The International Classification of Headache Disorders 2nd Edition


CLUSTER HEADACHE AND OTHER TRIGEMINAL AUTONOMIC CEPHALALGIAS



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3. CLUSTER HEADACHE AND OTHER TRIGEMINAL AUTONOMIC CEPHALALGIAS


3.1 Cluster headache

3.1.1 Episodic cluster headache

3.1.2 Chronic cluster headache

3.2 Paroxysmal hemicrania

3.2.1 Episodic paroxysmal hemicrania

3.2.2 Chronic paroxysmal hemicrania (CPH)

3.3 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)

3.4 Probable trigeminal autonomic cephalalgia

3.4.1 Probable cluster headache

3.4.2 Probable paroxysmal hemicrania

3.4.3 Probable SUNCT

Coded elsewhere:

4.7 Hemicrania continua

General comment

Primary or secondary headache or both?

When a headache with the characteristics of a trigeminal autonomic cephalalgia (TAC) occurs for the first time in close temporal relation to another disorder that is a known cause of headache, it is coded according to the causative disorder as a secondary headache. When a pre-existing TAC 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 TAC diagnosis or be given both the TAC 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 TAC, very good evidence that the disorder can cause or aggravate the TAC and, finally, improvement or resolution of the TAC after relief from the disorder.

Introduction


The trigeminal autonomic cephalalgias share the clinical features of headache and prominent cranial parasympathetic autonomic features. Experimental and human functional imaging suggests that these syndromes activate a normal human trigeminal-parasympathetic reflex with clinical signs of cranial sympathetic dysfunction being secondary.

Hemicrania continua, whose cranial autonomic features are less constant, is to be found under 4. Other primary headaches.

3.1 Cluster headache

Previously used terms:

Ciliary neuralgia, erythro-melalgia of the head, erythroprosopalgia of Bing, hemicrania angioparalytica, hemicrania neuralgiformis chronica, histaminic cephalalgia, Horton’s headache, Harris-Horton’s disease, migrainous neuralgia (of Harris), petrosal neuralgia (of Gardner), Sluder’s neuralgia, spheno-palatine neuralgia, vidian neuralgia
Coded elsewhere:

Symptomatic cluster headache is coded to the underlying causative disorder.
Description:

Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15-180 minutes and occurring from once every other day to 8 times a day. The attacks are associated with one or more of the following, all of which are ipsilateral: conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis, eyelid oedema. Most patients are restless or agitated during an attack.
Diagnostic criteria:

A. At least 5 attacks fulfilling criteria B-D

B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes if untreated1

C. Headache is accompanied by at least one of the following:


  1. 1. ipsilateral conjunctival injection and/or lacrimation

2. ipsilateral nasal congestion and/or rhinorrhoea

3. ipsilateral eyelid oedema

4. ipsilateral forehead and facial sweating

5. ipsilateral miosis and/or ptosis

6. a sense of restlessness or agitation

D. Attacks have a frequency from one every other day to 8 per day2

E. Not attributed to another disorder3

Notes:

1. During part (but less than half) of the time-course of cluster headache, attacks may be less severe and/or of shorter or longer duration.

2. During part (but less than half) of the time-course of cluster headache, attacks may be less frequent.

3. 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 attacks do not occur for the first time in close temporal relation to the disorder.

Comments:

Acute attacks involve activation of the posterior hypothalamic grey matter. Cluster headache may be inherited (autosomal dominant) in about 5% of cases.

Attacks usually occur in series (cluster periods) lasting for weeks or months separated by remission periods usually lasting months or years. However, about 10-15% of patients have chronic symptoms without remissions.

In a large series with good follow-up, 27% of patients had only a single cluster period. These should be coded as 3.1 Cluster headache.

During a cluster period, and in the chronic subtype, attacks occur regularly and may be provoked by alcohol, histamine or nitroglycerine. Pain is maximal orbitally, supraorbitally, temporally or in any combination of these sites, but may spread to other regions of the head. Pain almost invariably recurs on the same side during an individual cluster period. During the worst attacks, the intensity of pain is excruciating. Patients are usually unable to lie down and characteristically pace the floor.

Age at onset is usually 20-40 years. For unknown reasons prevalence is 3-4 times higher in men than in women.

Cluster headache with coexistent trigeminal neuralgia (cluster-tic syndrome):

Some patients have been described who have both 3.1 Cluster headache and 13.1 Trigeminal neuralgia. They should receive both diagnoses. The importance of this observation is that both conditions must be treated for the patient to be headache free.

