultant Neurologist The Walton Centre for Neurology and Neurosurgery Liverpool July 2008

2 Outline Background Red Flags and secondary headaches The main primary headache disorders Rarer and unusual primary headache disorders Migraine and its many disguises

3 Learning Aims Necessity of detailed and directed history Red flags Distinguish main primary headache disorders Recognise migraine as by far the commonest presentation to hospital with headache Recognise non-headache manifestations of migraine Treatment of migraine and other headache disorders For copy of slides: e-mail Anne.McCann@thewaltoncentre.nhs.uk

4 Background

5 A Needle in a Haystack Primary Headaches are Very Common A common symptom: –10% of patients see GPs each year with headaches –45% of general neurology consults to Walton Centre Lifetime prevalence of headache: –M: 93% (8% migraine, 69% tension type) –F : 99% (25% migraine, 88% tension type) Population-based studies: –4-5% of population fulfil criteria for 1 0 chronic daily headache –0.5% have severe daily headaches

6 The importance of headache Headache as an indicator of disease Invisible and disabling –World Health Organisation Most common presenting neurological symptom Socioeconomic cost Easy to treat Often poorly managed

7 Presentation to hospital with headache Primary headaches outnumber secondary headaches Tension type headache hardly ever seen –A featureless headache that by definition is never severe Chronic Migraine (+/- medication overuse) is extremely common –> 95% of my DGH ward and clinic referrals –Often poor history leads to wrong diagnosis of acute migraine –Acute migraine treatments will exacerbate –Associated symptoms poorly recognised –Often overinvestigated or referred to wrong specialists Trigeminal Autonomic Cephalgias are rare –Cluster headache is overdiagnosed

8 Headache or facial pain V nerve ? Migraine commonest cause of facial pain Misdiagnosis rate high –Sinusitis –Eye disorder –Tooth disorder –TMJ

9 Part 1 Secondary Headaches For most secondary headaches, the features of the headache itself are poorly described in the scientific literature Even for those where it is well described, there are usually few diagnostically important features The International Classification of Headache Disorders 2nd Edition, 2004

10 Secondary Headaches Diagnosed –Not by the type of headache, but 1.By the additional features 2.By the neurological symptoms and signs 3.By looking at the whole patient Importance of systemic and endocrine enquiry Importance of general medical examination 4.By vigilance, experience, methodical approach, sixth sense and luck!

11 Red Flags Raised ICP: –Headaches: night > day –Often waking with headaches + vomiting –Relief on getting up –Clear postural headache –HA increased by ValsalvaCough Bending forward –Visual Obscurations –Pulsatile Tinnitus Low ICP –Fully disappears if flat (<30 mins) –May have sudden onset (e.g. speed bump; fairground; LP)

12 Red Flags Change in Headache Pattern New onset headache after 50yrs Focal neurological signs Acute confusion Papilloedema / absent SVP Sudden onset New Daily Persistent Headache (onset over 1-3 days, usually clearly recall day it started) Other illness - cancer / HIV Systemic symptoms –fever –nuchal rigidity –weight loss, etc. Features of GCA –Jaw claudication –Localised temporal tenderness –Myalgia / stiffness –Unilateral visual loss –Reduced appetite –Consider in all older patients

13 Some serious secondary headaches to consider in young people Meningitis Cerebral abscess Encephalitis Brain tumour Subarachnoid haemorrhage Cerebral venous thrombosis –eg on OCP, pregnant, sinus or middle ear disease Arnold Chiari malformation Pituitary adenoma

14 Scans for headache TO SCAN –Only 1 in 1,000 with headache >3/12 + no red flags have abnormal scan OR NOT: –MRI is too sensitive: approx 3-4% abnormal MRI scan (e.g. aneurysm, AVM) –White matter hyperintensities are very common (what do they mean?) –Risk of scans and need for pre-scan counselling radiation & cancer risk for CT contrast allergy implications for future insurance policies Note: –Plain CT is not sensitive enough to exclude metastases –Scans are not a substitute for good history and examination

15 Part 2 Primary Headaches

16 IHS Classification ICHD-II Part 1: The primary headaches –1. Migraine –2. Tension-type headache –3. Cluster headache and other trigeminal autonomic cephalgias cephalgias –4. Other primary headaches Part 2: The secondary headaches Part 3: Cranial neuralgias, facial pain and other headaches

17 Tension type headache Is it really the commonest headache disorder? Ask the audience!

18 Tension-type headache Never severe Featureless –No sensitivity to noise, light, smell –No nausea –No throbbing –No worse with exertion / movement

19 Case History 1 24 year male New headaches – always L side Last 30-40 minutes Wake patient at 1am – severe V restless - pacing around room, bangs head against wall, rocks Occasional in day – start with blocked L nostril, may get red or runny eye May occur within 30 minutes of alcohol SH – smoker, MDMA, cocaine

20 Diagnosis?

21 But….. Occasional identical attacks in day; notes preceded by 20 – 30 minutes of evolving cerescentic scotoma with zig-zags On enquiry, reports: –Nausea –Photophobia –phonophobia

