1. A Case of Headache Scot Hill, MD Associate Medical Director Mount Sinai School of Medicine Department of Emergency Medicine New York, New York

2. Critical Questions in the ED Management of HA What is first line therapy for the treatment of HA? What is first line therapy for the treatment of HA? Does a response to headache pain therapy predict the underlying etiology of the HA? Does a response to headache pain therapy predict the underlying etiology of the HA? Which patients with an acute headache require neuroimaging in the ED? Which patients with an acute headache require neuroimaging in the ED? What are the indications for a lumbar puncture in the patient with an acute headache? What are the indications for a lumbar puncture in the patient with an acute headache?

3. ED VISIT CC: “I have a severe migraine” HPI: 32 year old female complained of a sudden, acute onset vertex headache radiating into her neck for 3 hours associated with nausea and lightheadedness. Similar headache 5 days prior that resolved with naprosyn.

4. ED VISIT Past history of migraines with aura: scintillating lights followed by nausea and right temporal throbbing headache Past history of migraines with aura: scintillating lights followed by nausea and right temporal throbbing headache Present headache was different in intensity, onset, and location Present headache was different in intensity, onset, and location

5. ED VISIT #1 PMH: Migranes Q-month MEDS: Naprosyn PRN; BCP LNMP: 7 Days prior SH: No Tob / ETOH / drugs FH:Mother - Migraines

6. ED VISIT Appearance: 32 year old female, alert, cooperative but appeared uncomfortable, holding the top of her head VSS: 118/76, 72, 16, 98.6 Head: Atraumatic Neck: Nontender, supple Heart:Regular, no murmurs, no clicks Lungs:Clear Abdomen:Soft, nontender

7. ED VISIT MS: Alert; Oriented X 3 PUPILS:Not documented CN:”Intact” GAIT:”Normal”

8. A diagnosis of migraine was made. Which of the following is your drug of choice in treating acute severe migraine? A Opioid (Meperidine or morphine) B Nonsteroidal (Ketorolac) C Sumitriptan D DHE E Prochlorperazine

9. Migraine: Pathophysiology Common pathway for headache pain regardless of the underlying etiology Common pathway for headache pain regardless of the underlying etiology Headache pain is transmitted via the trigeminal nerve Headache pain is transmitted via the trigeminal nerve Trigeminovascular axon stimulation results in a release of neurogenic peptides stored in the afferent C fibers innervating cephalic blood vessels Trigeminovascular axon stimulation results in a release of neurogenic peptides stored in the afferent C fibers innervating cephalic blood vessels Vasoactive neuropeptides mediate an inflammatory cascade, “neurogenic inflammation” Vasoactive neuropeptides mediate an inflammatory cascade, “neurogenic inflammation” Vasodilatation and enhanced permeability of plasma proteins result in a perivascular reaction Vasodilatation and enhanced permeability of plasma proteins result in a perivascular reaction

10. Migraine: Pathophysiology Serotonin receptors modulate neurogenic peptide release and cause vasoconstriction Serotonin receptors modulate neurogenic peptide release and cause vasoconstriction Goal of migraine therapy is to abort the neurogenic peptide release Goal of migraine therapy is to abort the neurogenic peptide release 5-HT 1c receptor is most involved in mediating headache 5-HT 1c receptor is most involved in mediating headache Drugs working at the 5-HT receptor are the preferred therapy for headache Drugs working at the 5-HT receptor are the preferred therapy for headache Narcotics cause initial pain relief but result in vasodilatation with a high incidence of rebound Narcotics cause initial pain relief but result in vasodilatation with a high incidence of rebound

11. Migraine Therapy First line agents: Prochlorperazine 5-10 mg IV First line agents: Prochlorperazine 5-10 mg IV Metoclopramide Metoclopramide Chlorpromazine Chlorpromazine Second line agents: DHE.5-1 mg IM / IV or sumatriptan 6 mg SQ Second line agents: DHE.5-1 mg IM / IV or sumatriptan 6 mg SQ Third line agent: Ketorolac Third line agent: Ketorolac Fourth line agent: Butorphanol 1 mg intranasally Fourth line agent: Butorphanol 1 mg intranasally Fifth line agent: Opioids Fifth line agent: Opioids Canadian Headache Society. Guidelines for the diagnosis and management of Migraine in clinical practice. Can Med Assoc J 1997; 156:1273-1287 US Headache Consortium. www.aan.com/public/practice guidelineswww.aan.com/public/practice

12. ED VISIT Diagnosis:Migraine Treatment:Prochlorperazine Disposition:Headache resolved

13. Does response to therapy predict the etiology of an acute severe headache? All headache pain is mediated by serotonin receptors All headache pain is mediated by serotonin receptors Case series / case reports (Class III evidence) Case series / case reports (Class III evidence) Seymour. Am J Emerg Med 1995. 3 patients treated with ketorolac or prochlorperazine with resolution of headache / Discharged / All with catestrophic outcomes Seymour. Am J Emerg Med 1995. 3 patients treated with ketorolac or prochlorperazine with resolution of headache / Discharged / All with catestrophic outcomes Gross. Headache 1995. 3 cases of meningitis with resolution of pain with DHE and metoclopramide Gross. Headache 1995. 3 cases of meningitis with resolution of pain with DHE and metoclopramide Pain response can not be used as an indicator or the underlying etiology of an acute headache. Pain response can not be used as an indicator or the underlying etiology of an acute headache.

