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The Acute , a Clinical Update and Literature Review Bryan Malcolm, Capt, USAF, PA-C, EMPA Fellow Overview • Background • The Headache Spectrum • Approach to the Acute Headache • High Risk Features • Review of Specific Headache Syndromes Significance • A top five Emergency Department presenting complaint • Accounts for over 2 million visits per year • Affects people across all ethnic groups, geographical locations, and economic levels The Benign Headache Spectrum

headache

• Cluster headache Non-benign, “Killers” • / • Retropharyngeal abscess • Subarachnoid hemorrhage/sentinel bleed • Acute obstructive • Space occupying lesions • Cerebral vascular accident • Carbon monoxide poisoning • Basilar artery dissection • Preeclampsia • Cerebral venous thrombosis Maimers • Giant cell arteritis of temporal artery (temporal arteritis) • Idiopathic intracranial hypertension (Pseudotumor Cerebri) • Acute Angle Closure Glaucoma • Cavernous sinus thrombosis or cerebral sinus thrombosis Others • • TMJ pain • Post-lumbar puncture headache • Dehydration • Analgesia abuse • Infectious (ocular, dental, zoster, cryptococcus infections) • Febrile headache (e.g. pyelonephritis, nonspecific viral infection) • Ophthalmoplegic migraine Approach to the Acute Headache

• Identify patients at risk for rapid deterioration

• Rapid identification of high-risk features associated with a headache

• Provide appropriate headache therapy and disposition Approach to the Acute Headache

Identify patients at risk for rapid deterioration

• The very young patient

• The older patient

• The immunocompromised patient High Risk Headache Features

• Sudden onset • Onset during exertion • Altered mental status • • Neurologic deficits • Fever • Visual changes High Risk Headache Features

Past Medical History • No prior headache • Change in headache quality • Pregnancy or post-pregnancy status • Systemic lupus erythematous • Behcet’s disease • Sarcoidosis • Cancer Headache Clues By History

• Substance Use History • Cocaine, amphetamine, methamphetamine carry risk of intracranial hemorrhage • abuse carries risk of intracranial bleeding secondary to falls, interpersonal violence and liver dysfunction • Family History • First degree relative with history of aneurysm or family history of polycystic kidney disease Objective Data

• Review vitals • Temp, blood pressure • Head and neck exam • Cranial nerve testing • Testing for neck stiffness and pain • exam • Visual acuity, extraocular movements, visual field testing • Fundoscopic exam • Papilledema • Neurologic exam • Strength, DTRs, special tests Objective Data

• Review vitals • Temp • Head and neck exam • Cranial nerve testing • Testing for neck stiffness and pain • Eye exam • Visual acuity, extraocular movements, visual field testing • Fundoscopic exam • Papilledema • Neurologic exam • Strength, DTRs, special tests Objective Data

• Review vitals • Temp • Head and neck exam • Cranial nerve testing • Testing for neck stiffness and pain • Eye exam • Visual acuity, extraocular movements, visual field testing • Fundoscopic exam • Papilledema • Neurologic exam • Strength, DTRs, special tests Benign Headache Spectrum Migraine Headache

• Presentation: • POUNDing – meet 4 or 5 criteria, likelihood ratio of migraine is 24 • Pulsatile quality • Onset/duration of 4-72 hours • Unilateral • Nausea or Vomiting • Disabling in quality Migraine Headache

• History • History of prior headache • Family history – 2-4 fold increased risk of migraine headache when has a first degree family member who has migraine • Start in childhood and peak around age 40 • Labs • HCG (all female patients with child bearing capacity) Migraine Management

• 2012 Systematic review • Looks at effectiveness of common medications given for migraine headaches as documented in prior studies Migraine Management • • Not as effective as first-line treatments • Renders acute migraine treatment medications, such as , less effective • Potential to promote chronic migraine headaches • When used as first-line treatment lead to increased risk of relapse • ACEP 2019 recommendation level A, “Preferentially use nonopioid medications in the treatment of acute primary headaches” Migraine Management • Dopamine Receptor Antagonists Migraine Management • Serotonin Receptor Agonists Migraine Management • Derivatives Migraine Management • NSAIDs Migraine Management • Antiepileptics Tension Headache

• Presentation: • Bilateral non-pulsating pain in a band-like or vice distribution around the head • Lasting 30 minutes to seven days • Features • Bilateral location • Described as pressure or tightening • Mild to moderate intensity • Not aggravated by physical activity • No nausea or vomiting • May have or phonophobia but not both • Management • NSAIDs Cluster Headache

