Dr David PB Watson GPwSI Headache Hamilton Medical Group Aberdeen Top Ten Headache Tips Dr David PB Watson GPwSI Headache Hamilton Medical Group Aberdeen

Top Tip 1 Diagnosis is by history, history and history T timing O other associated symptoms S site S severity I influences aggravating/relieving factors T type: what it feels like

Top Tip 2 Secondary Headache is Rare Studies show PPV headache 0.09% in primary care for brain tumour i.e. 1 in 1000 Migraine CT Scan 2 in 1000 abnormal Neurology OPD: 1 in 100 secondary cause A and E :1 in 10 secondary cause

“Red flags” Single cohort (Level 3) or expert opinion (Level 4) new onset headache in patients who are aged over 50 29-31 abrupt onset (thunderclap) 28-30, 32, 33 focal symptoms including atypical aura greater than one hour 28, 32, 34, 35 abnormal neurological examination 28, 29, 35, 36 altered mental status 28, 30, 34 altered characteristics or associated features of headache 28, 31 headache that changes with posture 37 headache worse during physical activity, and the valsalva manoeuvre 28, 38 patients with risk factors for thrombosis 34, 39, 40 new onset headache in a patient with a history of HIV infection 41 jaw claudication 16 neck stiffness 30 fever 42 new onset headache in a patient with a history of cancer 9

Episodic primary headaches Migraine +/- aura Tension-type headache (TTH) Cluster Probable migraine There are four types of episodic primary headaches (IHS 2004): Cluster: episodic cluster headaches occur in periods lasting from 7 days to 1 year and are separated by pain-free periods lasting 1 month. Pain is severe and unilateral. Attacks are associated with 1 of the following signs: conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and eyelid oedema. Tension-type: episodes of headache lasting days to months. The pain is typically bilateral, pressing or tightening in quality, of mild-moderate intensity and does not worsen with physical activity. There is no nausea, but photophobia may be present. Migraine ± aura: patients have periods of severe impairment followed by return to a normal neurological baseline. Headache pain is often unilateral, of moderate intensity and pulsating quality. The headache is worsened by physical activity and is frequently associated with nausea, photophobia and phonophobia. Probable migraine: previously categorised as ‘migrainous headache’. This diagnosis is given if the headache is missing 1 features of migraine ± aura.

Chronic primary headaches / chronic daily headaches Chronic cluster Chronic migraine +/- medication overuse Chronic tension Chronic daily headache (CDH) Hemicrania continua New daily persistent There are five forms of chronic primary headaches (also known as chronic daily headaches [CDH]) (IHS 2004): Chronic migraine: diagnostic criteria for migraine without aura and occurs on 15 days/month for >3 months in a row. May be associated with medication overuse. Chronic tension: diagnostic criteria for tension headache and occurs on 15 days/month for >3 months in a row. May be associated with medication overuse. Chronic cluster: diagnostic criteria for cluster headache, but with attacks for 1 year without remission or with remission periods of <1 month. New daily persistent headache: starts acutely and continues as constant unremitting pain, lasting >4 hours/day and occurring 15 days/month for 1 month. Hemicrania continua: persistent unilateral, stabbing, continuous pain specifically located in the trigeminal nerve. Accompanied by 1 of conjunctival injection ± lacrimation, nasal congestion ± rhinorrhoea, ptosis ± miosis.

Top Tip 3 Episodic disabling headache is migraine Over diagnosis of sinus headache and TTH 40% migraineurs miss ICHD-II criteria Cody Jarrett

What features make migraine more likely? episodic severe headache that causes disability11, 23, 24 nausea16, 23 sensitivity to light during migraine headache16, 23 sensitivity to light between migraine attacks 25 aura16, 18 sensitivity to noise16 exacerbation by physical activity16 positive family history of migraine16 The features which give the greatest sensitivity and specificity are Disability, Nausea and Sensitivity to light23 ID Migraine validation study (Level 3)

Top Tip 4 Choose acute migraine medication according to the patient’s symptoms Can use a step approach

Top Tip 5 Response to triptans is idiosyncratic and consistency across attacks is variable Diamond headache clinic