22 Cluster Headache Male>>femaleRestless++Severe++++ Autonomic features usual Attacks < 4 hrs ETOH: immediate trigger Sidelocked in individual cluster Aura – 20% Photophobia, phonophobia GTN challenge (3 sprays): provokes within 90 mins in all Migraine Female > male Need to stay still Severe++ Autonomic features +/- Attacks > 4 hours ETOH: delayed trigger undeserved hangovers May vary side Aura – 20% Photophobia, phonophobia GTN challenge (3 sprays): may induce headache within hours

23 Investigations?Treatment?

24 Investigation of cluster headache MRI brain + pituitary ?MR angiography Pituitary function blood tests

25 Case 4 Management of Cluster Headache Acute attacks: –100% Oxygen Air Products –S/C Sumatriptan 6mg up to 2 doses per day –Nasal Zomig 5mg up to 3 doses per day Preventatives: –Prednisolone 60mg, reducing each 3-5 days by 10mg –Verapamil (start 120mg bd, increase up to 960mg / day) – very effective; must have ECG prior to each dose increase to ensure normal ECG axis, PR interval, and QRS complexes –[Methysergide, topiramate, Epilim]

26 Case History 2 37 year man Recalls exact onset of headache whilst sitting at desk 18/12 ago Persistent fluctuating bilateral vertex / occipital headache No headache-free days since onset Boring and Pressure - Moderate to severe intensity Featureless: –No exacerbation with exertion, climbing stairs –No nausea, vomiting –No photophobia, phonophobia, osmophobia –No autonomic features –No change with posture Mild relief with cocodamol

27 Diagnosis?Investigations?Treatment?

28 New Daily Persistent Headache (tension-type phenotype) Onset over < 3/7; Often recall initial day of onset of headache Migraine or tension type phenotype Primary or secondary Investigate all to exclude primary cause: –MRI brain + Gd, MR pituitary / MRA –FBC, ESR+CRP, Prolactin, TFT (+/- IGF1), Ca, B12, folate, biochemistry, autoantibody screen Prognosis: variable – can be difficult to treat Rx: as for chronic migraine – eliminate caffeine and medication overuse first

29 Learning points New Daily Persistent Headache requires investigation Tension-type headache (TTH) is overdiagnosed +++++ TTH is –Rare –Never severe –Featureless –Not exacerbated by movement –Rx by simple analgesia Chronic TTH is Rx by stopping acute attack medication and starting amitriptyline

30 Case History - 3 54 year male Evolving constant but fluctuating daily moderate unilateral (side-locked) head pain for 9/12 Boring ache above and around R ear Phonophobia, photophobia Conjunctival injection and nasal congestion Jabs and jolts of pain in parietal area No response to –Acute attack medications: codeine, paracetamol, tramadol, ibuprofen, sumatriptan –Preventatives: pizotifen, propranalol, gabapentin, epilim or amitriptyline

31 Diagnosis? Investigation of choice?

32 Hemicrania Continua A rare, indometacin responsive headache disorder characterised by continuous moderately severe headache that varies in intensity, waxing and waning without disappearing completely Usually unilateral Rarely alternates sides or bilateral Jabs and joltscommon Exacerbations of pain often associated with autonomic disturbance Exacerbations of pain most commonly associated with restlessness Often migrainous features: photophobia, phonophobia, nausea May remit and relapse or be continuous Diagnosis established by complete indometacin response Rare secondary cases described Rx: –Indometacin 25-250mg daily (withdrawal each 4-6 months as may remit) –Other NSAIDs - ibuprofen, celecoxib, and naproxen, –Tricyclic antidepressants –Topiramate

33 Learning points Strictly unilateral pains should always make one consider a trigeminal autonomic cephalgia Always consider indometacin-responsive headache syndromes where no treatment has any benefit Hemicrania Continua is diagnosed by a complete response to indometacin Other indometacin-responsive primary headache syndromes include: –Paroxysmal Hemicrania –Primary Cough Headache –Benign Sex Headache

34 Case History - 4 24 year lady Extremely severe pains for 8/12 Last 5-20 seconds Occurs up to 150 times / day Triggered by eating, brushing hair, touching face, talking, washing and cold wind Above eye in V1 Side-locked No response to carbamazepine, amitriptyline or gabapentin Suicidal; worst pain in life++++++

35 Diagnosis? Any other questions to ask?

36 Autonomic disturbance with pain –Red eye –Tearing –Swelling of eyelid No refractory period –Continued stimulus continues to provoke severe pain

37 SUNCT SUNCT = Short-lived Unilateral Neuralgiform headache with conjunctival injection and tearing SUNCT = Short-lived Unilateral Neuralgiform headache with conjunctival injection and tearing Newly recognised condition Short pains, most often in V 1 Pains may last longer than trigeminal neuralgia Trigeminal neuralgia is rare in V 1 SUNCT has no refractory period Autonomic features are prominent Responds to lamotrigine Poor if any response to carbamazepine / gabapentin)

