The International Classification Of Headache Disorders, 3rd Edition .

ICHD-3 beta633PrefaceAfter two very successful editions of the InternationalClassification of Headache Disorders (ICHD), a thirdis now close to being final. The members of theClassification Committee have all worked hard forthree years in order to accomplish this beta version.Most members have chaired the work on a specificchapter of the classification, assisted by a number ofother experts. For this edition, there has been a substantial body of evidence available for the classification work, in contrast to our previous editions, whichwere mostly based on the opinions of experts. Wehave tried to be conservative, making changes onlywhere there was good published evidence to supportchange or where the need for change was intuitivelyobvious.This is the first time that we have published a betaversion ahead of the final version. The main reason is tosynchronize ICHD-3 with the World HealthOrganization’s next revision (11th edition) of theInternational Classification of Diseases (ICD-11). Thisclassification is already well advanced, and we have notonly secured a very good representation of headachewithin ICD-11 but also ensured congruence betweenICD-11 and ICHD-3 beta. However, ICD-11 nowenters a phase of field trials, and ICHD-3 should dothe same. Such a test period will allow identificationand correction of mistakes and enable a broad inputfrom the members of the International HeadacheSociety.ICD-11 diagnostic codes will not be finalized untiltwo or three years from now, but it would be a majoradvantage for ICHD-3 to be able to include thesecodes along with our own. WHO’s ICD-11 codeswill be used by health authorities for official diagnostic coding, and in many cases they will be employedfor reimbursement purposes; we must have themright.We publish ICHD-3 beta immediately on theInternational Headache Society’s website, and shortlyafter as an issue of Cephalalgia. Field-testing will continue for 2 or maybe 3 years. Small amendments arelikely both to ICHD-3 and to the diagnostic codes ofICD-11, and these will be incorporated. At that time,we shall publish ICHD-3 in final form in Cephalalgia.ICHD-3 beta is published only in English, but thosethroughout the world who wish to make their own careful translations of parts or in toto are welcome to do sosubject to the conditions stated above. The final versionof ICHD-3 should be translated into as many languagesas possible, and these translations published, as happened to the second and first editions. As we expectICHD-3 beta to be very similar to the final version,translation work begun now is likely to remain useful.Any changes necessitated later by the outcomes of fieldtesting can be made easily.Clinicians and researchers should start using the criteria of ICHD-3 beta. There are many improvementsover ICHD-II, and it would be unhelpful to continue touse ICHD-II for scientific work. We encourage readersto study ICHD-3 beta very closely, and document andcomment on any inconsistencies they may find.Comments should be sent not to me but to the chairmenof the relevant working groups. Their names and emailaddresses are found in this publication and on the IHSwebsite.Jes OlesenChairmanHeadache Classification CommitteeInternational Headache Society