Top Tip 6 A good response to migraine prophylaxis is a 50% response in 50% of patients Choice of prophylaxis is guided by co-morbidites Pizotifen is a poor drug

Top Tip 7 Migraine with aura is an absolute contraindication to the combined contraceptive due to increased stroke risk

Top Tip 8 All headache medications can cause headache

Top Tip 9 There is no magic answer to the management of medication overuse headache other than to stop the medication

Top Tip 10 Short lasting unilateral headaches with autonomic symptoms think of the trigeminal autonomic cephalalgias (TACs)

What features make TACs more likely? The following features differentiate trigeminal autonomic cephalalgias from migraine: 16, 26 (Level 4) Onset: rapid in TAC, gradual in migraine Duration: TACs < 3 hours, migraine 4 - 72 hours Frequency: multiple attacks may occur daily in TACs Restlessness during an attack: 100% in cluster headache, 50% in paroxysmal hemicrania Prominent ipsilateral autonomic features in TACs

Take home points migraine common history is key to diagnosis impact is important remember overuse of medication tailor treatment to patient Refer if red flags, consider for chronic migraine/MOH, TACs, untoward patient angst

Any Questions?

Case 1 22 years age Episodic headache for 5 years Attended for COP check What do you want to know?

Case 2 47 year old man 2 migraine headaches 6 and 10 years ago 3/12 ago had 3 weeks of headache awaking him from sleep What do you want to know?

Case 3 27 year old lady Migraine since 16 Frequency is 1-2 per month Woke at 5 am with worst ever migraine What do you want to ask her?

Case 4 75 year old male migraine since 15, none for 10 years Last 3 days had migraine like headache Called GMEDs at 6 am as had D and V all night and still has headache What do you want to ask him?

Case 5 40 year old man severe headache for 1 hour Previous similar headaches diagnosed as migraine Not responded to naratriptan 2.5 mg What do you want to ask him?

Case 6 48 year old lady Migraine since 16, menstrually associated, none when pregnant Last 2 years more frequent Big headaches twice a month Little headaches 3 times a week What do you want to know?

Case 7 53 years, migraine since 15 Last 6 years headache every day History of depression, agoraphobia, back pain Very noticeable profound parkinsonism tremor What do you want to know?

Abbreviated diagnostic checklist based on IHS 2004 criteria Migraine Probable migraine Tension-type Essential (3) Essential (2) Essential (1) Recurrent No organic disease Duration 4-72 h Unilateral Pulsating Moderate / severe Aggravated by movement Nausea / vomiting Photo / phonophobia Recurrent No organic disease Duration 4-72 h Moderate / severe + one other Recurrent No organic disease Duration 0.5 h-7 days Generalised Pressure / tightness Slight / moderate Photo / phonophobia Although migraine, migrainous headache and tension-type headache are all primary episodic headaches, each has characteristic differentiating features. The IHS has used these features to develop diagnostic criteria for each of these headache types (IHS 2004). This slide presents an example of how an abbreviated diagnostic checklist for headaches can be derived from the clinical criteria provided within the International Classification of Headache Disorders (IHS 2004). The checklist was developed by Dr Robert Smith in order to facilitate use of the IHS criteria in the primary care setting (unpublished). Essential (3) = all items essential for diagnosis; Essential (2) = two items from list essential for diagnosis; Essential (1) = one item from list essential for diagnosis IHS 2004

The migraine attack Symptom intensity Associated symptoms Prodrome For any given patient, or any given attack, migraine attacks typically progress through several phases: prodrome; aura; headache phase ± associated symptoms; postdrome. Not all patients will experience every phase. Migraine attacks will differ in severity and characteristics for each patient. Prodrome Aura Headache Postdrome Time