14. Should this patient have received a head CT? Yes Yes No No

15. Should this patient have received a head CT? Infection Infection CNS mass lesion CNS mass lesion Tumor, IIH, Hydrocephalus Tumor, IIH, Hydrocephalus Collagen vascular disease Collagen vascular disease Temporal arteritis, vasculitis Temporal arteritis, vasculitis Ophthamologic etiologies Ophthamologic etiologies Glaucoma, optic neuritis Glaucoma, optic neuritis Metabolic abnormalities Metabolic abnormalities Toxins Toxins Pregnancy related Pregnancy related Eclampsia, dural sinus thrombosis Eclampsia, dural sinus thrombosis CNS vascular event CNS vascular event Subdural, epidural, SAH Subdural, epidural, SAH Primary headache disorder Primary headache disorder

16. Which patients with acute headache require neuroimaging in the ED? Neuroimaging is obtained to assess for treatable lesions: SAH, CVT, tumors, hydrocephalus Neuroimaging is obtained to assess for treatable lesions: SAH, CVT, tumors, hydrocephalus (Less tangible: Patient reassurance) (Less tangible: Patient reassurance) (Less tangible: Doctor reassurance) (Less tangible: Doctor reassurance) Abnormal neuro exam increases the likelihood of a positive CT 3 times (95% CI 2.3-4) Abnormal neuro exam increases the likelihood of a positive CT 3 times (95% CI 2.3-4) Normal neuro exam is not predictive Normal neuro exam is not predictive Location, vomiting, headache waking patient up, worsening with valsalva are not predictive Location, vomiting, headache waking patient up, worsening with valsalva are not predictive

17. Which patients with acute headache require neuroimaging in the ED? Severe sudden onset headache: Severe sudden onset headache: Lledo Headache 1994, prospective study: 9 of 27 had SAH (only 4 had a positive CT) Lledo Headache 1994, prospective study: 9 of 27 had SAH (only 4 had a positive CT) Mills Ann Emerg Med 1986, prospective study 42 patients: 29% with worst headache had a postive CT Mills Ann Emerg Med 1986, prospective study 42 patients: 29% with worst headache had a postive CT Headache in the HIV patient: Headache in the HIV patient: Lipton Headache 1991, prospective 49 patients: 35% had mass lesion Lipton Headache 1991, prospective 49 patients: 35% had mass lesion Rothman Acad Emerg Med 1999, prospective 110 pts: 24% had a focal lesion Rothman Acad Emerg Med 1999, prospective 110 pts: 24% had a focal lesion

18. Which patients with acute headache require neuroimaging in the ED? Patients presenting with an acute HA and an abnormal neurologic exam should have an emergent head CT Patients presenting with an acute HA and an abnormal neurologic exam should have an emergent head CT Patients presenting with a sudden severe HA should have an emergent head CT Patients presenting with a sudden severe HA should have an emergent head CT HIV patients with a new type of headache should have an urgent head CT HIV patients with a new type of headache should have an urgent head CT Patients over the age of 50 with a new type of headache should have an urgent neuroimaging study Patients over the age of 50 with a new type of headache should have an urgent neuroimaging study

19. Should this patient have had a head CT? History: History: HA was sudden and severe in onset HA was sudden and severe in onset HA was different from past headaches HA was different from past headaches Physical: Physical: No neurologic exam documented: No neurologic exam documented: In the HA patient, the neuro exam focuses on pupil, fundoscopy, and cranial nerves III, IV, VI In the HA patient, the neuro exam focuses on pupil, fundoscopy, and cranial nerves III, IV, VI

20. ED Visit #2 Patient returned 24 hours later with worsening headache Patient returned 24 hours later with worsening headache Positive findings on the physical examination: Positive findings on the physical examination: Papilledema Papilledema Left 6 th cranial nerve palsy on far lateral gaze Left 6 th cranial nerve palsy on far lateral gaze A noncontrast head CT was normal A noncontrast head CT was normal

21. What are the indications for LP in acute HA? Suspected SAH in a patient with a normal head CT Suspected SAH in a patient with a normal head CT CT is 90 – 98% sensitive for acute SAH CT is 90 – 98% sensitive for acute SAH Sensitivity decreases over time Sensitivity decreases over time Suspected meningitis Suspected meningitis LP without CT in patients with normal neuro exam including normal mental status and normal fundoscopic exam LP without CT in patients with normal neuro exam including normal mental status and normal fundoscopic exam Suspected idiopathic intracranial hypertension Suspected idiopathic intracranial hypertension Headache with papilledema Headache with papilledema Normal CT Normal CT