634Cephalalgia 33(9)How to use this classificationThis extensive document is not intended to be learnt byheart. Even members of the Classification Committeeare unable to remember all of it. It is a document thatshould be consulted time and time again. In this wayyou will soon get to know the diagnostic criteria for 1.1Migraine without aura, 1.2 Migraine with aura, themajor subtypes of 2. Tension-type headache, 3.1Cluster headache and a few others. The rest willremain something to look up. In clinical practice youdo not need the classification for the obvious case ofmigraine or tension-type headache, but it is useful whenthe diagnosis is uncertain. For research, the classification is indispensable and every patient entered into aresearch project, be it a drug trial or a study of pathophysiology or biochemistry, must fulfil a set of diagnostic criteria.1. This classification is hierarchical, and you mustdecide how detailed you want to make your diagnosis. This can range from the first-digit level to thefifth. First, one gets a rough idea about whichgroup the patient belongs to. Is it, for example,1. Migraine or 2. Tension-type headache or 3.Trigeminal autonomic cephalalgias? Then oneobtains information allowing a more detailed diagnosis. The desired detail depends on the purpose. Ingeneral practice only the first- or second-digit diagnoses are usually applied, whereas in specialistpractice and headache centres a diagnosis at thefourth- or fifth-digit level is appropriate.2. For most purposes, patients receive a diagnosisaccording to the headache phenotypes that theycurrently present, or that they have presentedwithin the last year. For genetic and some otheruses, occurrence during the whole lifetime is used.3. Each distinct type, subtype or subform of headachethat the patient has must be separately diagnosedand coded. Thus, a severely affected patient in aheadache centre may receive three diagnoses andcodes: 1.1 Migraine without aura, 1.2 Migrainewith aura and 8.2 Medication-overuse headache.4. When a patient receives more than one diagnosis,these should be listed in the order of importance tothe patient.5. When one type of headache in a particular patientfulfils two different sets of diagnostic criteria, thenall other available information should be used todecide which of the alternatives is the correct ormore likely diagnosis. This could include the6.7.8.9.longitudinal headache history (how did the headache start?), the family history, the effect of drugs,menstrual relationship, age, gender and a range ofother features. Fulfilment of the diagnostic criteriafor 1. Migraine, 2. Tension-type headache or 3.Trigeminal autonomic cephalalgias, or any of theirsubtypes, always trumps fulfilment of criteria forthe probable diagnostic categories of each, whichare last-described in the respective groups. Inother words, a patient whose headache fulfils criteria for both 1.5 Probable migraine and 2.1Infrequent episodic tension-type headache shouldbe coded to the latter. Nevertheless, considerationshould always be given to the possibility that someheadache attacks meet one set of criteria, whereasother attacks meet another set. In such cases, twodiagnoses exist and both should be coded.To receive a particular headache diagnosis thepatient must, in many cases, experience a minimumnumber of attacks of (or days with) that headache.This number is specified in the diagnostic criteriafor the headache type, subtype or subform.Further, the headache must fulfil a number ofother requirements described within the criteriaunder separate letter headings: A, B, C etc. Someletter headings are monothetic: that is, they expressa single requirement. Other letter headings arepolythetic, requiring for example any two out offour listed characteristics.The full set of diagnostic criteria is provided forsome headache disorders only at the first- andsecond-digit levels. Diagnostic criteria at thethird- and fourth-digit levels then demand, as criterion A, fulfilment of the criteria for levels oneand/or two and, in criterion B and onwards, specifythe further specific criteria to be fulfilled.The frequency of primary headache disorders variesfrom attacks every one to two years to attacksdaily. The severity of attacks also varies. ICHD-3beta does not generally provide a possibility to codefor frequency or severity, but recommends that frequency and severity be specified in free text.Primary or secondary headache or both: When anew headache occurs for the first time in close temporal relation to another disorder that is known tocause headache, or fulfils other criteria for causation by that disorder, the new headache is coded asa secondary headache attributed to the causativedisorder. This remains true even when the

ICHD-3 beta10.11.12.13.headache has the characteristics of a primary headache (migraine, tension-type headache, clusterheadache or one of the other trigeminal autonomiccephalalgias). When a pre-existing primary headache becomes chronic in close temporal relation tosuch a causative disorder, both the primary and thesecondary diagnoses should be given. When a preexisting primary headache is made significantlyworse (usually meaning a two-fold or greaterincrease in frequency and/or severity) in close temporal relation to such a causative disorder, both theprimary and the secondary headache diagnosesshould be given, provided that there is good evidence that the disorder can cause headache.The last criterion for almost every headache disorder is ‘Not better accounted for by another ICHD3 diagnosis’. Consideration of other possible diagnoses (the differential diagnosis) is a routine part ofthe clinical diagnostic process. When a headacheappears to fulfil the criteria for a particular headache disorder, this last criterion is a reminderalways to consider other diagnoses that mightbetter explain the headache.In particular this applies to assessing whether headache is secondary or primary. It may also apply toalternative causative disorders: for example, headache occurring in close temporal relation to acuteischaemic stroke may be a consequence not of thestroke but of the cause of the stroke (e.g. dissection).Many patients with headache attacks fulfilling oneset of explicit diagnostic criteria also have attacksthat, although similar, do not quite satisfy the criteria. This can be a result of treatment, inability torecall symptoms exactly or other factors. Ask thepatient to describe a typical untreated or unsuccessfully treated attack, and ascertain that there havebeen enough of these to establish the diagnosis.Then include the less-typical attacks when describing attack frequency.When a patient is suspected of having more thanone headache type or subtype, it is highly recommended that he or she fill out a diagnostic headachediary in which, for each headache episode, theimportant characteristics are recorded. It has beenshown that such a headache diary improves diagnostic accuracy as well as allowing a more precisejudgement of medication consumption. The diaryhelps in judging the quantity of two or more different headache types or subtypes. Finally, it teachesthe patient how to distinguish between differentheadaches, for example between migraine withoutaura and episodic tension-type headache.In each chapter on secondary headaches, themost well-known and well-established causes are International Headache Society 2013635mentioned and criteria for these are given.However, in many chapters, for example 9.Headache attributed to infection, there are analmost endless number of possible causes. Inorder to avoid a very long list, only the most important are mentioned. In the example, rarer causes areassigned to 9.2.3 Headache attributed to other systemic infection. The same system is used in the otherchapters on secondary headaches.14. The diagnostic criteria for the secondary headachesno longer require remission or substantial improvement of the underlying causative disorder beforethe headache diagnosis can be made. The diagnostic criteria of ICHD-3 beta may be applied alreadyon presentation or as soon after as the underlyingdisorder is confirmed. Criterion A is presence of theheadache; criterion B is presence of the causativedisorder; criterion C is the evidence of causation.In acute conditions, a close temporal relationbetween onset of headache and onset of the presumed causative disorder is often sufficient to establish causation, whereas less acute conditions usuallyrequire more evidence of causation. In all cases, thelast criterion must be applied as a check: ‘Notbetter accounted for by another ICHD-3diagnosis’.15. In a few secondary headaches, 5.2 Persistent headache attributed to traumatic head injury being agood example, persistent headache subforms arerecognized to occur; that is, headache that wascaused initially by another disorder fails to remitafter that disorder has resolved. In such cases, thediagnosis changes from the acute subform (e.g. 5.1Acute headache attributed to traumatic head injury)to the persistent subform (5.2 Persistent headacheattributed to traumatic head injury) after a specifiedtime interval (three months in this example).Evidence of causation depends on earlier fulfilmentof the criteria for diagnosis of the acute subform,and persistence of the same headache.Most such diagnoses are in the Appendixbecause of insufficient evidence for their existence.They will not usually be applied, but are thereto stimulate research into better criteria forcausation.16. The Appendix is for research. It helps clinical scientists study orphan entities for later inclusion in (or,in some cases, exclusion from) the main body of theclassification. Most diagnoses and diagnostic criteria in the Appendix are either new or alternativesto criteria in the main body. Some are old entitiesnot yet sufficiently validated; these are expected tobe deleted in the next revision of ICHD if evidenceis not produced.

