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REVIEW
A J Larner
Consultant Neurologist, Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley, Liverpool L9 7LJ, UK; a.larner@thewaltoncentre.nhs.uk 10.1136/jnnp.2008.171140
in PaCO2 and cerebral vasodilatation,1 and possibly increased brain metabolism during REM (rapid eye movement) sleep.2 Such headaches are almost invariably associated with papilloedema, and sometimes with vomiting which may lead to hyperventilation and reduction of ICP. In the UK, headache with vomiting and papilloedema is enshrined in Department of Health guidelines for urgent evaluation (the two-week rule), although in practice very few patients
Larner 81 referred under these guidelines have such features, or indeed cerebral tumours.3 In fact, any patient with all three features should be seen immediately, certainly not wait for even two weeks, because they may very well have raised ICP. So, are all morning headaches due to brain tumours with raised ICP? Clearly not, but neurologists are often referred patients with a history of nocturnal and/or awakening headachesquery raised ICP, in the apparent absence of other neurological symptoms and signs. The differential diagnosis is in fact quite broad (see box), encompassing not only intracranial hypertension but also a number of primary and secondary headache disorders, as well as general neurological, medical and psychiatric conditions.
and tearing (SUNCT).7 In both these conditions, attacks may occur throughout the 24-hour period.
Hemicrania continua
The precise nosological position of hemicrania continua is still debated. Although not
82 Practical Neurology currently classified with the trigeminal autonomic cephalalgias in the International Headache Society schema (ICHD2),8 it has certain features in common with them, including autonomic manifestations and indomethacin responsiveness as seen in paroxysmal hemicrania. Pain is, by definition, daily and continuous, but exacerbations may occur and these frequently awaken the patient from sleep. domestic and occupational reasons, be restricted to certain times of the day, or more precisely, night.
Hypnic headache
A defining characteristic of this rare primary headache disorder is onset during sleep, usually at a consistent time each night, between 01:00 h and 03:00 h, hence it too has sometimes been known as alarm clock headache (cluster headache is another alarm clock headache, see above). Recurrent attacks of headache occur, often in the middle or later stages of sleep, possibly emerging during REM sleep.9 The pathogenesis remains unknown but may be related to impaired inactivation of anti-nociceptive brain structures, such as the locus coeruleus which is normally inactivated during REM sleep. Differentiating factors from cluster headache include frequently bilateral headache, the absence of autonomic features, onset in later life, and female preponderance.10
Hangover headache
Diagnosis of hangover headache (delayed alcohol-induced headache is the preferred ICHD2 terminology8) should be obvious from the history.
Sphenoid sinusitis
Headache is the most common symptom of acute sphenoid sinusitis (or rhinosinusitis, as in the ICHD2 classification,8 as sinusitis in the absence of rhinitis is uncommon) often interfering with sleep. It is rare, particularly in isolation, and misdiagnosis common. The pain is severe, intractable, not specifically localised, sometimes aggravated by bending or coughing, not relieved by simple analgesics, and may be associated with facial pain. Diagnosis may be difficult because not all patients have pyrexia or purulent nasal discharge.14 As the condition has potential morbidity and mortality, it is very important not to miss it. Diagnostic investigations include CT, MRI and fibreoptic nasal endoscopy.
Larner 83
(and possibly right) temporal region. Before her follow-up appointment was due, the patient was found dead in bed one morning, perhaps as the consequence of a seizure. Other clues to the diagnosis of nocturnal seizures are waking up on the floor, and dishevelled bedclothes.
Subarachnoid haemorrhage
Onset of subarachnoid haemorrhage during sleep is extremely rare, if it occurs at all. The history here is critical, particularly whether the patient was actually asleep, because headache secondary to sexual activity enters the differential. Stroke apparent on awakening is more likely to be ischaemic than haemorrhagic.16
Depression
Early morning waking is one of the classic vegetative symptoms of depression, often in association with depressive thinking. Although headache is not a feature of depressive disorders as enshrined in the Diagnostic and Statistical Manual (DSM-IV) criteria, it should not be forgotten that this symptom is not uncommon in clinical practice. Moreover, mood disorders can complicate migraine and possibly chronic tension type headache, and hence need to be identified and treated in their own right.
PRACTICE POINTS
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Patients with headaches which wake them during the night or are present on waking and improve after getting up, and who have associated papilloedema and vomiting, require immediate assessment because they may have raised intracranial pressure. Many other and more common conditions may also be associated with nocturnal or early morning headaches. The chronobiology of many primary headache disorders has a circadian pattern with preferential headache timing during the night or early morning, including migraine, cluster headache and hypnic headache. Medication overuse headache is a common cause of nocturnal headache, with the patient waking to consume more analgesia. Non-headache disorders such as depression and nocturnal epileptic seizures also enter the differential diagnosis.
