The International Classification of Headache Disorders 2nd Edition



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4. Other primary headaches


4.1 Primary stabbing headache

4.2 Primary cough headache

4.3 Primary exertional headache

4.4 Primary headache associated with sexual activity

4.4.1 Preorgasmic headache

4.4.2 Orgasmic headache

4.5 Hypnic headache

4.6 Primary thunderclap headache

4.7 Hemicrania continua

4.8 New daily-persistent headache (NDPH)



General comment

Primary or secondary headache or both?

When a new headache occurs for the first time in close temporal relation to another disorder that is a known cause of headache, this headache is coded according to the causative disorder as a secondary headache. This is also true if the headache has the characteristics of migraine or other primary headache. When a pre-existing primary headache is made worse in close temporal relation to another disorder that is a known cause of headache, there are two possibilities, and judgment is required. The patient can either be given only the diagnosis of the pre-existing primary headache or be given both this diagnosis and a secondary headache diagnosis according to the other disorder. Factors that support adding the latter diagnosis are: a very close temporal relation to the disorder, a marked worsening of the pre-existing headache, very good evidence that the disorder can cause or aggravate the primary headache and, finally, improvement or resolution of the primary headache after relief from the disorder.

Introduction


This chapter includes headaches that are clinically heterogeneous. The pathogenesis of these types of headache is still poorly understood, and their treatment is suggested on the basis of anecdotal reports or uncontrolled trials.

Several headache disorders included in this chapter can be symptomatic and need careful evaluation by imaging and/or other appropriate tests.

The onset of some of these headaches, 4.6 Primary thunderclap headache especially, can be acute and affected patients are usually assessed in Emergency Departments. Appropriate and full investigation (neuroimaging, in particular) is mandatory in these cases.

The chapter also includes some clinical entities, such as 4.1 Primary stabbing headache and 4.5 Hypnic headache (this latter recently described), that are primary in most cases.


4.1 Primary stabbing headache

Previously used terms:

Ice-pick pains, jabs and jolts, ophthalmodynia periodica
Description:

Transient and localised stabs of pain in the head that occur spontaneously in the absence of organic disease of underlying structures or of the cranial nerves.
Diagnostic criteria:

A. Head pain occurring as a single stab or a series of stabs and fulfilling criteria B-D

  1. Exclusively or predominantly felt in the distribution of the first division of the trigeminal nerve (orbit, temple and parietal area)

  2. Stabs last for up to a few seconds and recur with irregular frequency ranging from one to many per day

  3. No accompanying symptoms

  4. Not attributed to another disorder1



Note:

1. History and physical and neurological examinations do not suggest any of the disorders listed in groups 5-12, or history and/or physical and/or neurological examinations do suggest such disorder but it is ruled out by appropriate investigations, or such disorder is present but pain does not occur for the first time in close temporal relation to the disorder.
Comments:

In a single published descriptive study, 80% of stabs lasted 3 seconds or less. In rare cases, stabs occur repetitively over days, and there has been one description of status lasting one week.

Stabs may move from one area to another in either the same or the opposite hemicranium. When they are strictly localised to one area, structural changes at this site and in the distribution of the affected cranial nerve must be excluded.

Stabbing pains are more commonly experienced by people subject to migraine (about 40%) or cluster headache (about 30%), in which cases they are felt in the site habitually affected by these headaches.

A positive response to indomethacin has been reported in some uncontrolled studies, whilst others have observed partial or no responses.


4.2 Primary cough headache

Previously used terms:

Benign cough headache, Valsalva-manoeuvre headache
Description:

Headache precipitated by coughing or straining in the absence of any intracranial disorder.
Diagnostic criteria:

  1. Headache fulfilling criteria B and C

  2. Sudden onset, lasting from one second to 30 minutes

  3. Brought on by and occurring only in association with coughing, straining and/or Valsalva manoeuvre

  4. Not attributed to another disorder1



Note:

1. Cough headache is symptomatic in about 40% of cases and the large majority of these present Arnold-Chiari malformation type I. Other reported causes of symptomatic cough headache include carotid or vertebrobasilar diseases and cerebral aneurysms. Diagnostic neuroimaging plays an important role in differentiating secondary cough headache from 4.2 Primary cough headache.
Comment:

Primary cough headache is usually bilateral and predominantly affects patients older than 40 years of age. Whilst indomethacin is usually effective in the treatment of primary cough headache, a positive response to this medication has also been reported in some symptomatic cases.

