Headache for the PCP: Evaluation and Initial Management Chris Jackman, MD Assistant Professor of Neurology Child Neurology of Riley Hospital Director, Riley Headache Center

Objectives Identify a systemic evaluation of a headache patient Evaluate for causes of secondary headache Recognize how to diagnose common primary headache symptoms of childhood Identify how to treat primary headache syndromes

Initial Evaluation

1. Shoulder shrug and look to parents 2. “I don’t know” 3. “Headaches

It’s in the history Time course Pain description Associated symptoms Location Severity Quality Associated symptoms

Other questions: Pain description Associated symptoms Location Severity Quality Associated symptoms Aura Nausea, vomiting Photophobia, phonophobia Light-headedness, vertigo Autonomic features

Red Flags Time course Location Postural Focal neurologic signs Progressive Morning Location Posterior Postural Focal neurologic signs Any Systemic signs Fevers, rash Family history As in, none Age Under 6 years

Physical exam Eyes / Fundus TMJ Face Muscles Skin Neurologic

Secondary Headaches

Non-neurologic causes of secondary headaches Dental/ TMJ Allergies/ congestion Sinus inflammation/ infection Ear infection/ Mastoiditis Hypothyroidism Pheochromocytoma (Hypertension) Eye-strain

It is (probably) not a tumor Brain tumors are very rare BUT… You only need to miss one to be incompetent The chance of finding a tumor in a patient with headaches and a normal neurological exam is…

It is not a tumor Very low, but not quite zero Brain tumors typically cause headache when they cause increased pressure A much more common presentation is focal neurologic signs with minor headache

It is a tumor Key features Time course (Progressive) Timing (On awakening) Postural (Supine) Focal Neurologic signs Seizures

If it’s not a tumor, what is it?

Intracerbral Hemorrhage Features Time course (Acute) History of trauma Focal Neurologic signs Types of hemorrhage Subdural Epidural Subarachnoid Paranchymal Interventricular

Venous sinus thrombosis Associated with primary or secondary hypercoagulable state Present with signs of increased intracranial pressure Sometimes hemorrhage Red Flags Time course (Progressive or static) Postural Neurologic signs Papilledema 6th nerve palsies

Ideopathic intracranial hypertension Mechanism unknown More female, more obese Headache with visual loss Red Flags Time course (Progressive or static) Postural Neurologic signs Papilledema 6th nerve palsies

Ideopathic intracranial hypotension Seen in some connective tissue diseases from dural ectasia (or ideopathic) Mimics LP headache Red Flags Time course (Progressive or static) Postural

Meningitis / Encephallitis Red flags: Systemic signs (fever) Focal Neurologic signs (meningismus, encephalopathy, seizures)

Chiari I Malformation Protrusion of cerebellar tonsils below the foramen of Monro Red flags: Location (posterior) Postural, pain with neck movements Focal Neurologic signs (ataxia) Worse with cough, sneezing, valsalva

Post-traumatic or Post-concussive Headache Red flags: See hemorrhage Will get better, may take months Cognitive changes are common, will also improve

Headache Evaluation

Do I order LABS?

Study of 104 children referred to Child Neurology Headaches in children younger than seven years of age Chu ML, Shinnar S. Arch Neurol, 49:1992; 79-82 Study of 104 children referred to Child Neurology Studies performed prior by the pediatrician Studies included: Cell counts Basic electrolytes Tranaminases Urinalysis “Uniformly unrevealing” Similar prospective study in adults of 193 patients showed same results

Do I order a SCAN?

American Academy of Neurology Practice Parameter: Evaluation of children and adolescents with recurrent headaches 2002 Neuroimaging Combined 6 studies 605 of 1275 had imaging (CT in 116, MRI in 483, both in 75) 97 children with imaging abnormalities (16%) 79 considered incidental 14 surgically treatable 4 medically treatable

American Academy of Neurology Practice Parameter: Evaluation of children and adolescents with recurrent headaches 2002 Of the 14 surgical lesions: 10 tumors 3 symptomatic vascular malfomations 1 significant arachnoid cyst All had an abnormal neurologic examination Papilledema Abnormal eye movements Motor dysfunction Gait dysfunction

American Academy of Neurology Practice Parameter: Evaluation of children and adolescents with recurrent headaches 2002 Parameters which distinguish headache patients with space occupying lesions Headache of less than one month duration Absence of a family history of migraine Abnormal neurological examination Gait abnormalities Seizures Those patients with headaches for less than 6 months and at least one of the above symptoms are considered “high-risk” “High-risk” = 4% chance of space occupying lesion

CT vs. MRI?