• Presentation: • Typically occurring in middle aged men • Unilateral excruciating • Need for the patient to “pace” • Ipsilateral symptoms • History • Recurrent, brief, self limiting headaches • Recurring for greater than one week and remitting for at least four weeks • Circadian and circannual pattern • Management • 100% at 12L/min via non-rebreather for 15 min • 6mg Subcutaneously The Headache Continuum

• Decades of discussion • Significance • Treating the undifferentiated headache Headache Treatment Adjuncts

• Fluids • Common practice • Dehydration established trigger for migraine headaches

• 2018 pilot randomized control trial with number of 49 patients Headache Treatment Adjuncts

• Benadryl

• 2015 randomized, double-blind clinical trial with a number of 208 patients (planned for 374 patients) • One hour after medication administration, those randomized to diphenhydramine improved by a mean of 5.1 on the 0 to 10 scale versus 4.8 for those randomized to placebo • “Intravenous diphenhydramine, when administered as adjuvant therapy with metoclopramide, does not improve migraine outcomes.” • Rates of side effects, including , were comparable between the groups. Headache Treatment Adjuncts • Oxygen as an adjunct?

Randomized, crossover-design, placebo-controlled trial (oxygen vs “medical air”) Number of 22 enrolled self treating 64 attacks. Non-benign Headache, “Killers” Meningitis

• Presentation: • Classic triad of altered mental status, fever, neck stiffness • May have photophobia, vomiting, focal neurologic deficits, • History • Immunosuppression • Prodrome upper respiratory infection • Recent pneumonia, otitis media, or neurosurgical procedure • Evaluation • PE: Nuchal rigidity, Kernig’s sign, Brudzinski’s sign • Labs: CBC, chemistry, blood culture, spinal fluid analysis • Imaging: Chest x-ray, CT Head (before lumbar puncture) Meningitis • Lumbar puncture • Do not delay giving antibiotics if meningitis is suspected • If your patient is awake, alert, without focal neurologic deficits and papilledema and no history to suggest immunocompromised state or new onset seizures, CT head can be delayed until after LP. • Management • Bacterial: • Less than 1 month old - Ampicillin with Gentamycin or Cefotaxime • Greater than one month - Ceftriaxone with Vancomycin • Greater than 50 yo or immunocompromised - Ceftriaxone, Vancomycin, and Gentamycin • Dexamethasone • Viral – Consider Acyclovir • Disposition • Admit Subarachnoid Hemorrhage • Presentation: • Sudden onset of severe headache (usually maximal intensity within minutes) • 11-25% of patients with “thunderclap” headache will have SAH • History • Genetics – Polycystic kidney disease, Ehler-Danlos, family history • Hypertension, Atherosclerosis, Smoking, alcohol, age greater than 50, Cocaine, Estrogen deficiency • Evaluation • CT head – Sensitivity depends on time from onset of symptoms • If CT is within 6 hours of onset, negative predictive value is 99.4% and positive predictive value is 100% (91% at 24 hours, 50% at one week) • High suspicion with negative CT, next get LP • CT Angiogram – In place of LP. 98% sensitivity for aneurysm greater than 3mm • Ottawa SAH rules – 100% sensitive to rule out SAH

ACEP Clinical Policy 2019 ACEP Clinical Policy 2019 • In the adult ED patient presenting with acute headache, does a normal noncontrast head CT scan performed within 6 hours of headache onset preclude the need for further diagnostic workup for SAH? • Level B recommendations. Use a normal noncontrast head CT performed within 6 hours of symptom onset in an ED headache patient with a normal neurologic examination, to rule out nontraumatic SAH. • Level B recommendations. Recommendations for patient care that may identify a particular strategy or range of strategies that reflect moderate clinical certainty (eg, based on evidence from 1 or more Class of Evidence II studies or strong consensus of Class of Evidence III studies). ACEP Clinical Policy 2019 • In the adult ED patient who is still considered to be at risk for SAH after a negative noncontrast head CT, is CTA of the head as effective as LP to safely rule out SAH? • Level C recommendations. Perform LP or CTA to safely rule out SAH in the adult ED patient who is still considered to be at risk for SAH after a negative noncontrast head CT result. • Level C recommendations. Recommendations for patient care that are based on evidence from Class of Evidence III studies or, in the absence of adequate published literature, based on expert consensus. In instances in which consensus recommendations are made, “consensus” is placed in parentheses at the end of the recommendation. Subarachnoid Hemorrhage

