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Evaluation of the Sudden and Severe Headache: Diagnosis and Management Michael Gerardi, MD, FAAP, FACEP Vice-Chairman, Department of Emergency Medicine.

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Presentation on theme: "Evaluation of the Sudden and Severe Headache: Diagnosis and Management Michael Gerardi, MD, FAAP, FACEP Vice-Chairman, Department of Emergency Medicine."— Presentation transcript:

1 Evaluation of the Sudden and Severe Headache: Diagnosis and Management Michael Gerardi, MD, FAAP, FACEP Vice-Chairman, Department of Emergency Medicine Morristown Memorial Hospital Director, Pediatric Emergency Medicine Childrens Medical Center Morristown, New Jersey

2 Michael Gerardi, MD The Case One hour prior to ED presentation, a 42 year old man was jogging and hit by the worst headache of his life. It was associated with some nausea and the feeling as if he was going to pass out. He rested for 30 minutes but the headache persisted as a diffuse, throbbing pain radiating to the base of his skull. One hour prior to ED presentation, a 42 year old man was jogging and hit by the worst headache of his life. It was associated with some nausea and the feeling as if he was going to pass out. He rested for 30 minutes but the headache persisted as a diffuse, throbbing pain radiating to the base of his skull.

3 Michael Gerardi, MD EMS was called. The patient felt as if he could not concentrate, there was no confusion, nor was there any other focal neurologic complaint. There was no past medical history, no medications, no family history, and no significant use of alcohol, tobacco or other drugs. The Case (Continued)

4 Michael Gerardi, MD If a patient presented with the worst headache of his life, what is the work- up that should be initiated? a. Non-contrast CT b. LP after neg. CT c. LP without CT d. CT, LP, and angiography

5 Michael Gerardi, MD l What is the differential of a thunderclap headache? l What is the sensitivity of neuroimaging in subarachnoid hemorrhage (SAH)? l What constitutes a positive lumbar puncture in SAH and when should it be performed? l Do patients with suspected SAH who have a negative CT and lumbar puncture require additional imaging to rule-out expanded but unruptured aneurysm? Objectives

6 Michael Gerardi, MD 1 of 10 top presenting complaints 1 to 2% of visits to ED 1 to 2% of visits to ED 18 million outpatient visits 18 million outpatient visits 638 million days of work lost per year 638 million days of work lost per year 78% of women and 64% of men had experienced at least one in the prior year 78% of women and 64% of men had experienced at least one in the prior year 36% of women and 19% men suffer from recurrent headaches 36% of women and 19% men suffer from recurrent headaches

7 Michael Gerardi, MD Most have primary headache disorders Most have primary headache disorders migraine migraine tension tension Only a few have treatable secondary causes that threaten life, limb, brain such as subarachnoid hemorrhage Only a few have treatable secondary causes that threaten life, limb, brain such as subarachnoid hemorrhage 1 - 4 % of headache visits 1 - 4 % of headache visits

8 Michael Gerardi, MD Worst Headache l Normal exam: 12- 33% SAH l Abnormal exam:25% SAH l Initial hemorrhage may be fatal l Early definitive surgery improves outcomes l Patients with greatest likelihood of benefiting from surgery are most likely to receive incorrect diagnosis

9 Michael Gerardi, MD Physicians Consistently Misdiagnose SAH 1. Failure to appreciate spectrum of clinical presentation 2. Failure to understand limitations of CT 3. Failure to perform and correctly interpret the results of LP

10 Michael Gerardi, MD ED Goals in Headache Patients 1. Differentiate life-threatening from benign 2. Initiate prompt treatment 3. Provide prompt pain relief 4. Prevent drug seeking and refer 5. Minimize resource utilization in ED 6. Optimize patient use of ED 7. Increase pre-ED treatment and reduce ED use

11 Michael Gerardi, MD Differential Diagnosis of Headache l Onset l Location l Associated symptoms l Pain characteristics l Duration l Prior history l Diagnostic tests l Physical exam