4.3 Primary exertional headache

Previously used terms:

Benign exertional headache
Coded elsewhere:

Exercise-induced migraine is coded under 1. Migraine according to its subtype.
Description:

Headache precipitated by any form of exercise. Subforms such as “weight-lifters’ headache” are recognised.
Diagnostic criteria:

  1. Pulsating headache fulfilling criteria B and C

  2. Lasting from 5 minutes to 48 hours

  3. Brought on by and occurring only during or after physical exertion

  4. Not attributed to another disorder1



Note:

1. On first occurrence of this headache type it is mandatory to exclude subarachnoid haemorrhage and arterial dissection.
Comments:

Primary exertional headache occurs particularly in hot weather or at high altitude. There are reports of prevention in some patients by the ingestion of ergotamine tartrate. Indomethacin has been found effective in the majority of the cases.

Headache described in weight-lifters has been considered a subform of 4.3 Primary exertional headache; because of its sudden onset and presumed mechanism it may have more similarities to 4.2 Primary cough headache.


4.4 Primary headache associated with sexual activity

Previously used terms:

Benign sex headache, coital cephalalgia, benign vascular sexual headache, sexual headache
Description:

Headache precipitated by sexual activity, usually starting as a dull bilateral ache as sexual excitement increases and suddenly becoming intense at orgasm, in the absence of any intracranial disorder.

4.4.1 Preorgasmic headache

Diagnostic criteria:

  1. Dull ache in the head and neck associated with awareness of neck and/or jaw muscle contraction and meeting criterion B

  2. Occurs during sexual activity and increases with sexual excitement

  3. Not attributed to another disorder



4.4.2 Orgasmic headache

Coded elsewhere:

Postural headache resembling that of low CSF pressure has been reported to develop after coitus. Such headache should be coded as 7.2.3 Headache attributed to spontaneous (or idiopathic) low CSF pressure because it is due to CSF leakage.
Diagnostic criteria:

  1. Sudden severe (“explosive”) headache meeting criterion B

  2. Occurs at orgasm

  3. Not attributed to another disorder1



Note:

1. On first onset of orgasmic headache it is mandatory to exclude conditions such as subarachnoid haemorrhage and arterial dissection.
Comments:

An association between 4.4 Primary headache associated with sexual activity, 4.3 Primary exertional headache and migraine is reported in approximately 50% of cases.

Two subtypes (dull type and explosive type headache) were included in the first edition of The International Classification of Headache Disorders. No specific investigation has been undertaken since then to clarify whether they are separate entities. In most published reports of headache with sexual activity, only explosive (“vascular type”) headache has been reported. The dull type may be a subtype of tension-type headache, but no evidence supports this hypothesis.

No firm data are available on the duration of primary headache associated with sexual activity, but it is usually considered to last from 1 minute to 3 hours.

4.5 Hypnic headache

Previously used terms:

Hypnic headache syndrome, “alarm clock” headache
Description:

Attacks of dull headache that always awaken the patient from asleep.
Diagnostic criteria:

A. Dull headache fulfilling criteria B-D

B. Develops only during sleep, and awakens patient

C. At least two of the following characteristics:

1. occurs >15 times per month

2. lasts 15 minutes after waking

3. first occurs after age of 50 years

D. No autonomic symptoms and no more than one of nausea, photophobia or phonophobia

E. Not attributed to another disorder1



Note:

1. Intracranial disorders must be excluded. Distinction from one of the trigeminal autonomic cephalalgias is necessary for effective management.
Comments:

The pain of hypnic headache is usually mild to moderate, but severe pain is reported by approximately 20% of patients. Pain is bilateral in about two-thirds of cases. The attack usually lasts from 15 to 180 minutes, but longer durations have been described.

Caffeine and lithium have been effective treatments in several reported cases.


4.6 Primary thunderclap headache

Previously used terms:

Benign thunderclap headache
Coded elsewhere:

4.2 Primary cough headache, 4.3 Primary exertional headache and 4.4 Primary headache associated with sexual activity can all present as thunderclap headache but should be coded as those headache types, not as 4.6 Primary thunderclap headache.
Description:

High-intensity headache of abrupt onset mimicking that of ruptured cerebral aneurysm.
Diagnostic criteria:

  1. Severe head pain fulfilling criteria B and C

  2. Both of the following characteristics:

1. sudden onset, reaching maximum intensity in <1 minute

2. lasting from 1 hour to 10 days



  1. Does not recur regularly over subsequent weeks or months1

  2. Not attributed to another disorder2



Notes:

1. Headache may recur within the first week after onset.

2. Normal CSF and normal brain imaging are required.


Comment:

Evidence that thunderclap headache exists as a primary condition is poor: the search for an underlying cause should be expedient and exhaustive. Thunderclap headache is frequently associated with serious vascular intracranial disorders, particularly subarachnoid haemorrhage: it is mandatory to exclude this and a range of other such conditions including intracerebral haemorrhage, cerebral venous thrombosis, unruptured vascular malformation (mostly aneurysm), arterial dissection (intra- and extracranial), CNS angiitis, reversible benign CNS angiopathy and pituitary apoplexy. Other organic causes of thunderclap headache are colloid cyst of the third ventricle, CSF hypotension and acute sinusitis (particularly with barotrauma). 4.6 Primary thunderclap headache should be the diagnosis only when all organic causes have been excluded.

4.7 Hemicrania continua

Description:

Persistent strictly unilateral headache responsive to indomethacin.
Diagnostic criteria:

A. Headache for >3 months fulfilling criteria B-D

B All of the following characteristics:

1. unilateral pain without side-shift

2. daily and continuous, without pain-free periods

3. moderate intensity, but with exacerbations of severe pain

C. At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side of pain:

1. conjunctival injection and/or lacrimation

2. nasal congestion and/or rhinorrhoea

3. ptosis and/or miosis

D. Complete response to therapeutic doses of indomethacin

E. Not attributed to another disorder1

Note:

1. History and physical and neurological examinations do not suggest any of the disorders listed in groups 5-12, or history and/or physical and/or neurological examinations do suggest such disorder but it is ruled out by appropriate investigations, or such disorder is present but headache does not occur for the first time in close temporal relation to the disorder.
Comment:

Hemicrania continua is usually unremitting, but rare cases of remission are reported. Whether this headache type can be subdivided according to length of history and persistence is yet to be determined.

4.8 New daily-persistent headache (NDPH)

Previously used terms:

De novo chronic headache; chronic headache with acute onset
Description:

Headache that is daily and unremitting from very soon after onset (within 3 days at most). The pain is typically bilateral, pressing or tightening in quality and of mild to moderate intensity. There may be photophobia, phonophobia or mild nausea.
Diagnostic criteria:

A. Headache that, within 3 days of onset1, fulfils criteria B-D

B. Headache is present daily, and is unremitting, for >3 months

C. At least two of the following pain characteristics:

1. bilateral location

2. pressing/tightening (non-pulsating) quality

3. mild or moderate intensity

4. not aggravated by routine physical activity such as walking or climbing

D. Both of the following:

1. no more than one of photophobia, phonophobia or mild nausea

2. neither moderate or severe nausea nor vomiting

E. Not attributed to another disorder2

Note:

1. Headache may be unremitting from the moment of onset or very rapidly build up to continuous and unremitting pain. Such onset or rapid development must be clearly recalled and unambiguously described by the patient. Otherwise code as 2.3 Chronic tension-type headache.

2. History and physical and neurological examinations do not suggest any of the disorders listed in groups 5-12 (including 8.2 Medication-overuse headache and its subforms), or history and/or physical and/or neurological examinations do suggest such disorder but it is ruled out by appropriate investigations, or such disorder is present but headache does not occur for the first time in close temporal relation to the disorder.


Comments:

This second edition of the classification recognises 4.8 New daily-persistent headache as a separate entity from 2.3 Chronic tension-type headache. Although it has many similarities to tension-type headache, NDPH is unique in that headache is daily and unremitting from or almost from the moment of onset, typically in individuals without a prior headache history. A clear recall of such an onset is necessary for the diagnosis of 4.8 New daily-persistent headache.

The headache of NDPH can have associated features suggestive of either migraine or tension-type headache. Secondary headaches such as low CSF volume headache, raised CSF pressure headache, post-traumatic headache and headache attributed to infection (particularly viral) should be ruled out by appropriate investigations.

If there is or has been within the last 2 months medication overuse fulfilling criterion B for any of the subforms of 8.2 Medication-overuse headache, the rule is to code for any pre-existing primary headache plus 8.2.78.2.8 Probable medication-overuse headache but not for 4.8 New daily-persistent headache.

NDPH may take either of two subforms: a self-limiting subform which typically resolves without therapy within several months and a refractory subform which is resistant to aggressive treatment programmes. The subcommittee aims to stimulate further clinical characterisation and pathophysiological research of this syndrome, especially studies comparing 4.8 New daily-persistent headache with 2.3 Chronic tension-type headache.