3.1.1 Episodic cluster headache

Description:

Cluster headache attacks occurring in periods lasting 7 days to 1 year separated by pain-free periods lasting 1 month or longer.
Diagnostic criteria:

A. Attacks fulfilling criteria A-E for 3.1 Cluster headache

B. At least two cluster periods lasting 7-365 days1 and separated by pain-free remission periods of 1 month



Note:

1. Cluster periods usually last between 2 weeks and 3 months.
Comment:

The duration of the remission period has been increased in this second edition to a minimum of 1 month.

3.1.2 Chronic cluster headache

Description:

Cluster headache attacks occurring for more than 1 year without remission or with remissions lasting less than 1 month.
Diagnostic criteria:

A. Attacks fulfilling criteria A-E for 3.1 Cluster headache

B. Attacks recur over >1 year without remission periods or with remission periods lasting <1 month



Comments:

Chronic cluster headache may arise de novo (previously referred to as primary chronic cluster headache) or evolve from the episodic subtype (previously referred to as secondary chronic cluster headache). Some patients may switch from chronic to episodic cluster headache.

3.2 Paroxysmal hemicrania

Description:

Attacks with similar characteristics of pain and associated symptoms and signs to those of cluster headache, but they are shorter-lasting, more frequent, occur more commonly in females and respond absolutely to indomethacin.
Diagnostic criteria:

A. At least 20 attacks fulfilling criteria B-D

B. Attacks of severe unilateral orbital, supraorbital or temporal pain lasting 2-30 minutes

C. Headache is accompanied by at least one of the following:


  1. 1. ipsilateral conjunctival injection and/or lacrimation

  2. 2. ipsilateral nasal congestion and/or rhinorrhoea

  3. 3. ipsilateral eyelid oedema

  4. 4. ipsilateral forehead and facial sweating

  5. 5. ipsilateral miosis and/or ptosis

D. Attacks have a frequency above 5 per day for more than half of the time, although periods with lower frequency may occur

E. Attacks are prevented completely by therapeutic doses of indomethacin1

F. Not attributed to another disorder2

Notes:

1. In order to rule out incomplete response, indomethacin should be used in a dose of 150 mg daily orally or rectally, or 100 mg by injection, but for maintenance smaller doses are often sufficient.

2. 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 attacks do not occur for the first time in close temporal relation to the disorder.


Comments:

There is no male predominance. Onset is usually in adulthood, although childhood cases are reported.

In the first edition all paroxysmal hemicranias were referred to as chronic paroxysmal hemicrania. Sufficient clinical evidence for the episodic subtype has accumulated to separate it in a manner analogous to cluster headache.


Paroxysmal hemicrania with coexistent trigeminal neuralgia (CPH-tic syndrome):

Patients who fulfil criteria for both 3.2 Paroxysmal hemicrania and 13.1 Trigeminal neuralgia should receive both diagnoses. The importance of this observation is that both conditions require treatment. The pathophysiological significance of the association is not yet clear.

3.2.1 Episodic paroxysmal hemicrania

Description:

Attacks of paroxysmal hemicrania occurring in periods lasting 7 days to 1 year separated by pain-free periods lasting 1 month.
Diagnostic criteria:

A. Attacks fulfilling criteria A-F for 3.2 Paroxysmal hemicrania

B. At least two attack periods lasting 7-365 days and separated by pain-free remission periods of 1 month



3.2.2 Chronic paroxysmal hemicrania (CPH)

Description:

Attacks of paroxysmal hemicrania occurring for >1 year without remission or with remissions lasting <1 month.
Diagnostic criteria:

A. Attacks fulfilling criteria A-F for 3.2 Paroxysmal hemicrania

  1. Attacks recur over >1 year without remission periods or with remission periods lasting <1 month



3.3 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)

Description:

This syndrome is characterised by short-lasting attacks of unilateral pain that are much briefer than those seen in any other TAC and very often accompanied by prominent lacrimation and redness of the ipsilateral eye.
Diagnostic criteria:

A. At least 20 attacks fulfilling criteria B-D

B. Attacks of unilateral orbital, supraorbital or temporal stabbing or pulsating pain lasting 5-240 seconds

C. Pain is accompanied by ipsilateral conjunctival injection and lacrimation

D. Attacks occur with a frequency from 3 to 200 per day

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 attacks do not occur for the first time in close temporal relation to the disorder.
Comments:

This syndrome was described after the publication of the first edition of The International Classification of Headache Disorders and has become well recognised in the last decade.

Patients may be seen with only one of conjunctival injection or tearing, or other cranial autonomic symptoms such as nasal congestion, rhinorrhoea or eyelid oedema may be seen. 3.3 SUNCT may be a subform of A3.3 Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms (SUNA), described in the appendix.