38 Chronic Migraine (+ Medication Overuse) 3 types of headache usually seen: 1.Severe migraine attacks 2.Background fills in with milder migrainous headaches (often misdiagnosed as TTH) 3.Primary stabbing headaches Migraine-associated symptoms Often associated medication and/or caffeine overuse, leading to failed trials of preventative medication

39 Learning points Take a thorough history Beware typical conditions with atypical treatment response Any facial pain may cause autonomic disturbance Autonomic disturbance is not only seen with cluster headache

40 Differential diagnosis of primary headache FrequencyDurationLateralitySeverity Migrainousfeatures (aura, photophobia, nausea etc) Autonomic Behaviour / characteristic features Tension- type headache Daily to monthly hrs - months Bilateral Never severe NEVER TTH is featureless NEVER Never limits activity Migraine Daily to monthly > 4 hours to days Unilateral or bilateral +/- severe Yes+ Stay flat and still Hemicrania Continua* ContinuousContinuousSidelocked +/- severe +/-++- Cluster headache* Clusters or chronic: 1-4 / day < 4hours ~ 15–40 mins Sidelocked Usually severe +++ +/-+++ Restless agitation, pacing, holding head Paroxysmal Hemicrania* 10 – 40 / day ~ 10–20 mins Sidelocked Usually severe +++ +/-++- SUNCT* * MRI / MRA recommended 60 – 400 / day ~ < 2 minutes Sidelocked Severe +++ +/-++ V 1 distribution / No refractory period

41 Treatment of primary headache Acute Attacks Preventative Strategies Tension-type headache ParacetamolNSAID Stop all painkillers / caffeine AmitriptylineMirtazepine Migraine [Aspirin 900mg or paracetamol 1G] + domperidone Triptan – generic oral 50mg or 100mg sumatriptan is cheapest Stop all painkillers / caffeine Tricyclics, propranalol, epilim, topiramate Hemicrania Continua N/AIndometacin Cluster headache Pure 100% O2, 12-14l/min, sealed rebreathing mask s/c imigran 6mg prn bd Nasal zomig 5mg prn tds Verapamil (off licence) – slow increase to 960mg / day with ECG monitoring Epilim, topiramate, GON Block Paroxysmal Hemicrania N/AIndometacin SUNCTN/ALamotrigine Intravenous Lidocaine

42 Part 3 Rarer and unusual primary headache disorders

43 Primary Cough Headache Brief headache induced by: –Cough, sneeze, etc –May be severe Diagnosed after MRI brain scan to exclude cause of posterior fossa crowding Rx: Therapeutic LP (50%), Indometacin

44

45 Benign Sex Headache Sudden headache At climax or before Severe Investigate for SAH – CT / CSF (xanthochromia) Rx – reassure, different positions, propranalol, indometacin

46 Benign Exertional Headache Similar to benign sex headache (BSH) Often occurs in those who also have BSH Occurs with weight-lifting etc –NB migraine with exercise = far commoner Rx as for BSH

47

48

49 Case History 5 42 year female Facial pain Bouts lasting 3-4 days; increasingly frequent Right cheek / upper teeth / jaw Rarely involves left face Throbbing / exploding pain Feels unwell Mild rhinorrhoea Mild puffiness around left face – intermittent swelling and numbness Nausea, tired, neck ache and looks depressed

50 Diagnosis – ideas?? What to do next??

51 More history Attacks increasing in frequency Gaps filling in with milder pain May radiate behind eyes and to temples Photophobia, phonophobia Alcohol-intolerant: increased pain next day In past: –Travel sickness, abdominal pains in childhood, sinusitis episodes often in teens

52 Diagnosis?

53 Important questions Painkillers used Caffeine consumption Sleep disturbance –Restless legs? –Periodic limb movements? –OSA?

54

55 Part 4 Migraine and its many disguises

56 Case 1 What is M.E.?

57 21 year lady Referred for management of intermittent headache Studying GCSEs Longstanding symptoms of ME (diagnosed age 13) –Severe disability with fatigue & very poor exercise tolerance –Neck and low back pain –Dizzy spells – lightheaded with depersonalisation –Dizzy +++ with visual stimuli – road markings, escalators –Insomnia – wakes 3x between 2 and 6 am –Irritable and emotional with low mood –Forgetful, word-finding …….difficulties, comes out with the wrong worms Headaches –severe headaches 1-2 per month, particularly week before period –Mild headaches between – tight band around head, as if wearing a hat Rx –Nurofen 2-3 tablets per day

58 Questions: 1. What other information would you want to know about the headaches? 2. Is there a unifying diagnosis? 3. Management?