Bibliography

4.1 Primary stabbing headache

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Dangond F, Spierings EL. Idiopathic stabbing headaches lasting a few seconds. Headache 1993; 33: 257-8.

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4.2 Primary cough headache

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Pascual J, Iglesias F, Oterino A, Vazquez-Barquero A, Berciano J. Cough, exertional, and sexual headaches: an analysis of 72 benign and symptomatic cases. Neurology 1996; 46: 1520-4.

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Smith WS, Messing RO. Cerebral aneurysm presenting as cough headache. Headache 1993; 33: 203-4.

4.3 Primary exertional headache

Edis RH, Silbert PL. Sequential benign sexual headache and exertional headache (letter). Lancet 1988; 30: 993.

Green MW. A spectrum of exertional headaches. Headache 2001; 4: 1085-92.

Heckmann JG, Hilz MJ, Muck-Weymann M, Neundorfer B. Benign exertional headache/benign sexual headache: a disorder of myogenic cerebrovascular autoregulation? Headache 1997; 37: 597-8.

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Lipton RB, Lowenkopf T, Bajwa ZH, Leckie RS, Ribeiro S, Newman LC, Greenberg MA. Cardiac cephalgia: a treatable form of exertional headache. Neurology 1997; 49: 813-6.

Pascual J, Iglesias F, Oterino A, Vazquez-Barquero A, Berciano J. Cough, exertional, and sexual headaches: an analysis of 72 benign and symptomatic cases. Neurology 1996; 46: 1520-4.

Silbert PL, Edis RH, Stewart-Wynne EG, Gubbay SS. Benign vascular sexual headache and exertional headache: interrelationships and long term prognosis. J Neurol Neurosurg Psychiatry 1991; 54: 417-21.

4.4 Primary headache associated with sexual activity

D’Andrea G, Granella F, Verdelli F. Migraine with aura triggered by orgasm. Cephalalgia 2002; 22: 485-6.

Jacome DE. Masturbatory-orgasmic extracephalic pain. Headache 1998; 38: 138-41.

Kumar KL, Reuler JB. Uncommon headaches: diagnosis and treatment. J Gen Int Med 1993; 8: 333-41.

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Pascual J, Iglesias F, Oterino A, Vazquez-Barquero A, Berciano J. Cough, exertional, and sexual headaches: an analysis of 72 benign and symptomatic cases. Neurology 1996; 46: 1520-4.

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4.5 Hypnic headache

Arjona JA, Jimenez-Jimenez FJ, Vela-Bueno A, Tallon-Barranco A. Hypnic headache associated with stage 3 slow wave sleep. Headache 2000; 40: 753-4.

Bruni O, Galli F, Guidetti V. Sleep hygiene and migraine in children and adolescents. Cephalalgia 1999; 19 (Suppl 25): 57-9.

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Dodick DW. Polysomnography in hypnic headache syndrome. Headache 2000; 40: 748-52.

Dodick DW, Jones JM, Capobianco DJ. Hypnic headache: another indomethacin-responsive headache syndrome? Headache 2000; 40: 830-5.

Dodick DW, Mosek AC, Campbell IK. The hypnic (“alarm clock”) headache syndrome. Cephalalgia 1998; 18: 152-6.

Ghiotto N, Sances G, Di Lorenzo G, Trucco M, Loi M, Sandrini G, Nappi G. Report of eight new cases of hypnic headache and a mini-review of the literature. Funct Neurol 2002; 17: 211-9.

Gould JD, Silberstein SD. Unilateral hypnic headache: a case study. Neurology 1997; 49: 1749-51.

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Martins IP, Gouveia RG. Hypnic headache and travel across time zones: a case report. Cephalalgia 2001; 21: 928-31.

Morales-Asin F, Mauri JA, Iniguez C, Espada F, Mostacero E. The hypnic headache syndrome: report of three new cases. Cephalalgia 1998; 18: 157-8.

Newman LC, Lipton RB, Solomon S. The hypnic headache syndrome: a benign headache disorder of the elderly. Neurology 1990; 40: 1904-5.

Raskin NH. The hypnic headache syndrome. Headache 1988; 28: 534-6.

Ravishankar K. Hypnic headache syndrome. Cephalalgia 1998; 18: 358-9.