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84 Practical Neurology Headache patients with depression (and anxiety) have greater functional impairment than those without.20
4. 5. Fox AW, Davis RL. Migraine chronobiology. Headache 1998;38:43641. Bahra A, May A, Goadsby PJ. Cluster headache: a prospective clinical study with diagnostic implications. Neurology 2002;58:35461. Kayed K, Godtlibsen OB, Sjaastad O. Chronic paroxysmal hemicrania IV: REM sleep locked nocturnal attacks. Sleep 1978;1:915. Cohen AS, Matharu MS, Goadsby PJ. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA): a prospective clinical study of SUNCT and SUNA. Brain 2006;129:274660. International Headache Society Classification Subcommittee. The international classification of headache disorders. Second edn. Cephalalgia 2004;24(Suppl 1):1160. Dodick DW, Mosek AC, Campbell JK. The hypnic (alarm clock) headache syndrome. Cephalalgia 1998;18:1526. Evers S, Goadsby PJ. Hypnic headache. Neurology 2003;60:9059. Frese A, Eikermann A, Frese K, et al. Headache associated with sexual activity. Demography, clinical features, and comorbidity. Neurology 2003;61:796800. Larner AJ. Late presentation of primary headache associated with sexual activity: is non-invasive angiography worthwhile? J Headache Pain 2006;7:13940. British Association for the Study of Headache. Guidelines for all healthcare professionals in the diagnosis and management of migraine, tensiontype, cluster and medication-overuse headache. BASH: London, 2007:17. Lew D, Southwick FS, Montgomery WW, et al. Sphenoid sinusitis: a review of 30 cases. N Engl J Med 1983;319:114954. Hampson NB, Hampson LA. Characteristics of headache associated with acute carbon monoxide poisoning. Headache 2002;42:2203. Wroe SJ, Sandercock P, Bamford J, et al. Diurnal variation in incidence of stroke: Oxfordshire community stroke project. BMJ 1992;304:1557. Larner AJ. Obstructive sleep apnoea syndrome presenting in a neurology outpatient clinic. Int J Clin Pract 2003;57:1502. Stradling JR. Sleep apnoea syndromes. In: Brewis RAL, Corrin B, Geddes DM, Gibson GJ, eds. Respiratory medicine. Second edn. WB Saunders: London, 1995:9731005. Rains JC, Poceta JS. Sleep-related headache syndromes. Semin Neurol 2005;25:6980. Page LA, Howard LM, Husain K, et al. Psychiatric morbidity and cognitive representations of illness in chronic daily headache. J Psychosom Res 2004;57:54955. Pearce JM. Clinical features of the exploding head syndrome. J Neurol Neurosurg Psychiatry 1989;52:90710. Wall M. The headache profile of idiopathic intracranial hypertension. Cephalalgia 1990;10:3315. Davenport R. Headache. Pract Neurol 2008;8:33543.
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CONCLUSIONS
Although raised ICP is the most alarming possible cause of nocturnal and/or awakening headaches, the sensitivity and specificity of this symptom for the diagnosis of intracranial hypertension has not, to my knowledge, been systematically evaluated. ICHD2 lists only two categories in which headache worse in the morning is included among the diagnostic criteria, namely Headache attributed directly to neoplasm and Headache attributed to intracranial hypertension secondary to hydrocephalus.8 However, morning headaches may occur with other causes of raised ICP for example, the headache of idiopathic intracranial hypertension may awaken the patient at night.22 Nocturnal and/or awakening headache is clearly not a pathognomonic symptom for raised ICP, far from it. Many other headache disorders, some very common such as migraine, as well as other neurological and medical conditions enter the differential diagnosis. History taking is key to identifying them,23 and hence determining the most appropriate pathway for investigation (if any) and management.
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ACKNOWLEDGEMENTS
This article was reviewed by David HiltonJones, Oxford, UK.
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REFERENCES
1. 2. North B, Reilly P. Raised intracranial pressure. A clinical guide. Oxford: Heinemann, 1990:268. Cooper R, Hulme A. Intracranial pressure and related phenomena during sleep. J Neurol Neurosurg Psychiatry 1966;29:56470. Abernethy Holland AJ, Larner AJ. Central nervous system/brain tumour 2-week referral guidelines: prospective 3-year audit. Clin Oncol 2008;20:2012. 21.
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10.1136/jnnp.2008.171140