59 What other information would you want to know about the headaches? Onset of headache? –approx 13 years (mild and occasional) How did headaches change? –gradual increase frequency / severity How many actual headache-free days? –<1-2 per month Are there any other features of the hat like headaches –mild movement exacerbation causes throbbing –Subtle phonophobia and photophobia –+ very mild nausea Any other symptoms –Craves sweet foods before bad headaches Caffeine intake? –2 cups of tea per day, occasional chocolate

60 Is there a unifying diagnosis? –Chronic migraine –? Migraine-related fatigue Tests –All blood tests normal Management? 1.Stop all caffeine + all painkillers 2.Lifestyle 3L fluid / day, 3L fluid / day, avoid missing meals, avoid missing meals, set 9 hours in bed with same bedtime and no lie ins set 9 hours in bed with same bedtime and no lie ins 3.Preventative: Dosulepin (dothiepin) 25mg 3 hours before bed increase by 25mg each 2/52 according to benefit and side effects increase by 25mg each 2/52 according to benefit and side effects

61 Reviewed at 3/12: Initial thoughts after first consultation: –Mother told her that she had migraine, her daughter definitely did not and that my diagnosis was ludicrous –Patient had initially been very sceptical and did not think anything other than headaches might get better Carried out plan: Taking only 25mg dothiepin 1 migraine headache in 3/12 Now completely headache free 28-29 days per month Outcome: –Feels brilliant, totally back to normal but a bit unfit –All associated symptoms fully resolved: Neck pain, back pain, mood change, concentration, emotionalism, insomnia, visual vertigo, panic type symptoms

62 Case 2 Mad as a hatter??

63 26 year male hairdresser Referred by GP for second opinion –2 year history of severe headaches Separate vivid visual hallucinations (most < 10 seconds) flushingdizzinessnausea occasional pupillary dilatation –? Psychiatric, ? Organic brain syndrome –Managed locally as migraine – mother not happy with diagnosis

64 Hallucinations: Train on a cycle path Same train, outside his house Vivid images of children or objects Church parapet rising up in hall Stinky yellow gunge +/- followed by headache, fatigue, dizziness, vomiting Saw GP 3 years ago with occasional episodes of being completely split in two. GP laughed at me

65 Severe Headaches: Triggers: perfumes, hair perm products (osmophobia) Premonitory features: –Irritable, very agitated, confusion + slurred speech Headache: –Bilateral throbbing pain with movement exacerbation –Photophobia, phonophobia, osmophobia –Nausea and / or vomiting –+ vertigo or lightheaded / depersonalisation Postdrome – 1-2 days (fatigue, fragile, scalp tenderness)

66 Headaches: Gradual evolution with increasing attack frequency and severity Mild and severe migrainous headaches Rare complete headache free days Rx: –12-16 paracetamol and 4 nurofen plus / month Caffeine: –2 cups of coffee per day

67 PMH –Childhood abdominal pain + pallor (hours) –Childhood cyclical vomiting –Childhood travel sickness Examination: Normal mental state, systemic, and neurological examinations

68 Diagnosis in clinic: Previous acute migraine without aura Chronic Migraine –Migraine vertigo –Migraine syncope Alice in Wonderland Syndrome Full and maintained recovery to headache / symptom free with stopping painkillers and caffeine, regular food / fluids / sleep

69 Case 3 Driving her dizzy, driving me mad!

70 36 year community nurse – on long term sick leave 18/12 history of severe vertigo Triggers: –Driving on straight roads –Escalators (fallen) –Supermarket aisles –Bright walls Had to stop driving, as couldnt drive straight if prominent road markings S/B ENT – no cause found, ? non-organic

71 Neurological history in clinic: Mild pressure in head – most days –Around ears and temples –Very mild phonophobia –Mild movement exacerbation (may throb) Moderate undeserved hangover headaches every 1-2 months, especially if before period (e.g. after minimal alcohol) Wakes every hour between 2 and 6 a.m. Mild word finding difficulties / wrong words Occasional sensation of water dripping down on scalp Travel sickness as child No painkillers 2-3 tea, 1-2 coffee, 1-2 cans cola per day Examination normal

72 Diagnosis: Chronic migraine Migraine-related visual vertigo Treatment Stop all caffeine / painkillers Fluids, regular food and sleep – avoid lie ins Dosulepin 25mg, increased 50mg at 2/52 Outcome at 6/12: Dizziness completely disappeared for last 5/12 No headaches Returned to work

73 Migraine My definition: A primary headache disorder characterised by central sensitisation and various combinations of neurological, systemic, and autonomic featuresA primary headache disorder characterised by central sensitisation and various combinations of neurological, systemic, and autonomic features

74 Migraine is a relevant and very common cause of disability Very common in young adults 10% of the UK population consult a doctor each year for headache Migraine is usually invisible to others The World Health Organisation ranks acute migraine as one of the 4 most disabling / incapacitating afflictions, alongside dementia, psychosis and quadriplegia

75 Acute Migraine Triggers –Sleep deprivation –Sleep excess (eg saturday morning headaches) –Missing meals –Dehydration –Alcohol (deserved or undeserved hangovers) –Hormonal (eg premenstrual, menstrual, 1 st trimester, postpartum, menopause) –Exercise –Travel –Stress / after stress –Dietary (very rare - < 1-2%) – e.g. citrus fruit, cheese

76 The 4 stages of acute migraine Aura Aura Prodrome Postdrome Prodrome Postdrome Headache Headache + Associated + Associated features features Hours MinutesHours Usually 1-2 days to hoursto days to hoursto days