22. ED Visit #2 Lumbar puncture: Lumbar puncture: Opening pressure 280 mm Hg; Opening pressure 280 mm Hg; CSF: No cells, Normal protein and glucose CSF: No cells, Normal protein and glucose Diagnosis of idiopathic intracranial hypertension was made Diagnosis of idiopathic intracranial hypertension was made

23. Idiopathic Intracerebral Hypertension: Diagnositc Criteria Symptoms reflect only ICP or papilledema Symptoms reflect only ICP or papilledema HA (70-98%) HA (70-98%) Visual symptoms (57-72%) Visual symptoms (57-72%) Pulsatile noises /tinnitus (to 60%) Pulsatile noises /tinnitus (to 60%) Signs only of elevated ICP Signs only of elevated ICP Papilledema (virtually 100%) Papilledema (virtually 100%) Blind spot, field defect or 6 th palsy Blind spot, field defect or 6 th palsy Friedman. Neurology 2002:59:1492-1495 Ball. Lancet Neurology; 5: 433-42

24. Idiopathic Intracerebral Hypertension: Diagnositc Criteria ICP elevated above 20cm H 2 O (25cm in obese) ICP elevated above 20cm H 2 O (25cm in obese) CSF is normal CSF is normal No structural lesion on enhanced CT or MRI No structural lesion on enhanced CT or MRI No other cause ICP No other cause ICP Metabolic Metabolic Toxic Toxic Venous obstruction Venous obstruction Friedman. Neurology 2002:59:1492-1495 Ball. Lancet Neurology; 5: 433-42

25. Idiopathic Intracerebral Hypertension: Epidemiology.03- 2 cases per 100,000.03- 2 cases per 100,000 Most common 20-40 y.o. Most common 20-40 y.o. 4 -15 to 1 female to male 4 -15 to 1 female to male 20 cases per 100,000 in obese women of childbearing age. 20 cases per 100,000 in obese women of childbearing age.

26. Idiopathic Intracerebral Hypertension: Etiology Reduced absorption of CSF ? Reduced absorption of CSF ? Increased CSF production? Increased CSF production? Increased cerebral venous pressure? Increased cerebral venous pressure? Increased brain water content? Increased brain water content?

27. Idiopathic Intracranial Hypertension: Clinical Findings Papilledema100% Papilledema100% Headache94% Headache94% Visual disturbance 80% Visual disturbance 80% Transient visual obscuration68% Transient visual obscuration68% VI CN palsy (False localizing)38% VI CN palsy (False localizing)38% Decreased visual acuity30% Decreased visual acuity30% Pulsatile noises30% Pulsatile noises30% Blindness 10% Blindness 10% Giuseffi. Neurology 1991; 41:239-244

28. Idiopathic Intracranial Hypertension: Treatment Weight loss Weight loss Serial lumbar punctures Serial lumbar punctures Acetazolamide, 1-4 gms / day Acetazolamide, 1-4 gms / day Corticosteriods, 40-60 mg / day Corticosteriods, 40-60 mg / day Surgery Surgery Optic nerve sheath decompression Optic nerve sheath decompression Lumboperitoneal shunt Lumboperitoneal shunt Bariatric Bariatric Radhakrishnan. Mayo Clin Pro 1994; 69:169-180

29. Idiopathic Intracerebral Hypertension: Treatment 51 studies identified concerning IHH 51 studies identified concerning IHH 7 concerned treatment 7 concerned treatment 2 retrospective, none with control groups 2 retrospective, none with control groups No Studies met inclusion criteria No Studies met inclusion criteria “ There is insufficient evidence to recommend or reject any of the treatments currently available for IIH” “ There is insufficient evidence to recommend or reject any of the treatments currently available for IIH” Cochrane Database of Systematic Reviews 2005

30. CONCLUSIONS Errors in management Errors in management No fundoscopic exam: Opthalmoscope was not working No fundoscopic exam: Opthalmoscope was not working No CT: symptoms resolved and CT backed- up No CT: symptoms resolved and CT backed- up

31. CONCLUSIONS Lessons learned Lessons learned Patients with headache require a comprehensive neurologic exam Patients with headache require a comprehensive neurologic exam First line therapy for headache are drugs that work at serotonin receptors First line therapy for headache are drugs that work at serotonin receptors Response to therapy does not predict etiology Response to therapy does not predict etiology Patients with sudden severe headache require a CT; if negative followed by an lumbar puncture Patients with sudden severe headache require a CT; if negative followed by an lumbar puncture

32. Thank you. www.ferne.org ferne@ferne.org www.ferne.org ferne_pv_2007_hill_headache_080607_finalcd 5/17/2015 6:09 AM