636Cephalalgia 33(9)ClassificationICHD-3 3.23.2.13.2.2MigraineMigraine without auraMigraine with auraMigraine with typical auraTypical aura with headacheTypical aura without headacheMigraine with brainstem auraHemiplegic migraineFamilial hemiplegic migraine (FHM)Familial hemiplegic migraine type 1 (FHM1)Familial hemiplegic migraine type 2 (FHM2)Familial hemiplegic migraine type 3 (FHM3)Familial hemiplegic migraine, other lociSporadic hemiplegic migraineRetinal migraineChronic migraineComplications of migraineStatus migrainosusPersistent aura without infarctionMigrainous infarctionMigraine aura-triggered seizureProbable migraineProbable migraine without auraProbable migraine with auraEpisodic syndromes that may be associated with migraineRecurrent gastrointestinal disturbanceCyclical vomiting syndromeAbdominal migraineBenign paroxysmal vertigoBenign paroxysmal torticollisTension-type headache (TTH)Infrequent episodic tension-type headacheInfrequent episodic tension-type headache associated with pericranial tendernessInfrequent episodic tension-type headache not associated with pericranial tendernessFrequent episodic tension-type headacheFrequent episodic tension-type headache associated with pericranial tendernessFrequent episodic tension-type headache not associated with pericranial tendernessChronic tension-type headacheChronic tension-type headache associated with pericranial tendernessChronic tension-type headache not associated with pericranial tendernessProbable tension-type headacheProbable infrequent episodic tension-type headacheProbable frequent episodic tension-type headacheProbable chronic tension-type headacheTrigeminal autonomic cephalalgias (TACs)Cluster headacheEpisodic cluster headacheChronic cluster headacheParoxysmal hemicraniaEpisodic paroxysmal hemicraniaChronic paroxysmal hemicrania

