Headache & Facial Pain Vincent T. Martin, MD, FACP Co-director of the Headache & Facial Pain Center Professor of Medicine University of Cincinnati

Primary vs. Secondary Headache Disorders Migraine Tension-type Headache Trigeminal Autonomic Cephalalgias Other Primary Headache Disorders Headaches that arise as a result of another disorder

Worrisome Headache Red Flags—“SNOOP” LDER: new onset and progressive headache, especially in middle age >50 yr (giant cell arteritis) YSTEMIC SYMPTOMS (fever, weight loss) or ECONDARY RISK FACTORS (HIV, systemic cancer) EUROLOGIC SYMPTOMS or abnormal signs (confusion, impaired alertness or consciousness) NSET: sudden, abrupt, or split-second REVIOUS HEADACHE HISTORY: first headache or different (change in attack frequency, severity, or clinical features) Worrisome Headache Red Flags–”SNOOP” An attempt to elicit and exclude these worrisome features should be part of every new-headache evaluation because their presence may signify an underlying pathologic condition for which diagnostic testing is obligatory. Systemic symptoms, such as fever, malaise, or weight loss, should suggest an underlying infectious or systemic inflammatory disorder. Newly acquired neurologic signs or symptoms should always raise concern. The mode of onset is perhaps the most important characteristic of a headache to be delineated. Patients who have a sudden or abrupt headache that peaks in seconds or minutes require careful assessment to exclude causes such as subarachnoid hemorrhage (SAH), venous sinus thrombosis, arterial dissection, or raised intracranial pressure. Any new or progressive headache that begins in middle age or any headache that deviates significantly from a previous pattern should be investigated further. If these features are addressed, the chance of overlooking a sinister cause for headache is greatly diminished. Silberstein SD, Lipton RB, Dalessio DJ. Overview, diagnosis, and classification. In: Silberstein SD, Lipton RB, Dalessio DJ, eds. Wolff’s Headache and Other Head Pain. 7th ed. Oxford, UK: Oxford University Press; 2001:20. Dodick D. Adv Stud Med. 2003;3:S550-S555

Secondary Headache Disorders

Thunderclap Headache Headaches with the following characteristics: reaches peak intensity in < 60 seconds Very severe intense pain May represent subarachnoid hemorrhage or sentinel bleed Other etiologies- RCVS, dural venous thrombosis, pituitary apoplexy, crash migraine, carotid artery dissection Dodick D. J Neurol Neurosurg Psych 72: 6-11, 2002

Approach to Patient Thunderclap or “Worst Headache” Non contrast CT If negative (CT can miss 7% within 24 hours) Lumbar Puncture If negative and suspicion high Cerebral Arteriogram or MRA

Headache attributed to cervical artery dissection ICHD-3 Code 6.4 A. Any new headache or facial pain attributed to cervical artery dissection B. Cervical artery dissection (CAD) has been diagnosed C. Evidence of causation demonstrated by at least two of the following: 1) pain has developed in close temporal relationship to other local signs of cervical artery dissection, or has led to the diagnosis of cervical artery dissection 2) either or both of the following: a) pain has significantly worsened in parallel with other signs of the cervical artery lesion, b) pain has significantly improved or resolved within one month of its onset 3) either or both of the following: a) pain is severe and continuous for days or longer, b) pain precedes symptoms of acute retinal and/or cerebral ischemia 4) pain is unilateral and ipsilateral to the affected cervical artery E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 700

Horner’s Syndrome

Carotid Dissection

Headache attributed to low cerebrospinal fluid pressure ICHD-3 Code 7.2 A. Any headache fulfilling criteria C B. Low CSF pressure (< 60 mm CSF) and/or evidence of CSF leakage on imaging C. Headache has developed in temporal relationship to the low CSF pressure or CSF leakage, or led to it discovery D. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 715-6

Dural Enhancement

Headache attributed to idiopathic intracranial hypertension ICHD-3 Code 7.1.1 A. Any headache fulfilling criteria C B. Idiopathic intracranial hypertension has been diagnosed, with CSF pressure > 250 mm CSF (measured by lumbar puncture performed in the lateral decubitus position, without sedative medications or by epidural or intraventricular monitoring) C. Evidence of causation by at least two of the following: 1) headache has developed in temporal relation to the IIH or led to its discovery, 2) headache is relieved by reducing intracranial hypertension, 3) headache is aggravated in temporal relation to increase in intracranial pressure D. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 715

