Primary Headache Diagnosis RCGP 28.4.2009 Dr David PB Watson GPwSI Headache Hamilton Medical Group Aberdeen

Primary Headaches Tension Type headache Migraine Trigeminal Autonomic cephalalgias Others

Headache types Primary headache Secondary headache Episodic primary headaches Chronic primary headaches Primary headache No underlying medical cause: Tumour Meningitis Vascular disorders Systemic infection Head injury Drug-induced Secondary headache Underlying medical cause: Headache can be a primary disorder or a secondary symptom of another disease. Primary headache may also be secondary to another disorder, for example, the relationship between cervical spine disorders and migraine headaches. The headache classification system of the International Headache Society (IHS), first published in 1988 but recently revised (2004), lists >100 types of headache, many of which are attributable to pathological conditions (IHS 1988, 2004). Secondary headaches are common in patients with an intracranial process, including tumour, haemorrhage, infection or vascular disorders. They may also be associated with systemic infections, the use/overuse of various drugs and head injury. Primary headaches (eg episodic primary headache, chronic primary headache, migraine, tension-type headache [TTH] and cluster headache) have no obvious underlying cause. Accurate diagnosis of headache type is critical to the therapeutic recommendation. Secondary headache to an underlying disorder must be excluded, followed by a correct primary headache diagnosis and treatment plan.

Episodic primary headaches Probable Migraine Tension-type headache (TTH) TACs 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.

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

Key points Episodic disabling headache is migraine Over diagnosis of sinus headache and TTH 40% migraineurs miss ICHD-II criteria

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

Migraine Epidemiology High impact 6 million people in the UK Women 3x men Most sufferers 20 to 50 High impact WHO disability Personal impact

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

Aura v TIA AURA TIA Sequential Progression 20-60 minute duration Positive and negative symptoms Headache occurs in 50% Visual Sensory Simultaneous occurrence <15 minute duration Negative symptoms Headache uncommon

Headache phase Throbbing or pounding quality If left untreated, headache pain will progress to moderate / severe intensity Duration 4-72 hours in adults Exacerbated by movement* One-sided temporo-orbital* (but can be any site) 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

Cutaneous Allodynia Perception of pain when non-noxious stimulus applied to normal skin central sensitization of neurons in the trigemino-vascular system wind-up up to 70% migraineurs treat early

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)

Trigeminal Autonomic Cephalalgias Cluster headache Paroxysmal Hemicrania SUNCT

Cluster Headache Men smokers severe pain (Eagle’s Claw) high impact autonomic features

Cluster IHS Criteria Severe or very severe unilateral,supraorbital and or temporal pain lasting 15-180 min if untreated Headache accompanied by one of the following Ipsilateral conjunctival injection and or lacrimation Ipsilateral nasal congestion and or rhinorrhoea

Cluster IHS Attack frequency 1 every other day to 8 per day Forehead and facial sweating Ipsilateral eyelid oedema Ipsilateral forehead and facial sweating Ipsilateral miosis and ptosis A sense of restlessness or agitation Attack frequency 1 every other day to 8 per day

Cluster Episodic occurs in periods 7 days to 1 year separated by pain free periods lasting one month or more Chronic attacks occur for > 1 year without remission or remission lasting less than a month

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

Other Primary Headaches Primary Stabbing Headache Primary Cough Headache Primary Exertional Headache Primary Headache Associated with sexual Activity Hypnic Headache Hemicrania Continua

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