77 Acute Migraine – Prodrome (premonitory features)* Mental State NeurologicalGeneral FatigueIrritability Depressed mood EuphoriaHyperactivityRestlessnessDepersonalisationDerealisation YawningPhonophobiaPhotophobiaOsmophobiaLightheaded Food craving Dizziness Neck pain / stiffness Anorexia Frequent micturition Diarrhoea *prodrome seen in about 60% of patients

78 Migraine - Aura Only present in 20% of migraineurs Symptoms usually evolve over time –Most commonly 20-30 minutes –May persist hours - months Pathophysiology is not vascular (eg vasoconstriction / dilatation): –cortical spreading depression May occur without headache –acephalalgic migraine –More common in elderly A complex of focal neurological symptoms (positive or negative phenomena) that precede or accompany an attack

79 Migraine - Aura VisualSensory –unilateral or bilateral –e.g. pins and needles slowly spreading up arm to face to leg Motor –Weakness –Movement disorders Dysphasia / acute confusional state / coma, etc.

80

81 What are migrainous features of headache ? Throbbing / pounding Head, neck and / or face Unilateral or bilateral Tenderness Nausea +/- vomiting Exacerbating / trigger factors –Movement –Noise (photophobia) –Light (phonophobia) –Smell (osmophobia) Relieving factors –Flat –Still –Vomit –Sleep

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83 Non-headache symptoms of acute migraine Mental State NeurologicalGeneral DepressionAnxietyFatigueIrritabilityIncapacityConfusion Blurred vision Paraesthesiae Sensation of insects or water on scalp or water on scalpVertigo Acute confusion Word-finding difficulty AutonomicHemiplegiaComaLightheadednessSyncopeFlushing Cold extremities Scalp / face oedema Hair loss Neck stiffness Anorexia Nausea / vomit EructationDiarrhoeaPolyuria

84 Migraine Autonomic Symptoms Approx 20% of migraineurs Localised facial disturbance Conjunctival injection (red eye) Lacrimation (tearing) Eyelid swelling Ptosis Nasal congestion / rhinorrhoea (less common) Objective scalp or facial swellling Flushing (may be unilateral) Migraine is the commonest cause of facial autonomic disturbance facial autonomic disturbance

85

86 Migraine – postdrome Resolution often associated with: FatigueFragility Scalp tenderness

87 Treatment of acute migraine Be certain that > 25-27 brilliantly crystal clear headache-free days before prescribing acute attack drugs If previous acute attack drugs have failed, likely to have chronic migraine + medication/caffeine overuse Lifestyle: –?stop caffeine –Good fluid intake, regular meals, regular to sleep

88 Rules of acute attack drugs Treat as soon as throbbing headache starts Early treatment works better But: do not treat in aura, as drugs ineffective Do not keep using acute drugs in same attack if prolonged beyond 2 days (drugs will perpetuate attack to status migrainosus) Beware that any headache with migrainous features may respond to triptans regardless of underlying cause (eg tumour, SAH)

89 Treatment of acute migraine Fluids ++ at onset Ensure no nausea (domperidone) RestNon-invasive –Cold packs –4head –Menthol strips –Massage – neck, temples, scalp

90 Treatment of acute migraine 1 st line: –Painkiller Soluble aspirin 900mg Naproxen 500mg Paracetamol+ –Domperidone (aid absorption of painkiller / fluids) 20mg oral QDS PRN if nausea 30-60mg PR BD PRN if vomit Avoid other anti-emetics that compound gastric stasis –Stemetil, buccastem, cyclizine Avoid metoclopramide in young people –Risk of oculogyric crisis

91 Treatment of acute migraine 2 nd line –Triptans Start with oral –Eg sumatriptan 50mg, sumatriptan 100mg, If vomit, consider nasal spray –E.g. Zomig 5mg (head tipped forward) If wakes already with headache, consider s/c –E.g. Imigran 6mg s/c

92 Status migrainosus Continued attack for days –Treat with 1.Stop all painkillers / triptans 2.Avoid caffeine 3.Antiemetics 4.Hydrate+++ 5.+/- 3/7 course of prednisolone

93 Women and migraine Relative risk of stroke if aura and OCP –Discuss –Not absolute contraindication but avoid if possible –If necessary, use low oestrogen dose –Consider POP Menstrual migraine –Consider tri-packed low dose OCP

94 Migraine preventatives and OCP Topiramate induces pill (minimal) –Therefore use higher dose COCP or POP

95 Migraine preventatives and pregnancy Best to avoid if possible Teratogenic: –Topiramate (? Risk) –Valproate (risk of fetal valproate syndrome) High risk of significant learning disability / low IQ –Tricyclics – probably safe (dosulepin, amitript) –Beta-blockers – probably safe

96 Women and migraine HRT is OK in menopausal women with previous hx of aura as low dose replacement Note that worsening migraine may be presenting feature of menopause before LH / FSH changes seen in blood; HRT may be worthwhile as treatment of migraine in this instance

97 Migraine and stroke risk Migraine with aura: v slight increased risk Migraine without aura:no increased risk Migraine with aura + smoking: approx 8x increase risk (more if chronic migraine) –Independent of amount smoked –Occasional cigarettes may be just as harmful –Risk declines over 2 years back to non-smoker level if stop

