of migraine: without aura and with aura. The characteristics of
migraine without aura are shown in Box 8.2. In migraine with aura the patient may experience visual symp- toms, such as lines in the vision or loss of vision, sensory symptoms, or dysphasia. The aura lasts between 5 and 60 minutes and is fol- lowed within an hour by a headache fullling the criteria in Box 8.2. Some patients are able to identify particular triggers for their migraine. These may include alcohol, certain foods, alterations in sleep pattern or menstruation. Caution should be exercised in attributing focal neurological abnormalities, e.g. dysphasia or weakness to migraine, unless the patient has previously experienced a number of similar episodes from which they have recovered. In the acute setting migraine is a diagnosis of exclusion. Meningitis The headache in meningitis is not typically of sudden onset but given the dangers of missing the diagnosis it is a condition that should always be considered in the differential diagnosis of a patient presenting with a severe headache. Meningitis may result from bacterial, viral, or less commonly fungal or tuberculous infection. The diagnosis is suggested by the complaint of a headache in association with fever and neck stiffness, which is found in two-thirds of patients with bacterial meningitis. In addition, signs of meningeal irritation (see Table 8.1) should also be sought but may be absent early in the course of the illness. A high index of suspicion therefore needs to be maintained, particularly in the elderly or immunocompromised patient. In contrast, the absence of fever, neck stiffness and change in mental state reliably excludes meningitis. Space-occupying lesions It is uncommon for intracranial mass lesions to present with sudden severe headache. Such lesions only cause pain once they are large enough to cause traction on intracranial vessels or invade sen- sitive structures such as the dura. As a result, such lesions will often present with other features before headache becomes prominent. The headache caused by space-occupying lesions is usually the result of raised intracranial pressure. Typically this headache will be of gradual onset, progressively severe, worse in the morning, and aggravated by activities which raise intracranial pressure, for example coughing or straining. A careful neurological examination may reveal subtle abnormali- ties of which the patient is unaware. The presence of papilloedma supports the diagnosis but its absence does not rule it out. Sudden onset of headache may occur in a patient with a space-occupying lesion if there is haemorrhage into a tumour (see Figure 8.2). Other causes of headache not suggested by the history Tension headache It is estimated that 80% of people will experience a tension head- ache at some time during their life. In contrast to migraine, the headache may last up to 7 days. The key features of tension headache are shown in Box 8.3 and these headaches tend to be precipitated by stress. Table 8.1 Signs of meningeal irritation. Neck stiffness With the patient supine the head is held by the examiner. The neck is passively exed and the amount of resistance noted. The sign is positive when there is objective stiffness, not when the patient reports a subjective feeling of stiffness. Painful inammatory conditions of the pharynx, such as tonsillitis or quinsy may result in a false positive nding. Brudzinskis sign In Brudzinskis sign exion of the hips and knees occurring in response to passive exion of the neck indicates meningeal irritation. Kernigs sign With the patient supine the hip and knee are passively exed to 90 degrees. The knee is then passively extended. The sign is positive when there is resistance to extension. This test usefully distinguishes meningeal irritation from the local cause of neck stiffness described above. Box 8.2 Features of migraine The headache has at least two of the following features: unilateral location pulsating quality moderate or severe intensity aggravation by physical activity At least one of the following occur during the headache: nausea and/or vomiting Photophobia or phonophobia Figure 8.2 CT scan of an intracerebral metastatic neoplasm. 32 ABC of Emergency Differential Diagnosis Differentiating between migraine and tension headaches can be difcult and the two may coexist in the same patient. The non- pulsating quality and lack of aggravation by physical activity may usefully distinguish between the two. However, in the acute setting tension headache is not usually included in the differential diagno- sis of acute severe headache as it is rarely of sudden onset. Systemic illness Severe infections such as pneumonia and pyelonephritis are a fre- quent cause of headache. Diagnosis in such patients is rarely dif- cult. As the headache is not of sudden onset, a careful history and examination will identify the cause. Dental and ENT disease Pain in the head or face is a key feature in many dental and ENT problems, e.g. sinusitis. Although the diagnosis may be appar- ent from the history, inspection of the mouth, pharynx and tympanic membranes is required to exclude these as possible sources of pain. Temporal arteritis Temporal arteritis typically presents with gradual onset of a con- stant band-like headache and tenderness over the temporal arteries. It may also be associated with visual loss. Typically, patients will be over 55 years of age and feel generally unwell. The erythrocyte sedi- mentation rate is usually markedly raised. Urgent treatment with corticosteroids reduces the risk of visual loss. Case history revisited The history outlined by this woman suggests a number of possible diagnoses. The patient has a history of migraine, but this headache is clearly different to her usual migraine headache, so this is an unlikely cause. The history of breast cancer raises the possibility of a cerebral metastasis. Given the sudden onset of the headache and its severity, haemorrhage into a metastasis or into the subarachnoid space should be considered. Her history of fever preceding the headache suggests that menin- gitis should be considered although the sudden onset of the head- ache makes this diagnosis less likely. Examination On examination the patients observations are normal. Her temperature is 36.5C, blood pressure 107/60 mmHg and pulse rate 90 beats/minute. Box 8.3 Features of tension headache Headache has at least two of the following characteristics: bilateral location pressing/tightening (non-pulsating) quality mild or moderate intensity not aggravated by routine physical activity such as walking or climbing stairs And both of the following: no nausea or vomiting (anorexia may occur) no more than one of photophobia or phonophobia (a) (b) Figure 8.3 Intracranial aneurysm before (a) and after coiling (b). Acute Headache 33 Her neck was stiff but there is no other abnormality on neurological examination. Examination of the fundi and tympanic membranes is normal and there is no rash. Question: Given the history and examination ndings what is your principal working diagnosis? Principal working diagnosis subarachnoid haemorrhage Management The sudden onset of headache and the nding of neck stiffness suggest subarachnoid haemorrhage is the cause of her symptoms. The next step will be to obtain a CT scan. In patients with sub- arachnoid haemorrhage this will be abnormal in around 95% of cases if the scan is performed within the rst 24 hours, but only in 50% 1 week after onset of the headache. CT should also reliably exclude the possibility of a space-occupying lesion. If the CT scan is normal a lumbar puncture should be per- formed. When investigating possible subarachnoid haemorrhage this should be delayed until 12 hours after onset of the headache, when its sensitivity will be maximal. If subarachnoid haemorrhage is not found, examination of the cerebrospinal uid (CSF) should also identify cases of meningitis. In addition, the measurement of CSF pressure may also identify some uncommon diagnoses that might otherwise be missed, such as venous sinus thrombosis or spontaneous intracranial hypotension. Outcome The patient underwent a CT scan which demonstrated a subarach- noid haemorrhage (see Figure 8.3a). She was referred urgently to a neurosurgeon for further treatment. The causative aneurysm was subsequently coiled (see Figure 8.3b). Further reading Dowson AJ. Your Questions Answered: Migraine and Other Headaches. Churchill Livingstone, Edinburgh, 2003. Lance JW, Goadsby PJ. Mechanism and Management of Headache, Seventh Edition. Elsevier, New York, 2005.