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3. Classification and Clinical Features
3.1 Classification
Cluster headache (CH) and other trigeminal autonomic cephalgias (TACs) are primary headaches, and their clinical diagnosis depends on the classification system of the International Headache Society (IHS) [1]. Goadsby and Lipton were the first to propose the term “Trigeminal Autonomic Cephalgias” in 1997 [2]. The headache field has enjoyed a systematic hierarchical classification system and associated explicit (operational) diagnostic criteria since 1988 [3]. Currently, after much field testing of the previous editions, the third edition of the International Classification of Headache Disorders is in force. It is named ICHD-3, and it will be coordinated with the forthcoming International Classification of Diseases edition 11 (ICD-11) of the World Health Organization [1]. According to this hierarchical classification, patients can be diagnosed in groups and subgroups with various levels of diagnostic refinement. With such a system, patients can be diagnosed according to the first or second digit in general clinical practice and to third or fourth digit for specialist or research purposes.
International headache classification for cluster headache
3.1 Cluster headache |
3.1.1 Episodic cluster headache |
3.1.2 Chronic cluster headache |
A. At least five attacks fulfilling criteria B–D |
B. Severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15–180 min (when untreated) |
C. Either or both of the following |
1. At least one of the following symptoms or signs, ipsilateral to the headache |
(a) Conjunctival injection and/or lacrimation |
(b) Nasal congestion and/or rhinorrhea |
(c) Eyelid edema |
(d) Forehead and facial sweating |
(e) Miosis and/or ptosis |
2. A sense of restlessness or agitation |
D. Occurring with a frequency between one every other day and eight per day |
E. Not better accounted for by another ICHD-3diagnosis |
International headache classification for paroxysmal hemicrania
3.2 Paroxysmal hemicranias |
3.2.1 Episodic paroxysmal hemicrania |
3.2.2 Chronic paroxysmal hemicrania |
A. At least 20 attacks fulfilling criteria B–E |
B. Severe unilateral orbital, supraorbital, and/or temporal pain lasting 2–30 min |
C. Either or both of the following |
1. At least one of the following symptoms or signs, ipsilateral to the headache |
(a) Conjunctival injection and/or lacrimation |
(b) Nasal congestion and/or rhinorrhea |
(c) Eyelid edema |
(d) Forehead and facial sweating |
(e) Miosis and/or ptosis |
2. A sense of restlessness or agitation |
D. Occurring with a frequency of >5 per day |
E. Prevented absolutely by therapeutic doses of indomethacin |
F. Not better accounted for by another ICHD-3 diagnosis |
International headache classification for SUN
3.3 Short-lasting unilateral neuralgiform headache attacks |
A. At least 20 attacks fulfilling criteria B–D |
B. Moderate or severe unilateral head pain, with orbital, supraorbital, temporal, and/or other trigeminal distribution, lasting for 1–600 s and occurring as single stabs, series of stabs or in a sawtooth pattern |
C. At least one of the following cranial autonomic |
1. Symptoms or signs, ipsilateral to the pain |
2. Conjunctival injection and/or lacrimation |
3. Nasal congestion and/or rhinorrhea |
4. Eyelid edema |
5. Forehead and facial sweating |
6. Miosis and/or ptosis |
D. Occurring with a frequency of at least one a day |
E. Not better accounted for by another ICHD-3 diagnosis |
3.3.1 Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) |
3.3.1.1 Episodic SUNCT |
3.3.1.2 Chronic SUNCT |
A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks |
B. Both of the following, ipsilateral to the pain |
1. Conjunctival injection |
2. Lacrimation (tearing) |
3.3.2 Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) |
3.3.2.1 Episodic SUNA |
3.3.2.2 Chronic SUNA |
A. Attacks fulfilling criteria for 3.3 Short-lasting unilateral neuralgiform headache attacks and criterion B |
B. Not more than one of the following, ipsilateral to the pain |
1. Conjunctival injection |
2. Lacrimation (tearing) |
International headache classification for hemicrania continua
3.4 Hemicrania continua |
3.4.1 Hemicrania continua, remitting subtype |
3.4.2 Hemicrania continua, unremitting subtype |
A. Unilateral headache fulfilling criteria B–D |
B. Present for >3 months, with exacerbations of moderate or greater intensity |
C. Either or both of the following |
1. At least one of the following symptoms or signs, ipsilateral to the headache |
(a) Conjunctival injection and/or lacrimation |
(b) Nasal congestion and/or rhinorrhea |
(c) Eyelid edema |
(d) Forehead and facial sweating |
(e) Miosis and/or ptosis |
2. A sense of restlessness or agitation or aggravation of the pain by movement |
D. Responds absolutely to therapeutic doses of indomethacin |
E. Not better accounted for by another ICHD-3 diagnosis |
3.4.1 Hemicrania continua, remitting subtype |
A. Headache fulfilling criteria for 3.4 Hemicrania continua and criterion B below |
B. Headache is not daily or continuous but interrupted (without treatment) by remission periods of 24 h |
3.4.2 Hemicrania continua, unremitting subtype |
A. Headache fulfilling criteria for 3.4 Hemicrania continua and criterion B below |
B. Headache is daily and continuous for at least 1 year, without remission periods of 24 h |
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