98

99 Chronic Migraine

100 Gradual characteristic evolution from acute to chronic state 1. Frequency increases 2. Severity can increase or decrease 3. Gaps between severe attacks fill in with milder migrainous headaches (bilateral > unilateral) 4. Acute attack medication loses efficacy e.g. painkillers / triptans 5. Pervasive non-headache features usually diminish / disappear on complete headache-free days Frequent headaches with migrainous features + < 15 days per month headache-free

101 Features of Chronic Migraine 3 types of headache usually seen: 1.Incapacitating headaches (often typical of previous migraine) 2.Frequent background pressure or band-like headaches with mild or subtle migrainous features 3.+/- Idiopathic stabbing headaches Migraine-associated symptoms Often associated medication and/or caffeine overuse, leading to failed trials of preventative medication Chronic migraine may occur in relative absence of headache

102

103 Medication Overuse ? Main cause of lack of response to headache preventatives All acute attack medications can cause medication overuse: If co-morbid neck pain, back pain or fibromyalgia, still worth stopping painkillers, as central sensitisation may heighten other bodily pains.

104 Caffeine Overuse Not proven, but long recognised to cause headaches, especially on withdrawal Caffeine regarded as acute attack medication Often in combined analgesics Mild headaches (e.g. regarded as TTH) almost always disappear with complete elimination of acute medication and caffeine Caffeine withdrawal - first line for treatment- resistant depression

105 Tea and Coffee Headaches. – 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 useTea and Coffee Headaches. – 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 Virtues Household Physician – circa 1920

106 Chronic Migraine Triggers and Perpetuating Features An Inherited Predisposition: A genetic disorder +/- Family history Travel sickness ChildhoodChildhood Adulthood – with readingAdulthood – with reading +/- previous migraine Migrainous hangovers Undeserved hangovers Comorbid IBS Triggers:Hormones PregnancyPregnancy PostpartumPostpartum OCPOCP MenopauseMenopause Viral infection Head injury Systemic illness Neurological illness Neurosurgery Emotional stress Idiopathic Perpetuating Factors:Painkillers Opioids Opioids Paracetamol Paracetamol NSAIDS NSAIDS Triptans / Ergot Caffeine Coffee Coffee Tea Tea Cola Cola Chocolate Chocolate Lucozade Lucozade

107 Pani Chronic Migraine: More Than Just a Headache Coathanger Neck Pain Biological Disturbance: Insomnia, poor STM, word substitutions, irritability, emotionalism, depression, anhedonia Fatigue Migraine Vertigo; Visual Vertigo; Veering Reflex Syncope +/- Frequent Headache Sensory Disturbance (unilateral / bilateral) Panic-Type Symptoms Lightheaded, depersonalisation derealisation, P+N, tinnitus, mute hearing, blurred vision, etc. +/- panic Restless Legs syndrome Distortion of Reality / Perception Autonomic symptoms

108 Fatigue Fatigue is common in chronic migraine 1 : –84% scored >3 on Fatigue Severity Scale (FSS) 2 –67% met CDC 3 criteria for Chronic Fatigue Syndrome Headache is commonly not volunteered by patients when presenting with other complaints Chronic migraine should be considered in all patients presenting with chronic fatigue 1 Peres et al (Cephalagia 2002:22:720-724) 2 c.f. normal (<2.8), MS (5-6.5), depression (4.5), CFS (6.1) 3 Center for Disease Control and Prevention

109 Migraine-related dizziness 1. Panic type symptoms Lightheaded Depersonalisation / derealisation Hot, sweaty, flushed Blurred, dim, or spotty vision Mute and buzzy hearing +/- panic +/- situation-specific – hot, bright, noisy, crowded 2. Migraine vertigo 3. Visual vertigo 4. Unexplained veering

110 Distortion of reality as a manifestation of migraine –Visual aura –Teleopsia - zoom vision –Surroundings may appear very big or very small –Body image disturbances body parts appear large, small, distorted, reduplicated or absent –Entomopia – Insect eye - multiple copies of same image in grid-like pattern –Corona phenomena –Hallucinations Visual Auditory Olfactory Gustatory Tactile –Cognitive deficit apraxia, agnosia acute confusional state –Language disturbance foreign accent syndrome –Delusions –Paranoid psychosis

111 Alice in Wonderland Syndrome Lippman 1952: Certain Hallucinations peculiar to Migraine –1 patient with left ear ballooning out 6 inches or more –Body split in 2 halves as if by vertical line, with right size twice the size of left. Syndrome named by Todd, 1955, in relation to migraine and epilepsy: –Characterised by the core symptoms of body schema disturbances and by a number of facultative symptoms, including depersonalisation, derealisation, visual illusions and illusory alterations in the passage of time Bizarre visual illusions and spatial distortions –Macropsia – world appears larger than normal / subject appears smaller –Micropsia – opposite of macropsia –Metamorphosia - sensation of formed body distortions –Zoom vision (e.g. teleopsia - visual illusion of images moving away) –? Parietal phenomena Sense of time speeding up or slowing down More commonly reported in children Often occurs before the headache begins Usually followed by headache Also reported to occur with infectious mononucleosis, complex partial seizures, and drug ingestion.

