Field testing ICHD classifications Jes Olesen, professor of neurology Danish Headache Center, dept of neurology Rigshospitalet Glostrup Denmark Conflicts of interest: None

Improving criteria for a primary headache 1.2 Migraine with aura 1.2 and A 1.2

1.2 Migraine with aura 2716 patients with 1.2 MA, 1.2.1 MTA, 1.2.4 FHM, 1.2.5 SHM and 1.2.6 basilar-type migraine according to ICHD-2 were prospectively collected in a cross sectional study design

Results – Criteria for 1.2 MA All patients fulfilled the criteria for 1.2 MA in ICHD-3 beta. The new ICHD-3 beta criteria for 1.2 MA were thus 100% sensitive. The specificity for 1.2 MA in ICHD-3 beta compared to patients diagnosed with 1.1 MO was 89,3%.

Main body- and appendix criteria for MA. Differentiation from TIA TIA in anterior circulation 88% TIA in posterior circulation 12% Recurrent TIA 6% Duration of TIA: 42 patients (35%) less than 60 minutes 18 patients (15%) from 1 hour to 3 hours 60 patients (50 %) from 3 hours to 24 hours

Specificity for migraine with aura criteria 1.2 Migraine with aura main body specificity 0.73 A1.2 Migraine with aura alternative criteria specificity 0. 91 1.2.1 Migraine with typical aura main body specificity 0.88 A1.2.1 Migraine with typical aura alternative criteria specificity 1.0

Developing criteria for a new kind of primary headache 4.8 Nummular headache

Pareja JA, Caminero AB, Serra J, Barriga FJ, Dobato JL, Barón M, Vela L, Sánchez del Río M. Numular headache: a coin-shaped cephalgia. Neurology 2002; 58: 1678-1679 Guerrero AL, Cortijo E, Herrero-Velázquez S, Mulero P, Miranda S, Peñas ML, Pedraza MI, Fernández R. Nummular headache with and without exacerbations: comparative characteristics in a series of 72 patients. Cephalalgia 2012; 32: 649-653.

Nummular headache A. Continuous or intermittent head pain fulfilling criterion B B. Felt exclusively in an area of the scalp, with all of the following four characteristics: 1.sharply-contoured 2.fixed in size and shape 3.round or elliptical 4.1-6 cm in diameter C. Not better accounted for by another ICHD-3 diagnosis1.

Further testing desireable How well do the criteria perform tested against a migraine material, a TTH material etc. (specificity) In a mixed material of patients with localized headache, should the criteria be tightened or loosened? Is pain after minor localized trauma excluded or not?

Improving criteria for a secondary headache 6.1.2 Headache attributed to TIA 7.1.1 Headache attributed to Idiopathic Intracranial Hypertension

Headache Attributed to TIA according to ICHD-3 Any new headache fulfilling criterion C Transient ischemic attack (TIA) has been diagnosed Evidence of causation demonstrated by both of the following: Headache has developed simultaneously with other symptoms and/or clinical signs of TIA Headache resolves within 24 hours D. Not better accounted for by another ICHD-3 diagnosis

Test of criteria in 120 patients with TIA Extensive physician conducted validated semistructured interviews Characterisation of the TIA Characterisation of previous headache disorders Characterisation of headache occurring around the time of TIA A new type of headache A previous type with altered characteristics A previous type unaltered Many occurred hours after TIA and several lasted longer than 24 hours Lebedeva E.R. et al in preparation

Proposed diagnostic criteria for Headache Attributed to TIA Headache fulfilling criterion C Transient ischemic attack (TIA) has been diagnosed Evidence of causation demonstrated by one of the following: Any type of headache occurring within one hour of TIA onset Any new type of headache occurring within 24 hours of TIA onset D. Not better accounted for by another ICHD-3 diagnosis Lebedeva E.R. et al in preparation

Yri HM and Jensen RH: Cephalalgia 2015; 35: 553-562 Patient material

Distinguishing clinical features

Developing criteria for a new kind of secondary headache 10.1.2 Headache attributed to airoplane travel A 6.10 Persistent headache attributed to arterial dissection

Clinical picture of Aeroplane Headache Median age at diagnosis is 28.7 ± 4.8 years 148 males and 127 females Onset: Exclusively during take-off or landing – mainly during landing Duration: AH:10-30 min. Severity: very severe with a rating of 8-10/10 Quality: stabbing, jabbing or pulsating in the fronto-orbital region. Berlingen et al 2006, 2011, Mainardi et al 2012

Mainardi et al 2012

Headache attributed to aerplane travel A. At least two episodes of headache fulfilling criterion C B. The patient is travelling by aeroplane C. Evidence of causation demonstrated by at least two of the following: 1.headache has developed during the aeroplane flight 2.either or both of the following: a)headache has worsened in temporal relation to ascent following take-off and/or descent prior to landing of the aeroplane b)headache has spontaneously improved within 30 minutes after the ascent or descent of the aeroplane is completed 3.headache is severe, with at least two of the following three characteristics: a)unilateral location1 b)orbitofrontal location2 c)jabbing or stabbing quality3 D.Not better accounted for by another ICHD-3 diagnosis4.

ICHD-II at time of dissection A6.10 Persistent headache attributed to past cranial or cervical vascular disorder ICHD-II at time of dissection 17/19 patients were classified as having:  6.5.1 Headache or facial or neck pain attributed to cervical carotid or vertebral or intracranial artery dissection  2/19 patients were classified as having: usual primary headache triggered by dissection. ICHD-III six months after dissection 5/17 patients were classified as having: A6.10 Persistent headache attributed to past cranial or cervical vascular disorder (appendix criteria) Table of dissection, headache characteristics and headache classification of 19 patients with cervical carotid or vertebral artery dissection. Schytz et al. Cephalalgia 2014

Improving criteria for a cranial neuralgia 13.1.1 Classical trigeminal neuralgia

Field-testing of diagnostic criteria in classical trigeminal neuralgia    Sensitivity  Specificity ICHD 2 76.2% 89.2% ICHD 3 beta 74.3% 97.3% Author´s first proposed criteria caused large decrease in specificity* 76.7% 64.9% Authors final proposed criteria 96.1% 83.8% Firstly, we evaluated the sensitivity and specificity of ICHD-3 beta when the monothetic criterion B that excludes pain radiating outside the trigeminal distribution was removed. This caused a small insignificant increase in sensitivity to 76.7% (p = 0.074 compared to ICHD-3 beta) but the specificity dropped from 97.3% (ICHD-3 beta) to 64.9% (p = 0.002). We considered this modification unfavorable due to decreased specificity and no yield in sensitivity. Maarbjerg et al 2015

Conclusions ICHD-3 emminently suited for field testing of diagnostic criteria Good case series with prospective systematic recording of symptoms and sometimes laboratory investigations are necessary Claims based on studies labelled field testing are not necessarily correct. It has to be done right Many changes have already been based on good field testing There is much more to do, particularly for the secondary headaches

Classical trigeminal neuralgia, proposed criteria

Why field testing? ICHD classifications more and more evidence based ICHD classifications have explicit (operational) diagnostic criteria They are suited for field testing and modification ICHD-3beta to ICHD-3 entirely based on field testing studies All future changes to headache classification should be based exclusively on new scientific evidence (field testing)

Types of field testing Improving criteria for a primary headache Developing criteria for a new type of primary headache Improving criteria for a secondary headache Developing criteria for a new type of secondary headache Improving criteria for a cranial neuralgia