637ICHD-3 g unilateral neuralgiform headache attacksShort-lasting unilateral neuralgiform headache attacks with conjunctival injection andtearing (SUNCT)Episodic SUNCTChronic SUNCTShort-lasting unilateral neuralgiform headache attacks with cranial autonomicsymptoms (SUNA)Episodic SUNAChronic SUNAHemicrania continuaProbable trigeminal autonomic cephalalgiaProbable cluster headacheProbable paroxysmal hemicraniaProbable short-lasting unilateral neuralgiform headache attacksProbable hemicrania continuaOther primary headache disordersPrimary cough headacheProbable primary cough headachePrimary exercise headacheProbable primary exercise headachePrimary headache associated with sexual activityProbable primary headache associated with sexual activityPrimary thunderclap headacheCold-stimulus headacheHeadache attributed to external application of a cold stimulusHeadache attributed to ingestion or inhalation of a cold stimulusProbable cold-stimulus headacheHeadache probably attributed to external application of a cold stimulusHeadache probably attributed to ingestion or inhalation of a cold stimulusExternal-pressure headacheExternal-compression headacheExternal-traction headacheProbable external-pressure headacheProbable external-compression headacheProbable external-traction headachePrimary stabbing headacheProbable primary stabbing headacheNummular headacheProbable nummular headacheHypnic headacheProbable hypnic headacheNew daily persistent headache (NDPH)Probable new daily persistent headacheHeadache attributed to trauma or injury to the head and/or neckAcute headache attributed to traumatic injury to the headAcute headache attributed to moderate or severe traumatic injury to the headAcute headache attributed to mild traumatic injury to the headPersistent headache attributed to traumatic injury to the headPersistent headache attributed to moderate or severe traumatic injury to the headPersistent headache attributed to mild traumatic injury to the headAcute headache attributed to whiplashPersistent headache attributed to whiplashAcute headache attributed to craniotomyPersistent headache attributed to craniotomy International Headache Society 2013

7.3.27.3.37.3.47.3.5Cephalalgia 33(9)Headache attributed to cranial or cervical vascular disorderHeadache attributed to ischaemic stroke or transient ischaemic attackHeadache attributed to ischaemic stroke (cerebral infarction)Headache attributed to transient ischaemic attack (TIA)Headache attributed to non-traumatic intracranial haemorrhageHeadache attributed to non-traumatic intracerebral haemorrhageHeadache attributed to non-traumatic subarachnoid haemorrhage (SAH)Headache attributed to non-traumatic acute subdural haemorrhage (ASDH)Headache attributed to unruptured vascular malformationHeadache attributed to unruptured saccular aneurysmHeadache attributed to arteriovenous malformation (AVM)Headache attributed to dural arteriovenous fistula (DAVF)Headache attributed to cavernous angiomaHeadache attributed to encephalotrigeminal or leptomeningeal angiomatosis(Sturge Weber syndrome)Headache attributed to arteritisHeadache attributed to giant cell arteritis (GCA)Headache attributed to primary angiitis of the central nervous system (PACNS)Headache attributed to secondary angiitis of the central nervous system (SACNS)Headache attributed to cervical carotid or vertebral artery disorderHeadache or facial or neck pain attributed to cervical carotid or vertebral arterydissectionPost-endarterectomy headacheHeadache attributed to carotid or vertebral angioplastyHeadache attributed to cerebral venous thrombosis (CVT)Headache attributed to other acute intracranial arterial disorderHeadache attributed to an intracranial endovascular procedureAngiography headacheHeadache attributed to reversible cerebral vasoconstriction syndrome (RCVS)Headache probably attributed to reversible cerebral vasoconstrictionsyndrome (RCVS)Headache attributed to intracranial arterial dissectionHeadache attributed to genetic vasculopathyCerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts andLeukoencephalopathy (CADASIL)Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS)Headache attributed to another genetic vasculopathyHeadache attributed to pituitary apoplexyHeadache attributed to non-vascular intracranial disorderHeadache attributed to increased cerebrospinal fluid pressureHeadache attributed to idiopathic intracranial hypertension (IIH)Headache attributed to intracranial hypertension secondary to metabolic, toxic orhormonal causesHeadache attributed to intracranial hypertension secondary to hydrocephalusHeadache attributed to low cerebrospinal fluid pressurePost-dural puncture headacheCSF fistula headacheHeadache attributed to spontaneous intracranial hypotensionHeadache attributed to non-infectious inflammatory diseaseHeadache attributed to neurosarcoidosisHeadache attributed to aseptic (non-infectious) meningitisHeadache attributed to other non-infectious inflammatory diseaseHeadache attributed to lymphocytic hypophysitisSyndrome of transient Headache and Neurological Deficits with cerebrospinal fluidLymphocytosis (HaNDL) International Headache Society 2013

639ICHD-3 28.2.

Organization's next revision (11th edition) of the International Classification of Diseases (ICD-11). This classification is already well advanced, and we have not only secured a very good representation of headache within ICD-11 but also ensured congruence between ICD-11 and ICHD-3 beta. However, ICD-11 now