12 Michael Gerardi, MD Medical Conditions That Present With Headache l Pheochromocytoma l Hyperthyroidism l SLE l Giant Cell Arteritis l Fibromyalgia

13 Michael Gerardi, MD Types of Headaches in the ED Final DiagnosisPercentage Infection - not intracranial39.3 Tension HA19.3 Miscellaneous14.9 Post-traumatic9.3 Hypertension related4.8 Vascular (Migraine)4.5 No diagnosis6.0 SAH0.9 Meningitis0.6

14 Michael Gerardi, MD Ped HA Compared to Literature: Serious Conditions Author # Age Tumor Bleed Meningitis Burton 288 2-18 00 0.3 Fodden 106 0-90 4.78.5 0 Leicht 485 15-89 2.71.0 0.8 Dopeshi 872 2-92 0.10.9 0.6 Dickman 124 16-65 0 0 0

15 Michael Gerardi, MD Causes of Headache That Require Specific Therapy l Subarachnoid hemorrhage l Meningitis l Encephalitis l Cervicocranial-artery dissection l Temporal arteritis l Acute angle-closure glaucoma l Hypertensive emergency

16 Michael Gerardi, MD Causes of Headache That Require Specific Therapy l Carbon Monoxide poisoning l Pseudotumor cerebri l Cerebral venous and dural sinus thrombosis l Acute stroke (hemorrhagic or ischemic) l Mass Lesion v tumor v abscess v intracranial hematoma v parameningeal infection

17 Michael Gerardi, MD Headache Danger Signals l Onset v after 40 years v new or different headache v subacute HA that worsens v exertion, sex, coughing, straining l Worst ever experienced

18 Michael Gerardi, MD Headache Danger Signals: Associated With Neurologic Change l Memory impairment l Ataxia l Drowsiness l Sensory loss l Signs of meningeal irritation

19 Michael Gerardi, MD Headache Danger Signals: Associated With Neurologic Change l Progressive visual or neurologic change l Confusion l Weakness l Loss of coordination l Asymmetry of pupils, DTRs

20 Michael Gerardi, MD Headache Danger Signals: Abnormal Medical Evaluation l Fever l Chronic malaise l Arthralgia l HTN l Myalgia l Wt loss l Tender, poorly pulsatile temporal arteries

21 Michael Gerardi, MD Subarachnoid Hemorrhage l 10% of all acute CVAs l 30,000 persons/year v 10 -16/100,000 l 1% of all ED patients with acute cephalgia

22 Michael Gerardi, MD Subarachnoid Hemorrhage l Incidence of 16 /100,000 v about 33,600 cases per year v 54% secondary to ruptured aneurysm l Without treatment, 40% of aneurysm pts. have recurrent bleeding l Aneurysm pt who survives initial rupture and is treated conservatively: v 50% survival at one year

23 Michael Gerardi, MD Subarachnoid Hemorrhage l Onset: Acute l Location: Global l Ass Sx:N,V, meningismus, focal l Pain: Worst ever l Duration: Brief l Prior Hx: No l Dx tests:CT 80-90% l Phys ex: Focal signs, LOC, meningismus

24 Michael Gerardi, MD Subarachnoid Hemorrhage l Warning leaks in 50% l CT misses up to 10% small leaks l Suspect if: v> 35 years v no previous HA v no fading of HA v came on with exertion v altered LOC or neuro deficits v stiff neck

25 Michael Gerardi, MD Subarachnoid Hemorrhage: Neurologic Findings l Sudden HA without localizing findings l Altered mentation v Confusion, lethargy l Bilateral extensor plantar reflex l Unusual to find focal deficits

26 Michael Gerardi, MD Causes of Non-Traumatic Subarachnoid Hemorrhage l Berry aneurysms l AVM l Cerebral angiomas l Mycotic aneurysm l Extension from parenchymatous hemorrhage l Anticoagulation therapy