112 Corona phenomena Visual hallucinations in hemianopic visual field and corona phenomenon. Podoll and Robinson, Cephalagia 2001;21:712-717

113 Splitting of the body image Podoll and Robinson, Cephalagia 2002;22:62-65)

114 Macrosomatognosia Macrosomatognosia of both hands and arms. (Podoll and Robinson, Acta Neurolo Scand 2000;101:413-416)

115 Macrosomatognosia Macrosomatognosia of head, neck, both arms and hands. (Podoll and Robinson, Acta Neurolo Scand 2000;101:413-416)

116 Migraine The Chameleon in the Neurology Clinic Headache Dizziness and Vertigo Blackouts Sensory disturbance FatigueInsomnia Panic Attacks (+/- panic) Chronic Pain –Neck pain / Brachalgia –Facial pain –Fibromyalgia ? MS ? Epilepsy ? NEAD ? TIA ? Stroke Chronic Fatigue Syndrome ME ? Conversion disorder Depression

117 My approach to successful treatment of chronic migraine 1. Withdraw all acute attack medication / caffeine 2. Lifestyle –regular sleep times without lie ins or daytime sleep –good hydration (2 ½ to 3 litres) –regular meals 3. Preventative (6-12 months) –Start 2-3 weeks after analgesic / caffeine withdrawal Other Measures: – Encourage regular exercise as patient recovers – Ensure completely avoids smoking if migraine with aura, significantly increased stroke risk – Avoid oestrogens below 50 years if migraine with aura, as increased stroke risk The foundation 1.No painkillers 2.No caffeine 3.Good fluids 4.Regular meals 5.Regular sleep

118 My approach to successful treatment of chronic migraine The withdrawal: Warn of possible severe worsening for 1-2 weeks Worsening is a good sign and usually heralds reverse to acute migraine Admit for in-patient detoxification if severe triptan overuse, suicidal ideation –May assist withdrawal with: Fluids (+/- IV) Oral / rectal domperidone 5/7 Naproxen 500mg 8am + 4pm Clonidine (if opiates ++) IM Chlorpromazine IV Dihydroergotamine Steroids Combined pain syndromes: Advise that other pains often eventually improve off painkillers (especially neck and back), due to cessation of central sensitisation Consider other measures for other pains: –Back pain – Pilates, Extensor stretch exercises, swimming, pain clinic – epidurals etc –Neck Pain – usually improves ++ –Arthritis – glucosamine, large joint revision etc.

119 Trial Evaluation Criteria Study size (Cited trial (n)*) Study population (ITT/completers) Treatment period (no. weeks) Licensed for migraine prophylaxis? Topiramate 1 568ITT26Yes (tertiary care) Propranolol 1 568ITT26Yes Pizotifen 2 30ITT6Yes Sodium valproate 3 29Completer16No Amitriptyline 4 100Completer8No 1. Diener H-C et al. J Neurol 2004; 251: 943–950. 2. Osterman PO. Acta Neurol Scand 1977; 56: 17–28. 3. Hering R, Kuritzky A. 1992; 12: 81–84. 4. Couch JR, Hassanein RS. 1979; 36: 695–699. * Largest reported or most often cited double-blind, placebo-controlled trial Migraine prevention: Treatment options Evaluating migraine clinical trials ITT = intent-to-treat

120 Preventative Drugs for Migraine Licensed Unlicensed Beta Blockers* –Propranalol (best evidence for use) –Timolol, Metoprolol Antiepileptic Drugs (AED) –Topiramate*** Others –Clonidine (antihistamine and serotonin antagonist) – of no proven efficacy (BNF states Clonidine is not recommended and may aggravate depression and cause insomnia) –Pizotifen - evidence for effectiveness is poor; adverse effects severely limit use –Methysergide*** – considered very effective but concerns about about ergot side effects (retroperitoneal fibrosis etc) Beta Blockers –Atenolol (not licensed, but commonly used) –Nadolol Tricyclic antidepressants** –Amitriptyline (best studied) –Dosulepin (commonly used; potentially better tolerated) Antiepileptic Drugs (AED) –Sodium Valproate** –Gabapentin (limited evidence of efficacy – 1 study)NeurolepticsAlternative –Butterbur, coenzyme Q10, riboflavin, feverfew * Partial agonists unhelpful; ideal beta blocker is hydrophilic and cardioselective ** Unlicensed, but recommended for use in BNF! *** Hospital Supervision or Specialist Introduction only

121 Chronic Migraine - Rx Dosulepin (or amitriptyline) –Aim for dose causing dry mouth without persistent tiredness –Take approx 3 hours before bed –Aim initially for approx 1mg /kg – sometimes helps to go higher Epilim Chrono –+ Folic acid / contraception if fertile female –Warn – side effects: weight gain, hair loss, tremor –Up to 1000mg bd Topiramate –P+N at higher doses (often settles) –Fluids++ to avoid renal calculi + Folic acid / contraception if fertile female Weight loss may occur (< 10% body weight)+ Folic acid / contraception if fertile female Weight loss may occur (< 10% body weight) –Aim for 50mg bd; some respond to higher doses (< 250mg bd) Paroxetine –?avoid in children –Warn side-effects (dizzy, nausea, drowsy) typically last only 2/52 –Probably best starting 10mg, increase 20mg after 1/52 Propranalol (Inderal LA) –Avoid if depression –Up to 320mgFlunarizine –Rx of hemiplegic migraine, alternating hemiplegia of childhood –Helpful in resistant cases –s/e weight gain, sedation, parkinsonismGabapentin –Not very effective

