UCNS Course A Review of ICHD-3b

Andrew D. Hershey, M.D., Ph.D., FAHS Endowed Chair and Director of Neurology

Director, Cincinnati Children’s Headache Center Professor of Neurology and Pediatrics

University of Cincinnati

Disclosures

Support – grants, contracts, honoraria - NIH, CHRF research foundation, Allergan, Amgen, Curelator, Depomed, and Lilly

American Headache Society – Board Member NIH – Advisory Board, Common Data Elements American Migraine Foundation – Advisory Board

Assoc Ed – Headache, Cephalalgia, The Journal of Headache Pain

Objectives

• Understand basic concepts of ICHD • Discuss changes and implications • To explore where things go from here

Headache Classification History

History of Migraine

• 3000 BC, Sumeria “the sick headache” The sick-eyed says not

- ‘I am sick-eyed’ The sick-headed not

- ‘I am sick-headed’

History of Migraine

• 400 BC, Hippocrates

– Shining light followed by violent pain in the temples

History of Migraine

• 2nd Century AD, Aretaeus of Cappadocia

– Heterocrania (one-sided headache with blackness before the eyes, nausea, vomiting, photophobia and osmophobia

• 200 AD, Galen

– Migraine is Greek for hemicrania

History of Childhood Migraine • 1949, Vahlquist and Hackzell

– Attacks separated by Sx free intervals – Two of: • nausea, • flimmer scotoma, • hemicrania, • FHx

Classification of Headache Ad Hoc Committee, 1962

• Vascular headache of migraine type • Muscle-contraction headache • Combined headache • 12 other headache syndromes

Classification of Headache Ad Hoc Committee, 1962

• Vascular headache of migraine type – “Classic” migraine – “Common” migraine – “Cluster” headache – “Hemiplegic” and “ophthalmoplegic” migraine – “Lower-half” headache

History of Childhood Migraine Prensky, Ped Cl N Amer, 1976

• 1976, Prensky

– Recurrent with symptom free intervals – Three our of six features

• Abdominal pain, nausea, or vomiting • Localized unilateral/hemicrania • Throbbing, pulsatile quality • Complete relief after a brief period of sleep • Aura - visual, sensory, or motor • Family history

History of Childhood Migraine Prensky, Ped Cl N Amer, 1976

• 1976, Prensky

– Abrupt or slow worsening – Vary greatly in severity – Do not need to interrupt activity – Occur at any time of day – Abdominal pain/nausea/vomiting

without headache

ICHD

• 1988 – International Classification of Headache Disorders – 3 years to develop, 12 subcommittees, expert opinion

based

• 2004 – 2nd Edition – Research testing of ICHD led to changes – Revision later of Medication overuse – Website development

• 2013 – 3rd Edition (beta) – Beta for testing and to synchronize with ICD-11

ICHD

• Organizational structure – Primary Headaches – Secondary Headaches – Painful Cranial Neuropathies – Appendix

ICHD

• Organizational structure – Primary Headaches

• Migraine • Tension-type Headaches • Trigeminal Autonomic Cephalagias • Other Primary Headaches

– Secondary Headaches – Painful Cranial Neuropathies – Appendix

ICHD

• Organizational structure – Primary Headaches – Secondary Headaches

• Posttraumatic • Vascular Disease • Other Intracranial Pathology • Substances • CNS infection • Homeostatic disorders • Cranium, Neck, Eyes, ENT, Sinuses, Mouth, Teeth, TMJ • Psychiatric

– Painful Cranial Neuropathies – Appendix

• Basic Concepts – Hierarchical – Diagnosis for current headache or within the past

year (genetics is lifetime) – Diagnosis each distinct type

• 1.1 Migraine without aura • 1.2 Migraine with aura • 8.2 Medication-overuse headache

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

• Basic Concepts – Hierarchical – Diagnosis for current headache or within the past

year (genetics is lifetime) – Diagnosis each distinct type – In order of importance – Sometimes a minimum number of attacks to meet

criteria, but frequency not typically required

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

• Basic Concepts – Primary vs. secondary vs. both

• If new headache first occurs in close temporal relation, with causation = secondary disorder

– Even if primary headache characteristics

• When a pre-existing primary becomes chronic or significantly worsens in close temporal relation