The literature suggests that the most common mimics of 3.3 SUNCT are lesions in the posterior fossa or involving the pituitary gland.

SUNCT with coexistent trigeminal neuralgia: Patients have been described in whom there is an overlap between 3.3 SUNCT and 13.1 Trigeminal neuralgia. Such patients should receive both diagnoses. This differentiation is clinically difficult.

3.4 Probable trigeminal autonomic cephalalgia

Description:

Headache attacks that are believed to be a subtype of trigeminal autonomic cephalalgia but which do not quite meet the diagnostic criteria for any of the subtypes described above.
Diagnostic criteria:

  1. Attacks fulfilling all but one of the specific criteria for one of the subtypes of trigeminal autonomic cephalalgia

  2. Not attributed to another disorder



Comment:

Patients coded as 3.4 Probable trigeminal autonomic cephalalgia or one of its subforms either have had an insufficient number of typical attacks or fail to fulfil one of the other criteria.

3.4.1 Probable cluster headache

Diagnostic criteria:

A. Attacks fulfilling all but one of criteria A-D for 3.1 Cluster headache

B. Not attributed to another disorder



3.4.2 Probable paroxysmal hemicrania

Diagnostic criteria:

A. Attacks fulfilling all but one of criteria A-E for 3.2 Paroxysmal hemicrania

B. Not attributed to another disorder



3.4.3 Probable short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing

Diagnostic criteria:

A. Attacks fulfilling all but one of criteria A-D for 3.3 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)

B. Not attributed to another disorder



References

General

Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurology 2002;1:37-43.

Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic features, including new cases. Brain 1997;120:193-209.

May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiological implications for primary headache syndromes of the neural influences on the cerebral circulation. Journal of Cerebral Blood Flow and Metabolism 1999;19:115-127.

3.1 Cluster headache

Alberca R, Ochoa JJ. Cluster tic syndrome. Neurology 1994;44:996-999.

Bahra A, May A, Goadsby PJ. Diagnostic patterns in cluster headache. In: Olesen J, Goadsby PJ, editors. Cluster Headache and Related Conditions. Oxford: Oxford University Press 1999: 61-65.

Bahra A, May A, Goadsby PJ. Cluster headache: a prospective clinical study in 230 patients with diagnostic implications. Neurology 2002;58:354-361.

Bing R. Uber traumatische Erythromelalgie und Erthroprosopalgie. Nervenarzt 1930;3:506-512.

de Fine Olivarius B. Hemicrania neuralgiformis chronica (Chronic migrainous neuralgia). Quoted by Sjaastad O, editor. Proceedings of the Scandinavian Migraine Society Annual Meeting 1971: 8.

Ekbom K. Ergotamine tartrate orally in Horton’s ‘histaminic cephalalgia’ (also called Harris’s ciliary neuralgia). Acta Psychiatrica Scandinavia 1947;46:106.

Ekbom K. Nitroglycerin as a provocative agent in cluster headache. Archives of Neurology 1968;19:487-493.

Eulenberg A. Lehrbuch der Nervenkrankheiten. 2nd ed. Berlin: Hirschwald 1878.

Harris W. Ciliary (migrainous) neuralgia and its treatment. British Medical Journal 1936;1:457-460.

Horton BT. Histaminic cephalgia. Lancet 1952;2:92-98.

Kudrow L. Cluster headache: Mechanisms and Management. Oxford: Oxford University Press 1980.

Manzoni GC. Gender ratio of cluster headache over the years: a possible role of changes in lifestyle. Cephalalgia 1998;18:138-142.

Manzoni GC, Micieli G, Granella F, Tassorelli C, Zanferrari C, Cavallini A. Cluster headache- course over ten years in 189 patients. Cephalalgia 1991;11:169-174.

Manzoni GC, G.Terzano M, Bono G, Micieli G, Martucci N, Nappi G. Cluster headache - clinical findings in 180 patients. Cephalalgia 1983;3:21-30.

May A, Bahra A, Buchel C, Frackowiak RSJ, Goadsby PJ. Hypothalamic activation in cluster headache attacks. Lancet 1998;351:275-278.

Mulleners WM, Verhagen WIM. Cluster-tic syndrome. Neurology 1996;47:302.

Pascual J, Berciano J. Relief of cluster-tic syndrome by the combination of lithium and carbamazepine. Cephalalgia 1993;13:205-206.

Romberg MH. Lehrbuch der Nervenkrankheiten des Menschen. Berlin: Dunker 1840.