Prodrome 60% of migraine sufferers experience premonitory phenomena Excitatory Irritability Elation Hyperactivity Yawning Food cravings Photophobia / phonophobia Increased bowel / bladder activity Inhibitory Mental / physical slowing Poor concentration Word finding difficulty Weakness / fatigue Constipation / abdominal bloating Anorexia Chill The prodrome phase may occur between a few minutes to 48 hours before the onset of a headache pain. Approximately 25% of migraine sufferers experience premonitory feelings (elation, irritability, depression, hunger, thirst, or drowsiness) (Silberstein and Young 1995), which are generally classified as excitatory or inhibitory in origin (Waelkens 1985). Premonitory symptoms are not always well defined; some migraine sufferers only have a vague feeling that a migraine attack is imminent. More than half of all migraine sufferers can identify some premonitory phenomenon, but this information tends not to be volunteered or thought about unless patients are specifically asked about it. Headache diaries can therefore be invaluable for patients and clinicians to fully understand the migraine syndrome.

Aura Affects 33% of migraine sufferers, but not in all attacks Transient neurological symptoms resulting from cortical or brainstem dysfunction May involve visual, sensory or motor systems Can occur before or during headache phase Slow evolution of symptoms Lasts for 20-60 minutes Can be confused with transient ischaemic attack Although aura affects around 33% of patients, it does not present with every migraine attack they experience. The typical onset of aura is 5-20 minutes and it generally lasts <60 minutes (Ferrari 1998); it is not always clearly demarcated and may extend into the headache phase. Visual aura is the most common aura symptom and include loss of vision (eg scotoma), a blind spot that progresses or enlarges across the visual field, or less commonly, complete loss of vision in one visual field (hemianopia) (Spierings 2003; Russell and Olesen 1996). Sensory aura include paresthesia (tingling) typically starting in one hand, spreading to the arm, elbow, face, lips and tongue. Motor aura is typically experienced on one side and affects the hand and arm (Russell and Olesen 1996). A rare form of aura involves the brainstem, resulting in quadraplegia, loss of consciousness or cranial nerve deficits. Young children with migraine may experience confusion, ataxia or aphasia. Elderly patients may experience aura without headache, sometimes called acephalic migraine, that at times may be difficult to distinguish from transient ischaemic attacks. A non-vascular pattern and stereotyped recurrence, along with a prior history of migraine, can be reassuring for patients who experience aura. Ferrari 1998 Spierings 2003 Russell & Olesen 1996

Headache phase Throbbing or pounding quality If left untreated, headache pain will progress to moderate / severe intensity Duration 4-72 hours in adults 2-8 hours in children Exacerbated by movement* One-sided temporo-orbital* Abated by sleep* Resolves spontaneously Headache is often the first phase of the migraine to be recognised by the patient, as it is generally the most debilitating part of a migraine attack. The pain may build up gradually to a peak over a period of minutes or hours, or it may plateau and then gradually subside. Many migraine sufferers will have an occasional attack that wakens them at night with full-blown headache pain. If untreated, the headache pain generally lasts for 4-72 h in adults and 2-8 h in children. The severity of the headache pain can vary greatly between attacks. Most migraine sufferers, however, report pain of 5 on a scale of 0 to 10. Migraine headache pain is usually one-sided, although attacks may occur on alternate sides of the head. The site of maximal pain is usually temporo-orbital but often radiates into the ipsilateral occiput and neck, and may spread to the opposite side of the head (Pryse-Phillips et al 1997). Abatement with sleep can be a characteristic of migraine pain (Pryse-Phillips et al 1997). Exacerbation of pain by head movement or routine physical activity, such as climbing stairs, are also characteristic features. Left untreated, the pain of migraine will spontaneously resolve. *Usually

Postdrome Estimated to affect 90% of migraine sufferers Phase after pain relief duration up to 24 hours Sufferers may experience: hyperaesthesia, mood changes, muscular weakness, fatigue, difficulty in concentrating Extends period of migraine-related disability The postdrome phase occurs after pain relief is achieved and may persist for 24 hours (Blau 1982). Sufferers experience characteristic symptoms such as hyperaesthesia (scalp sensitivity, valsalva-induced headache), lethargy and depression. Other symptoms include mood changes, muscular weakness, fatigue and difficulties concentrating. These feelings may be attributed to migraine medications or may be caused by the migraine itself. The postdrome phase extends the migraine-related disability that is often associated with the headache, but is not actually associated with headache pain. Blau 1982