4.6 Primary thunderclap headache

Bassi P, Bandera R, Loiero M, Tognoni G, Mangoni A. Warning signs in subarachnoid hemorrhage: a cooperative study. Acta Neurol Scand 1991; 84: 277-81.

Dodick DW, Brown RD, Britton JW, Huston J. Nonaneurysmal thunderclap headache with diffuse, multifocal, segmental and reversible vasospasm. Cephalalgia 1999; 19: 118-23.

Garg RK. Recurrent thunderclap headache associated with reversible vasospasm causing stroke. Cephalalgia 2001; 21: 78-9.

Landtblom AM, Fridriksson S, Boivie J, Hillman J, Johansson G, Johansson I. Sudden onset headache: a prospective study of features incidence and causes. Cephalalgia 2002; 22: 354-60.

Linn FHH, Rinkel GJE, Algra A, van Gijn J. Headache characteristics in subarachnoid haemorrhage and benign thunderclap headache. J Neurol Neurosurg Psychiat 1998; 65: 791-3.

Linn FHH, Rinkel GJE, Algra A, van Gijn J. Follow-up of idiopathic thunderclap headache in general practice. J Neurol 1999; 246: 946-8.

Linn FHH, Wijdicks EFM. Causes and management of thunderclap headache: a comprehensive review. The Neurologist 2002; 8: 279-89.

Markus HS. A prospective follow-up of thunderclap headache mimicking subarachnoid haemorrhage. J Neurol Neurosurg Psychiat 1991; 54: 1117-25.

Mauriño J, Saposnik G, Lepera S, Rey RC, Sica RE. Multiple simultaneous intracerebral haemorrhages. Arch Neurol 2001; 58: 629-32.

Nowak DA, Rodiek SO, Henneken S, Zinner J, Schreiner R, Fuchs H-H, Topka H. Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option? Cephalalgia 2003; 23: 218-222.

Sturm JW, Macdonell RAL. Recurrent thunderclap headache associated with reversible intracerebral vasospasm causing stroke. Cephalalgia 2000; 20: 132-5.

Slivka A, Philbrook B. Clinical and angiographic features of thunderclap headache. Headache 1995; 35: 1-6.

Wijdicks EFM, Kerkhoff H, van Gjin J. Cerebral vasospasm and unruptured aneurysm in thunderclap headache. Lancet 1988; 2: 1020.

Witham TF, Kaufmann AM. Unruptured cerebral aneurysm producing a thunderclap headache. Am J Emergency Med 2000; 1: 88-90.



4.7 Hemicrania continua

Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua: anaesthetic blockades of pericranial nerves. Funct Neurol 1997; 1: 11-5.

Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua. Parenteral indomethacin: the “Indotest”. Headache 1998; 8: 235-6.

Bordini C, Antonaci F, Stovner LJ, Schrader H, Sjaastad O. “Hemicrania continua”: a clinical review. Headache 1991; 31: 20-26.

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Pareja J, Antonaci F, Vincent M. The hemicrania continua diagnosis. Cepahalalgia 2002; 7: 563-4.

Pareja J, Vincent M, Antonaci F, Sjaastad O. Hemicrania continua: diagnostic criteria and nosologic status. Cepahalalgia 2001; 9: 874-7.

Sjaastad O, Antonaci F. Chronic paroxysmal hemicrania (CPH) and hemicrania continua: transition from one stage to another. Headache 1993; 33: 551-4.

Sjaastad O, Antonaci F. A piroxicam derivative partly effective in chronic paroxysmal hemicrania and hemicrania continua. Headache 1995; 35: 549-50.

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4.8 New daily-persistent headache

Evans RW, Rozen TD. Etiology and treatment of new daily persistent headache. Headache 2001;41(8):830-2.

Goadsby PJ, Boes C. New daily persistent headache. J Neurol Neurosurg Psychiat 2002;72 Suppl 2:ii6-ii9.

Li D, Rozen TD. The clinical characterisation of new daily persistent headache. Cephalalgia 2002;22;66-9.

Silberstein SD, Lipton RB, Solomon S, Mathew NT. Classification of daily and near daily headaches: proposed revisions to the IHS-criteria. Headache 1994;34:1-7.



PART TWO



The Secondary Headaches

Headache attributed to head and neck trauma

Headache attributed to cranial or cervical vascular disorder

Headache attributed to non-vascular intracranial disorder and other causes

Headache attributed to substances or their withdrawal

Headache attributed to infection

Headache attributed to disturbance of homeostasis

Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures

Headache attributed to psychiatric disorder

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