Russell MB, Andersson PG, Thomsen LL, Iselius L. Cluster headache is an autosomal dominantly inherited disorder in some families: a complex segregation analysis. Journal of Medical Genetics 1995;32:954-956.

Sjostrand C, Waldenlind E, Ekbom K. A follow up study of 60 patients after an assumed first period of cluster headache. Cephalalgia 2000;20:653-657.

Sluder G. The syndrome of sphenopalatine ganglion neurosis. American Journal of Medicine 1910;140:868-878.

Solomon S, Apfelbaum RI, Guglielmo KM. The cluster-tic syndrome and its surgical therapy. Cephalalgia 1985;5:83-89.

Torelli P, Cologno D, Cademartiri C, Manzoni GC. Application of the International Headache Society classification criteria in 652 cluster headache patients. Cephalalgia 2001;21:145-150.

Vail HH. Vidian neuralgia. Ann Otol Rhinol Laryngol 1932;41:837-856.

Watson P, Evans R. Cluster-tic syndrome. Headache 1985;25:123-126.

3.2 Paroxysmal hemicrania

Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua. Parenteral indomethacin: the ‘Indotest’. Headache 1998;38:122-128.

Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania (CPH): a review of the clinical manifestations. Headache 1989;29:648-656.

Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicrania in a young child: possible relation to ipsilateral occipital infarction. Journal of Child Neurology 1993;8:235-236.

Caminero AB, Pareja JA, Dobato JL. Chronic paroxysmal hemicrania-tic syndrome. Cephalalgia 1998;18:159-161.

Hannerz J. The second case of chronic paroxysmal hemicrania-tic syndrome [Editorial Comment]. Cephalalgia 1998;18:124.

Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache 1989;29:280-281.

Sjaastad O, Dale I. Evidence for a new (?) treatable headache entity. Headache 1974;14:105-108.

Zukerman E, Peres MFP, Kaup AO, Monzillo PH, Costa AR. Chronic paroxysmal hemicrania-tic syndrome. Neurology 2000;54:1524-1526.



3.3 SUNCT

Benoliel R, Sharav Y. Trigeminal neuralgia with lacrimation or SUNCT syndrome? Cephalalgia 1998;18:85-90.

Bouhassira D, Attal N, Esteve M, Chauvin M. SUNCT syndrome. A case of transformation from trigeminal neuralgia. Cephalalgia 1994;14:168-170.

Bussone G, Leone M, Volta GD, Strada L, Gasparotti R. Short-lasting unilateral neuralgiform headache attacks with tearing and conjunctival injection: the first symptomatic case. Cephalalgia 1991;11:123-127.

De Benedittis G. SUNCT syndrome associated with cavernous angioma of the brain stem. Cephalalgia 1996;16:503-506.

Ferrari MD, Haan J, van Seters AP. Bromocriptine-induced trigeminal neuralgia attacks in a patient with pituitary tumor. Neurology 1988;38:1482-1484.

Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic features, including new cases. Brain 1997;120:193-209.

Goadsby PJ, Matharu MS, Boes CJ. SUNCT syndrome or trigeminal neuralgia with lacrimation. Cephalalgia 2001;21:82-83.

Levy MJ, Matharu MS, Goadsby PJ. Prolactinomas, dopamine agonist and headache: two case reports. European Journal of Neurology 2003;10:169-174.

Massiou H, Launay JM, Levy C, El Amran M, Emperauger B, Bousser M-G. SUNCT syndrome in two patients with prolactinomas and bromocriptine-induced attacks. Neurology 2002;58:1698-1699.

Matharu MS, Levy MJ, Merry RT, Goadsby PJ. SUNCT syndrome secondary to prolactinoma. J. Neurol. Neurosurg. Psychiatry 2003:in press.

Morales F, Mostacero E, Marta J, Sanchez S. Vascular malformation of the cerebellopontine angle associated with SUNCT syndrome. Cephalalgia 1994;14:301-302.

Moris G, Ribacoba R, Solar DN, Vidal JA. SUNCT syndrome and seborrheic dermatitis associated with craneosynostosis. Cephalalgia 2001;21:157-159.

Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review. Headache 1997;37:195-202.

Penart A, Firth M, Bowen JRC. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) following presumed dorsolateral brainstem infarction. Cephalalgia 2001;21:236-239.

Sjaastad O, Saunte C, Salvesen R, Fredriksen TA, Seim A, Roe OD, et al. Shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating, and rhinorrhea. Cephalalgia 1989;9:147-156.

ter Berg HWM, Goadsby PJ. Significance of atypical presentation of symptomatic SUNCT: a case report. J Neurol Neurosurg Psychiat 2001;70:244-246.





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