• Management: • Avoid hypotension (maintain MAP greater than 80) • Maintain systolic less than 160 to 180 • Discontinue/reverse anticoagulation • Nimodipine (Improved outcomes – decreases incidence of delayed cerebral ischemia) • Seizure prophylaxis • Elevated head of bed • Neurosurgery eval • Disposition • Admit Brain Tumor

• Presentation: • Headache with valsalva or headache that wakes patient at night • Nausea and vomiting • Focal neurologic deficits/weakness • History • New onset seizures • Cancer diagnosis • Mental status change Brain Tumor • Evaluation: • Physical exam: • Cranial nerve palsies • Weakness • Abnormal reflexes • Papilledema • Cushing’s triad: Bradycardia, hypertension, irregular respirations (brainstem herniation) • CT head • MRI brain • Management: • (reduction of vasogenic edema) • Elevated head of bed • Anti-epileptic medications – use to treat seizures; prophylactic not recommended • Neurosurgery consult Giant Cell Arteritis

• Presentation: • Headache with gradual worsening over days • Headache is worse at night • Usually unilateral • Higher incidence in women • Age 50-70 • History • May have weight loss • May have myalgias (polymyalgia rheumatica) • May have vision loss • May have jaw claudication Giant Cell Arteritis

• Evaluation: • Tender, pulseless temporal artery on exam • Elevated ESR (greater than 50) • GCA is a clinical diagnosis however biopsy is obtained to confirm diagnosis • Management • Goal is to protect and minimize vision loss • High dose corticosteroids • 40-60mg daily (typically 1-2 years of treatment) Review • Background • The Headache Spectrum • Approach to the Acute Headache • High Risk Features • Review of Specific Headache Syndromes References • Arca, Karissa N., and Rashmi B. Halker Singh. “The Hypertensive Headache: A Review.” Current Pain and Headache Reports, vol. 23, no. 5, Mar. 2019, p. 30. Springer Link, doi:10.1007/s11916-019-0767-z. • Becker, Werner J. “Cluster Headache: Conventional Pharmacological Management.” Headache: The Journal of Head and Face Pain, vol. 53, no. 7, July 2013, pp. 1191–96. DOI.org (Crossref), doi:10.1111/head.12145. • Friedman, Benjamin W., et al. “Diphenhydramine as Adjuvant Therapy for Acute Migraine. An ED-Based Randomized Clinical Trial.” Annals of Emergency Medicine, vol. 67, no. 1, Jan. 2016, pp. 32-39.e3. PubMed Central, doi:10.1016/j.annemergmed.2015.07.495. • Gelfand, Amy A., and Peter J. Goadsby. “A Neurologist’s Guide to Acute Migraine Therapy in the Emergency Room.” The Neurohospitalist, vol. 2, no. 2, Apr. 2012, pp. 51–59. PubMed Central, doi:10.1177/1941874412439583. • Godwin, Steven A., et al. “Clinical Policy: Critical Issues in the Evaluation and Management of Adult Patients Presenting to the Emergency Department With Acute Headache.” Annals of Emergency Medicine, vol. 74, no. 4, Oct. 2019, pp. e41–74. DOI.org (Crossref), doi:10.1016/j.annemergmed.2019.07.009. • Intravenous Fluid for the Treatment of Emergency Department Patients With Migraine Headache: A Randomized Controlled Trial - ScienceDirect. https://www-sciencedirect- com.ezproxy.baylor.edu/science/article/pii/S0196064418312629. Accessed 11 Feb. 2020. • Jones, Christopher W., et al. “Epidemiology of Intravenous Fluid Use for Headache Treatment: Findings from the National Hospital Ambulatory Medical Care Survey.” The American Journal of Emergency Medicine, vol. 35, no. 5, May 2017, pp. 778–81. ScienceDirect, doi:10.1016/j.ajem.2017.01.030. • Mehndiratta, Manmohan, et al. “Appraisal of Kernig’s and Brudzinski’s Sign in Meningitis.” Annals of Indian Academy of , vol. 15, no. 4, 2012, pp. 287–88. PubMed Central, doi:10.4103/0972-2327.104337. • Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 8e | AccessMedicine | McGraw-Hill Medical. https://accessmedicine-mhmedical-com.ezproxy.baylor.edu/book.aspx?bookid=1658. Accessed 5 Feb. 2020. Questions? Other Important Headache Causes Idiopathic intracranial hypertension •

Acute Glaucoma •

Cavernous Sinus Thrombosis •