Papilledema

Treatment of Choice Acetazolamide Topamax Optic nerve fenestration Shunts

Trigeminal Neuralgia ICHD-3 Code 13.1 A. At least three attacks of unilateral facial pain fulfilling criteria B and C B. Occurring in one or more divisions of the trigeminal nerve, with no radiation beyond the trigeminal nerve C. Pain has at least three of the four following characteristics: 1. Recurring in paroxysmal attacks lasting from a fraction of a second to 2 minutes, 2. severe intensity, 3. electric shock-like, shooting, stabbing or sharp in quality, 3. precipitated by innocuous stimuli to the affected side of the face D. No clinically evident neurological deficit E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 774

Treatment of choice Carbamezepine Other neuromodulators Surgical options Microvascular decompression GAMA knife Ablative procedures

ICHD-3 Beta Criteria for Medication Overuse Headache A. Headaches ≥ 15 days/month for 3 months B. Overuse of abortive meds for >3 months of one of following: Ergotamine, triptans, opioid or combination analgesics on ≥ 10 days per month Simple analgesics on ≥ 5 days per month C. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 733

Persistent headache attributed to traumatic injury to the brain ICHD-3 Code 5.2 A. Unilateral headache fulfilling criteria C and D B. Traumatic injury to the head has occurred C. Headache is reported to have developed within 7 days after one of the following: 1. the injury to the head, 2. regaining consciousness following the injury to the head, 3. discontinuation of medication that impair ability to sense or report headache following the injury D. Headache persists for >3 months after the injury to the head E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 688

Headache attributed to intracranal neoplasia ICHD-3 Code 7.4 A. Any headache fulfilling criteria C B. Intracranial neoplasia has been diagnosed C. Evidence of causation demonstrated by at least one of the following: 1) headache has developed in temporal relation to the intracranial neoplasm or led to its discovery, 2) headache has significantly worsened in parallel with worsening of the intracranial neoplasia, 3) headache has significantly improved in temporal relationship to successful treatment of the intracranial neoplasia D. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 719-720

Characteristics of Headaches Headache only occur in 36-86% Resemble tension-type headaches in 77% and migraine in 9% Pain is intermittent and resolves over several hours Unilateral in 25-30% Unilateral predicts ipsilateral malignancy The Headaches, 2nd edition, pp 851-852.

Primary Headache Disorders

Migraine without aura ICHD-3 Code 1.1 A. At least five attacks fulfilling criteria B-D B. Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated) C. Headache has at least two of the following four characteristics: 1. unilateral location 2. pulsating quality 3. moderate to severe pain intensity 4. aggravation by or causing avoidance of routine physical activity (walking or climbing stairs) D. During the headache at least one of the following: 1. nausea and/or vomiting 2. photophobia and phonophobia (both) E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 644

Migraine with typical aura ICHD-3 Code 1.2.1 A. At least two attacks fulfilling criteria B and C B. Aura consisting of visual, sensory and or speech/language symptoms, each fully reversible, but no motor, brainstem or retinal symptoms C. At least two of the following characteristics: 1. at least one aura symptom spreads gradually over ≥ 5 minutes, and/or two or more symptoms occur in succession 2. each individual aura symptom lasts 5-60 minutes 3. at least one aura symptom is unilateral 4. the aura is accompanied, or followed within 60 minutes by headache D. Not better accounted fro by another ICHD-3 diagnosis, and transient ischemic attack has been excluded ICHD-3 Beta. Cephalalgia 2013; 33: 647

Visual Aura Adapted from Lashley KS. Arch Neurol Psychiatry. 1941;46:331-339. 27

Chronic Migraine ICHD-3 Beta. Cephalalgia 2013; 33:650 ICHD-3 Code 1.3 Headache (tension-type like and/or migraine-like) on ≥ 15 days per month for > 3 months and fulfilling criteria B and C B. Occurring in a patient with at least five attacks fulling criteria for migraine with or without aura (1.1 and 1.2) C. On ≥ 8 days per month for > 3 months, fulfilling any of the following: 1. Criteria C and D for migraine without aura a. unilateral, throbbing, mod/severe intensity, worse with exertion (any 2) b. nausea/vomiting or both photo/phonophobia (any 1) 2. Criteria B and C for migraine with aura a. reversible aura without motor, brainstem or retinal symptoms b. gradual spread ≥ 5 minutes, duration 5-60 min, symptoms unilateral, headache within 60 minutes 3. Believed to be migraine and relieved by triptan or ergot D. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33:650