Primary Headache Disorders

48% had previously received a physician diagnosis Migraine Diagnosis and Treatment: Results From the American Migraine Study II Headache 2001;41:638-645 Survey mailed to 20,000 homes, identified 3577 individuals who met criteria for migraine 48% had previously received a physician diagnosis 24% of those undiagnosed had missed at least one day of work or school in the previous three months Those missed were: Lower income Younger age (18-29) Male

Migraine epidemiology Headache prevalence Tension type HA 78% Migraine 16% Children 3-8% by age 3 37-52% by age 7 57-82% in 7-15 year olds Peak incidence Women – age 12-13 (aura), 14-17 (without) Men – age 5 (aura), 10-11 (without) Comprehensive Review of Headache Medicine; Levin M Ed; Oxford 2008

“If nothing is wrong with me, doctor, why do I have these headaches?”

Migraine pathophysiology Primarily a NEUROGENIC process We think For now

Migraine pathophysiology Aura Cortical spreading depression Front of profound depolarization Moves across cortex ~ 3mm/min Following by suppression of neural activity lasting minutes A.P. Leão.

Cortical Spreading Depression

Migraine pathophysiology

Migraine without aura Pediatric diagnostic criteria At least five attacks fulfilling criteria B-D (below) Headache attacks lasting 1 to 72 h Headache having at least two of the following characteristics: Unilateral location, may be bilateral, frontotemporal (not occipital) Pulsing quality Moderate or severe pain intensity Aggravation by or causing avoidance of routine physical activity (eg, walking, climbing stairs) During the headache, at least one of the following: Nausea or vomiting Photophobia and phonophobia, which may be inferred from behavior Not attributed to another disorder

Migraine with aura Pediatric diagnostic criteria At least two attacks fulfilling the criteria B-D (below) Aura consisting of at least one of the following, but no motor weakness: Fully reversible visual symptoms, including positive features or negative features (e.g., flickering lights, spots, or lines) Fully reversible sensory symptoms, including positive features (i.e., pins and needles) or negative features (ie, numbness) Fully reversible dysphasic speech disturbances At least two of the following: Homonymous visual symptoms or unilateral sensory symptoms At least one aura symptom develops gradually over 5 min or different aura symptoms occur in succession over 5 min Each symptom lasts between 5 min and 60 min Not attributable to another disorder

And…

…Chronic Daily Headache…

Chronic Daily Headache Transformed (or chronic) migraine History of migraine Progresses to chronic, low level headache with periodic migraines Chronic tension type headache Lack significant migranous features Less severe intensity Tightening more than pulsating New daily persistent headache

Chronic daily headaches - evaluation Look for red flags* Ask about analgesic overuse * Especially in New Daily Persistent Headache

Practice Parameter: Pharmacological treatment of migraine headache in children and adolescents D. Lewis, MD; S. Ashwal, MD; A. Hershey, MD; D. Hirtz, MD; M. Yonker, MD; and S. Silberstein, MD NEUROLOGY 2004; 63: 2215–2224

Migraine treatment - Abortive Ibuprofen, acetaminophen, ketorolac, indomethacin, ASA Combinations (Acetaminophen/ASA/caffeine) Antiemetics (promethazine, chlorpromethazine Opiates, barbituates (no, no, never…) Corticosteroids Triptans 5HT1b, 1d, and 1f agonists Contraindications include cardiovascular disease or risk factors, Reynaud’s, hemiplegic migraine Side effects include nausea, dizziness, chest and throat tightness

Migraine treatment - Abortive

Migraine treatment - Prophylactic When to use prophylaxis Headaches frequent Headaches severe Headaches disruptive Side effects and burden of taking a daily medicine < the life disruption caused by (appropriately treated) headaches

Migraine treatment - Prophylactic Antihistamines Beta-blockers Tricyclics Anticonvulsants Calcium channel blockers

Migraine treatment - Prophylactic Antihistamines Cyproheptadine Little studied, often used Reduce headaches from 8.4 to 3.7 per month Somnolence, weight gain Initial dose 1-2 mg QHS, max 4 mg BID Lewis D, Diamond S, Scott D, et al. Prophylactic treatment of pediatric migraine. Headache 2004;44:230–237.