– Diagnose both primary and secondary headache

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

• Basic Concepts – Primary vs. secondary vs. both – Not all headaches required to meet criteria to make

diagnosis • May be due to treatment • Inability to recall • Can ask for typical or untreated headache

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

• Basic Concepts – Primary vs. secondary vs. both – Not all headaches required to meet criteria to make

diagnosis – Diary can help diagnosis and separation of multiple

types – Secondary headache does not require

remission/substantial improvement • Medication overuse can be diagnosed before recovery from

MOH

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

• Basic Concepts – Primary vs. secondary vs. both – Not all headaches required to meet criteria to make

diagnosis – Diary can help diagnosis and separation of multiple

types – Secondary headache does not require

remission/substantial improvement – Appendix for research

Classification of Headache International Classification of Headache Disorders – 3rd Edition (beta version),

Cephalalgia, 2013

ICHD – I to II to 3b

General evolution of diagnosis through testing and validation

Consistent Structure (although with some variability)

Primary Headaches A. Number of required attacks B. Time duration C. Headache Characteristics D. Associated features E. Rule out other causes

Secondary Headaches

A. Presence of Headache B. Presence of causative

disorder C. Evidence of causation

Changes in ICHD-3b

• Migraine – Migraine without aura

• Pediatric Note - Duration 2-72 hours (vs. 1-72 with diary) – Sleep included in duration

• Pediatric specific notes eliminated and in comments – Pediatric and adolescents (<18 years old) – Evolutionary change in location

– Migraine with aura • ICHD-3b changed to 6 auras (visual, sensory, speech and/or

language, motor, brainstem, and retinal) • Basilar-type migraine is now an aura (Migraine with

Brainstem Aura)

Changes in ICHD-3b

• Migraine – Chronic migraine

• Added in ICHD-II, modified in ICHD-2R • ICHD-3b

– Majority of headaches meet criteria for migraine or respond to migraine treatment

– Episodic syndromes that may be associated with migraine • Formerly Childhood periodic syndromes • Recognizes may occur in adults

Changes in ICHD-3b

• Tension-type Headaches – 3 subtypes by frequency

• Infrequent (10 episodes, < 1/month) • Frequent (10 episodes, 1-14 days/month) • Chronic (>15 days/month, >3 months)

Changes in ICHD-3b

• Trigeminal Autonomic Cephalagias – Cluster headache moved under this category – Paroxysmal Hemicrania – SUNCT and SUNA – Common TAC features

• Brief duration headaches • Autonomic abnormalities

– Divided into episodic and chronic • Hemicrania continua moved into this category

Changes in ICHD-3b

• Other primary – Headaches associated with exertion – Headaches associated with external stimuli – Epicrania – Miscellaneous

• NDPH

Changes in ICHD-3b

• Secondary headaches – General criteria A. Any headache fulfilling criterion C B. Another disorder scientifically documented to be able to cause

headaches has been diagnosed C. Evidence of causation (2 of 4)

1. Headache developed in temporal relation 2. One or both

a. Headache significantly worsened with causative disorder b. Headache significantly improved with treatment of causative disorder

3. Headache characteristics typical for causative disorder 4. Other evidence of causation

D. Rule out other causes

Changes in ICHD-3b

• Secondary headaches basic concepts – Cause and effect reduced, just needs to be consistent – Causative disorder can cause a primary headache

phenotype or worsen and existing primary phenotype

Changes in ICHD-3b

• Secondary headaches specific highlights – Post-traumatic – headache within 7 days from head

injury or awakening from head injury – ICH – Pressure required to be >250 mmCSF, but with

comment that up to 280 mmCSF may be normal in children

– MOH (should it just be Medication overuse?) • Example of transition to a relatively established criteria

– Removed specific agent requirement – Removed requirement to resolve after cessation – Can have both CM and MOH

Conclusions

• ICHD-3b is beta – Intended to be tested before finalization in 1-2 years

• ICHD revisions a continuing process • Evidence and validation is continuing • Genetics and biomarkers may be included in future • Recommended reading

– ICHD – 3b (Cephalagia, 2013) – ICHD-III – Changes and Challenges (M. Levin, Headache

Currents, 2013: 1383-