American Headache Society Evidence Assessment for Abortive Medications Level of Evidence Examples Level A Analgesic Ergots NSAIDS Combinations Opioids Triptans Acetaminophen 1000 mg DHE Nasal Spray 2 mg ASA 500 mg, Diclofenac 50/100 mg, Ibuprofen 200/400 mg, Naproxen 500/550 mg Acetaminophen/ASA/Caffeine 500/500/130 mg, Sumatriptan/naproxen 85/500 mg Butorphanol nasal spray 1 mg Almotriptan 12.5 mg, Eletriptan 20/40/80 mg, Naratriptan 1/2.5 mg, Rizatriptan 5/10 mg, Sumatriptan 25/50/100 mg tabs, 0/20 mg NS, 4/6 mg sq, Zolmitriptan 2.5/ 5 mg tabs, 2.5/5 mg NS Marmura M. Headache 2015; 55: 3-20

Classification of Migraine Preventatives Level of Evidence Examples Level A Antiepileptic Drugs Beta Blockers Topiramate, Divalproex Sodium , Sodium Valproate Propranolol, Metoprolol, Timolol Level B Antidepressants Amitriptyline, Venlafaxine Atenolol, Nadolol Level C ACE Inhibitors ARBs α-Agonists Beta-blockers Antihistamines Lisinopril Candesartan Clonidine, Guanfacine Carbamazepine Nebivolol Cyproheptadine Silberstein S. Neurology 2012; 78: 1337-1345

Infrequent and Frequent Episodic Tension- type headache ICHD-3 Codes 2.1 and 2.2 At least 10 episodes of headache occurring on the following: 1. Infrequent episodic: <1 day per month on average 2. Frequent epidodic: 1-14 days per month on average B. Lasting 30 minutes to 7 days C. At least two of the following four characteristics: 1. bilateral location 2. pressing or tightening quality 3. mild or moderate intensity 4. not aggravated by routine physical activity such as walking or climbing stairs D. Both of the following: 1. no nausea or vomiting, 2) no more then one of photo/phonophobia E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 660

Treatments of Choice Acute Preventative Acetaminophen, ASA, NSAIDS Amitriptyline

Cluster headache ICHD-3 Code 3.1 A. At least five attacks fulfilling criteria B-D B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes C. Either or both of the following: 1. At least on of the following symptoms: a) conjunctival injection and/or lacrimation, b) nasal congestion and/or rhinorrhea, c) eyelid edema, d) forehead and facial sweating, e) forehead and facial flushing, f) sensation of fullness of the ear, g) miosis and/or ptosis 2. A sense of restlessness or agitation D. Attacks have a frequency between one every other day and eight per day for more that half of the time when the disorder is active E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 665-666

Autonomic Features Sympathetic hypofunction Partial Horner’s syndrome Miosis Nasal Congestion Parasympathetic hyperfunction Lacrimation Rhinorrhea Clinical Symposisa 1981; 33 (20): p 13

Treatments Acute Acute Transitional therapy Prevention Oxygen Intranasal or parenteral triptans Acute Transitional therapy Prednisone Prevention Verapamil

Primary Stabbing Headache ICHD-3 Code 4.7 A. Head pain occurring spontaneously as a single stab or series of stabs and fulfilling criteria B-D B. Each stab lasts for up to a few seconds C. Stabs recur with irregular frequency, from one to many per day D. No cranial autonomic symptoms E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 677

Primary Cough Headache ICHD-3 Code 4.1 A. At least two headache episode fulfilling criteria B-D B. Brought on by and occurring only in association with coughing, straining and/or other Valsalva maneuver C. Sudden Onset D. Lasting between 1 second and 2 hours E. Not better accounted for by another ICHD-3 diagnosis ICHD-3 Beta. Cephalalgia 2013; 33: 673

Treatment of Choice Indomethacin