Migraine treatment - Prophylactic Beta-blockers Propranolol most studied Three small, prospective class II studies with conflicting results Exercise intolerance Contraindicated in asthma, depression Initial dose 20 mg, up to 160 mg

Migraine treatment - Prophylactic Tricyclics Amitriptyline most studied Anticholinergic effects, somnolence Black box warning re: suicidality Baseline EKG and monitor for QT prolongation Initial dose 10 mg up to 100 mg Give at dinner

Migraine treatment - Prophylactic Anticonvulsants Topiramate (or zonisamide) Best studied Valproate Effective but side effects can be significant Levetiracetam/ Lamotrigine Limited (poor) data

Migraine treatment - Prophylactic Calcium channel blockers Conflicting data Familial hemiplegic migraine Abdominal discomfort Monitor EKG and blood pressure

Chronic Daily Headache - Treatment Preventative medications – Evidence is spotty at best Topiramate is best studied, anecdotally all migraine medications may work Transformed migraine or for medication overuse – early prophylactic treatment Chronic tension type headache – late medical treatment New daily persistent headache – doesn’t matter

Non-pharmacologic Treatment Lifestyle! Lifestyle! Lifestyle! Analgesic overuse Sleep Diet Psychiatric

Non-pharmacologic Treatment Analgesic overuse Opiotes/ barbiturates > triptans >>NSAIDS Any used over 15 days/month, some over 10 days/month Can treat by a period of elimination or by moderation Headaches may take 4-6 weeks to improve

Non-pharmacologic Treatment Sleep Snoring Movements Quality Quantity Continuity

Non-pharmacologic Treatment Diet Meats (Iron, B12) Vegetables (Folate?) Skipping meals Hydration Caffeine

Non-pharmacologic Treatment Psychiatric evaluation Anxiety Depression Obsessive-compulsive disorder Non-pharmocologic management Biofeedback Self-hypnosis Relaxation

Take home points: Red flags Progressive time course Postural Worse in the morning Any neurologic sign or symptom Worse with valsalva Practice your fundoscopic and cranial nerve exam

Closing thoughts… Watch for red flags Know when to image If unsure whether to image, refer Know helpful lifestyle modifications Know when to start or refer for prophylactic medications Remember: “Your patient does not want to have a headache”

References Sargent JD, Solbach P. Medical evaluation of migraineurs: review of the value of laboratory and radiologic tests; Headache 1983; 23:62-65 Chu ML, Shinnar S. Headaches in children younger than seven years of age Arch Neurol, 49; 1992; pp79-82 Maytal J, Robert S. Bienkowski, Patel M and Eviatar L. The Value of Brain Imaging in Children With Headaches. Pediatrics 1995;96;413-416 Levin M Ed; Comprehensive Review of Headache Medicine: Oxford 2008 Lewis D, Ashwal, S; Hershey A; Hirtz D; Yonker, M; and Silberstein S, Practice Parameter: Pharmacological Treatment of migraine headache in children and adolescents. Neurology 2004;63:2215–2224 Ludvigsson J. Propranolol used in prophylaxis of migraine in children. Acta Neurol 1974;50:109–115. Forsythe WI, Gillies D, Sills MA. Propranolol (Inderal) in the treatment of childhood migraine. Dev Med Child Neurol 1984;26:737–741. Olness K, MacDonald JT, Uden DL. Comparison of self-hypnosis and propranolol in the treatment of juvenile classic migraine. Pediatrics 1987;79:593–597. D.W. Lewis, MD; S. Ashwal, MD; G. Dahl, BS; D. Dorbad, MD; D. Hirtz. Practice parameter: Evaluation of children and adolescents with recurrent headaches: Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2002;59:490–498