Classification: Migraine and Trigeminal Autonomic Cephalalgias Lauren Doyle Strauss, DO, FAHS Assistant Professor, Child Neurology Assistant Director, Child Neurology Residency @StraussHeadache No disclosures Disclosures Objectives Define the classification of migraine (chronic, episodic, with/without aura) and differences between adult and pediatric patients. Describe distinguishing characteristics of Trigeminal Autonomic Cephalalgias (TACs). Utilize treatment options that are best studied in each TAC. 1
Migraine without Aura (ICHD: 1.1) Minimum 5 attacks If < 5 attacks, diagnose with Probable migraine without aura Headache lasts 4*-72 hours This is a duration for an untreated headache This includes time spent sleeping Peds: Minimum is lower 2 hours Headache has 2 of the following: 1. Unilateral Peds: <15 yrs may be bilateral frontotemporal not occipital! 2. Pulsating quality 3. Moderate or severe pain intensity 4. Aggravation by or causing avoidance of routine physical activity (walking, climbing) During the headache, 1 of the following: 1. Nausea or Vomiting 2. Photophobia AND Phonophobia (can be inferred from behavior) Migraine with Aura Formerly classic migraine Aura: attack of a reversiblefocal neurological symptom, gradually over 5 mins, lasts < 1 hr ~15-30% of pediatric and 20% of adult migraineurshave visual disturbances, distortions, or obscurations Auras can occur without H/A ICHD Diagnostic Criteria for Aura A. 2 attacks fulfilling criteria B and C B. 1 of the following fully reversible aura symptoms: visual sensory speech and/or language motor brainstem retinal C. Two of four characteristics: 1 aura symptom spreads gradually over 5 mins, and/or 2 symptoms occur in succession Each individual aura symptom lasts 5 60 mins 1 aura symptom is unilateral Accompanied or followed within 60 mins by HA 2
ICHD Diagnostic Criteria for Aura A. 2 attacks fulfilling criteria B and C B. 1 of the following fully reversible aura symptoms: visual sensory speech and/or language motor brainstem retinal C. Two of four characteristics: 1 aura symptom spreads gradually over 5 mins, and/or 2 symptoms occur in succession Each individual aura symptom lasts 5 60 mins 1 aura symptom is unilateral Accompanied or followed within 60 mins by HA Other Diagnostic Considerations Chronic vs. Episodic Chronic Frequency 15 days/month Duration 3 months Features of migraine on 8 days/month Episodic Frequency <15 days/month With or without Aura Other Related Migraine Diagnosis 1.2.1.1 Typical aura with headache Headaches are not migraine 1.2.1.2 Typical aura without headache 1.2.2 Migraine with brainstem aura 2 of the following brainstem symptoms: 1. dysarthria 2. vertigo 3. tinnitus 4. hypacusis 5. diplopia 6. ataxia 7. decreased LOC 3
Other Related Migraine Diagnosis 1.2.3 Hemiplegic migraine Fully reversible aura of weakness does not need to be hemiplegic Needs to also have an additional typical aura symptom 1.2.4 Retinal migraine Repeated attacks of monocular visual disturbance scintillations, scotomata or blindness Complications of Migraine 1.4.1 Status migrainosus Debilitating migraine attack >72 hrs 1.4.2 Persistent aura without infarction Same as Migraine with aura, BUT persists for typical aura > 1 wk 1.4.3 Migrainous infarction Typical Aura with 1 aura symptoms persists >60 mins Neuroimaging: ischemic infarction Trigeminal Autonomic Cephalalgias Brief HAs of excruciating severity WITH prominent autonomic features Mostly occur in adulthood, but can occur earlier Cluster Headache 3.1 Paroxysmal Hemicrania 3.2 Short Lasting Unilateral Neuralgiform Headaches SUNCT (conjunctival injection and tearing) 3.3.1 SUNA (with cranial autonomic symptoms) 3.3.2 Hemicrania Continua 3.4 4
What if there is also another possible cause? HA appears to behave like a TAC but occurs in close temporal relation to another disorder known to cause headache Code: secondary headache attributed to the causative disorder Pre-existing TAC becomes chronic while a causative disorder is diagnosed Code: initial TAC diagnosis AND the secondary diagnosis Pre-existing TAC is made significantly worse (> 2x) with causative disorder Code: initial TAC diagnosis AND the secondary headache diagnosis Patient Case 45 yo man Right Eye popping 10/10 pain 2-4 episodes/day, usually lasting 2 hrs Q 10 mo, 6-8 weeks Pacing No N/V, Right eye waters Cluster Headache Males (3:1), onset 20 40 years Attacks-predictable times: alarm clock HA Often coming at the same time each day for a few weeks months each year Irritability, restlessness, pacing, mood concerns High comorbidity in heavy smokers Attacks provoked by alcohol, histamine or nitroglycerin Inheritance Pattern: AD in 5% PET Scan-ipsilateral posterior hypothalamic activation during attacks 5
ICHD Criteria: Cluster Headache (3.1) A. Min of 5 attacks B. Lasting 15 mins to 3 hrs (180min) - untreated Severe or very severe Strictly unilateral Orbital, supraorbital or temporal B. Associated ipsilateral autonomic: Conjunctival injection/lacrimation Nasal congestion/rhinorrhea Eyelid edema Forehead/facial sweating Forehead/facial flushing Sensation of fullness in the ear Miosis and/or ptosis C. Frequency: QOD to 8/day D. No evidence of another disorder Other Diagnostic Terms Attack: a particular headache episode Cluster Period: a series of attacks (can last wks - months) Remission Period: time between cluster periods (can last months - yrs) Episodic Cluster Headache (3.1.1) period 7 d - 1 yr Chronic Cluster Headache (3.1.2) period > 1 yr w/o remission, or < 1 mon remission Cluster Headache Treatment Attack Rescue: High Flow Oxygen SC Sumatriptan Indomethacin Stop the Cluster Period: Prednisone Course Occipital Nerve Block Long Cluster Period or Prevention: Verapamil, Lithium 6
Cluster Headache (aka Suicide Headache ) ICHD Criteria: Paroxysmal Hemicrania A. Min of 20 attacks B. Lasting 15 mins to 3 hrs (180min) - untreated Severe Strictly unilateral Orbital, supraorbital or temporal B. Associated ipsilateral autonomic (1 or more): Conjunctival injection/lacrimation Nasal congestion/rhinorrhea Eyelid edema Forehead/facial sweating Forehead/facial flushing Sensation of fullness in the ear Miosis and/or ptosis C. Frequency: QOD to 8/day D. No evidence of another disorder 2017 UpToDate 7
Comparison of Demographics 2017 UpToDate Pain Description Attack Characteristics 2017 UpToDate 8
Attack Characteristics 2017 UpToDate Treatment Options 2017 UpToDate Treatment for Paroxysmal Hemicrania and Hemicrania Continua Oral Indomethacin Trial Adults: PO dosing up 150mg/day, increased up to 225mg/day if needed Can try injection 100mg-200mg Can also try suppository or changing to liquid if oral pills not tolerated 9
Treatment Options 2017 UpToDate Thanks! The Headache Program at Wake Forest Baptist Health @StraussHeadache 10