Migraine characteristics that aid diagnosis Frequent association with menstrual cycle Characteristic triggers Paradoxical relationship to sleep Familial history of migraine Cognitive impairment with attacks Dizziness and / or vertigo Several characteristic features of migraine are not part of the IHS classification criteria but are recognised in clinical practice and aid diagnosis (Pryse-Phillips et al 1997): temporal association with the menstrual cycle is very characteristic of female migraine sufferers; triggers that may precipitate a migraine attack include changes in altitude, stress, weather changes, fasting, certain foods and sleep patterns; migraine can have a paradoxical relation to sleep; attacks frequently occur during sleep or upon awakening, however, migraine can also be relieved by sleep; a family history of migraine is present in up to 80% of migraine sufferers; migraine sufferers will frequently report fatigue and cognitive impairment associated with attacks, recognised as poor concentration, memory deficits or difficulty finding words.

Migraine triggers Climate High altitude Hot baths Intense smells Noise Glare Chocolate Cheese Alcohol Oral contraceptives Caffeine Menstruation Toothache External stimuli Dietary Hormonal Systemic Travel Fatigue Exercise Smoking Hunger Sleep Sex Migraine is a neurovascular response to internal or external triggers (Lance et al 1993; Raskin and Knittle 1976). Internal triggers may be dietary, hormonal or systemic (emotional or physical). Identification and avoidance of migraine triggers forms an essential part of the treatment strategy for migraine sufferers (Martin 2001). Anxiety Emotion Depression Shock Excitement Stress Physical stresses Emotional stresses

Mode of action of triptans Trigeminal sensory afferent nerve fibres Photophobia Phonophobia Neuropeptides Neurokinin A Substance P CGRP Post-junctional receptor Higher CNS centres PAIN PAIN Vasodilation extravasation Thalamus Post-junctional receptor Trigeminal ganglion Direct vasoconstriction Nerve activation reduced Peptide release inhibited Nerve activation reduced Most second-generation triptans cross the blood-brain barrier and exert their anti-migraine effects both peripherally and centrally, causing reduction of neurogenic inflammation and inhibition of pain transmission (Ferrari 1998). Triptans relieve migraine pain by stimulating peripheral and central serotonin receptors to: initiate vasoconstriction of the cerebral blood vessels (peripheral effect) inhibit release of vasoactive peptides causing dural neurogenic inflammation with fluid extravasation from its vessels (peripheral effect) inhibit pain neurotransmission in the trigeminal pathway (central effect). Unlike the second-generation triptans, first-generation triptans do not cross the blood-brain barrier and only mediate their anti-migraine effects by reducing neurogenic inflammation (Ferrari 1998). Decreased pain transmission Peptide release inhibited Post-junctional receptor Trigeminal nucleus Caudalis Autonomic activation Nausea emesis CNS, central nervous system CGRP, calcitonin gene-related peptide Ferrari & Saxena 1993 Goadsby & Hoskin 1996

Trigeminovascular system: Anti-migraine targets Cortex Higher CNS Centres PAIN Phonophobia Photophobia TARGET Thalamus Trigeminal ganglion Autonomic activation Nausea, Emesis Intracranial blood vessels TARGET Trigeminal nucleus caudalis 3 18 15 12 2

The triptans Stronger than sumatriptan 100 mg Rizatriptan, eletriptan Equal to sumatriptan 100 mg almotriptan, zolmitriptan Weaker than sumatriptan 100mg, frovatriptan, naratriptan

Other primary headache Trigeminal autonomic cephalalgias (TACs) Cluster headache Paroxysmal Hemicrania (indometacin) SUNCT Hemicrania continua (indometacin) New daily persistent headache

Cluster prophylaxis Prednisolone high dose (60 mg daily) Verapamil ( 240 to 720 mg daily) Lithium (600 to 900 mg daily) Methysergide( fibrosis retroperitoneum,pleural pericardial linings)

Cluster Acute Rx High flow oxygen (7 –12 l/ min) Sumatriptan subcutaneously Nasal zolmitriptan Lignocaine nose drops IV dihydroergotamine ( not UK)