122 Chronic Migraine - Rx Olanzepine –Helpful if very resistant cases –Some very positive experience in those resistant to all other drugs (in specialist clinics only) Pizotifen –Very poorly tolerated – weight gain and sedation –If tolerated, works reasonably –Rarely used in headache clinics Methysergide –Safe <12mg if monitor and drug holidays Clonidine –Licensed, but never been shown to help Lamotrigine, verapamil, carbamazepine –Unlikely to work as migraine preventativesAcupuncture –Real but clinically insignificant benefitPhysio –Helpful for short term relief only (e.g. hours to days) Occipital Nerve Stimulator –Experimental PFO Closure –Poor evidence –Only advocated by some for migraine + aura –No good trial data

123 Common misdiagnosis of chronic migraine Cervicogenic Headache - ? Exists – probably, but very rare Chronic Tension Type Headache – ? Overdiagnosed+++ (CTTH is never severe or associated with features) I have seen 3 cases in last 3 years! Eye Strain – headaches only when reading / computer etc. Dental Headaches –TMJ dysfunction - ? overdiagnosed –Dental disease – ? overdiagnosed Atypical facial pain - Migraine commonly causes pain down lower jaw, over saddle of nose, in teeth, or over maxilla Sinus headache - >95% of cases are migrainous Hypertensive Headaches – only if encephalopathy

124 Learning points: If in doubt, diagnose migraine Migraine may present with isolated facial pain Migraine may occur without any pain Migraine often misdiagnosed as: –Atypical facial pain –Trigeminal neuralgia –Sinusitis, TMJ dysfunction Always ask about migraine where unexplained symptoms Always ask about headache-free days to guide management and to help diagnose associated features as migrainous –E.g. chronic fatigue, fibromyalgia, ME, cervical spondylosis and depression would not disappear on headache-free days! A response to triptans does not confirm migraine and exclude serious underlying pathology

125 Summary Chronic Migraine accounts for more than 90% of referrals to a specialist headache clinic It is frequently misdiagnosed in hospital and primary care Chronic Migraine is a treatable disorder Chronic migraine may present with features other than headache It is highly worthwhile taking full migraine history in patients presenting with unexplained neurological symptoms Chronic migraine is an invisible cause of significant disability For copy of slides: e-mail Anne.McCann@thewaltoncentre.nhs.uk

126 Useful websites BASH: The British Association for the Study of Headache Management guidelines www.bash.org.uk IHS: International Headache Society Current IHS diagnostic criteria 2004 www.i-h-s.org Prodigy: new revised information and patient information sheets www.prodigy.nhs.uk www.prodigy.nhs.uk

127 Appendix – PFO Closure MIST Study: –Prospective double blind placebo-controlled study (n=147) –Patients had only failed 2 preventatives –Study of frequent but not chronic migraine –Based on observation that closure of large right to left cardiac shunts may abolish migraine –Studied Migraine with Aura (only 20% of patients with migraine experience aura, and only proportion of these have significant cardiac shunts) –Conclusion: Negative Primary Endpoint, not reaching 40% elimination of migraine at 6 months 37% (PFO Closure) vs 17% (sham) Complications included tamponade, pericardial effusion, retroperitoneal bleed, and atrial fibrillation Results can not be generalised to migraine without aura NB short (6/12) follow up - impossible to conclude curative Rx for chronic disease –BASH: On the basis of the current evidence, the cost and risk of this intervention are not commensurate with the benefits received.

128 Appendix – Indometacin Challenge Diagnostic test and Rx for Hemicrania Continua and Paroxysmal Hemicrania (protocol available as patient information sheet from Walton Centre) 25mg tds 2/7 50mg tds 2/7 75mg tds 2/7 100mg mane, 75mg midday, 75mg nocte 2/52 Always cover with PPI (eg omeprazole) Stay on lowest dose once diagnosis established by complete elimination of pain with Indometacin If not tolerated and diagnosis uncertain, consider double-blind IM Indotest –(protocol and patient info sheet available from Walton Centre Pharmacy) If not tolerated for treatment, consider GON blocks, GON stimulator, other anecdotal therapies

129 Appendix – IHS Criteria IHS diagnostic criteria for migraine without aura A: at least five attacks fulfilling criteria B–D B: headache attacks lasting 4–72 hours C: headache has >2 of the following: unilateral location pulsating quality moderate or severe pain intensity aggravation by/causing avoidance of routine physical activity D: during headache at least one of: nausea and/or vomiting photophobia and phonophobia E: not attributed to another disorder Use supplementary to normal enquiry practice


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