27 Michael Gerardi, MD Causes of Non-Traumatic Subarachnoid Hemorrhage l Systemic bleeding diathesis l Hemorrhagic encephalitis l Hemorrhagic cerebral vasculitis l Hemorrhage into CNS tumors or metastases l Unknown

28 Michael Gerardi, MD Intracranial Aneurysms l Women: men = 3 : 2 l 4 million Americans v 20% multiple aneurysms l Increase in mid-20s l Peak incidence of 12% by age 60 l Risk of spontaneous rupture 1 to 3%/yr v Peak 40 to 60 years

29 Michael Gerardi, MD Arteriovenous Malformations l 10-15% of SAH l Spontaneous hemorrhage v Any age but usually < 30 l Incidence 3% per year l Incidence of major neurologic deficit or mortality: 50%

30 Michael Gerardi, MD Conditions Associated with Cerebral Aneurysm Development l HTN l Polycystic kidney disease l Connective tissue disorders l Coarctation of aorta l Pregnancy induced HTN l Family history of CVAs l Bacterial endocarditis

31 Michael Gerardi, MD Warning Headache l 20 - 50% patients with SAH have HA days or weeks before index episode v unusually severe v distinct l Thunderclap headache v Day and Raskin 1996 v intense, acute, peak intensity at onset v develop in seconds v maximal intensity in minutes v lasts hours to days

32 Michael Gerardi, MD Thunderclap Headache l 25% associated with SAH l Warning headache v followed by SAH in 5% to 60% l Expansion or dissection of unruptured aneurysm l Cerebral venous thrombosis l Exertional / coital headache

33 Michael Gerardi, MD Subarachnoid Hemorrhage: Morbidity and Mortality 28,000 ruptured aneurysms 10,00018,000 dead/disabledavailable for Rx 3,000 7,000 8,000 10,000 died rapidly misdiagnoseddead orfunctional no warning or misseddisabled survivors

34 Michael Gerardi, MD Misdiagnosis of Symptomatic Cerebral Aneurysm: Mayer 1996 l 217 patients with symptomatic SAH v 54 / 217 misdiagnosed v 46 / 217 minimal findings v viral meningitis 15% v migraine 13% v uncertain etiology13% l Failure to consider SAH

35 Michael Gerardi, MD Missed Cerebral Aneurysms Mayer 1996 l 9 / 43 (21%) CTs initially read as neg. v 6 of these 9 : (+) SAH l 48% re-bleed or deteriorated (vs. 2%) l Good or excellent outcomes v 91% initially correct v 53% if misdiagnosed

36 Michael Gerardi, MD SAH…But not Classic l Roughly half have minor bleeding with atypical features l Nonstrenuous activities (34%) l Sleep (12%) l HA in any location (localized, generalized, mild) l May be relieved by non-narcotic analgesics l Diagnosed as migraine, tension-type, sinusitis

37 Michael Gerardi, MD SAH…But not Classic l Prominent neck pain v Cervical sprain, arthritis l Confusion, agitation, restless v psychiatric diagnoses l Syncope / trauma v Traumatic SAH l Syncope / abnormal ECG v MI and then trauma v 91% SAH have cardiac dysrhythmias and ECGs mimicking ischemia

38 Michael Gerardi, MD SAH: Most patients have... l Abrupt onset of severe, unique headache, or neck pain l Abnormal findings on neurologic examination l Subtle meningismus or ocular findings

39 Michael Gerardi, MD International Headache Society l A first episode of severe headache cannot be classified as migraine: v more than 4 episodes l nor as tension-type headache: v more than 9 episodes l First or worst headache requires evaluation vas do qualitatively different headaches

40 Michael Gerardi, MD Can a CT Scan Safely Rule Out SAH? l First diagnostic study l Thin cuts ( 3 mm) through base of brain l Blood on CT function of Hgb v Hgb < 10: blood isodense l Sensitivity decreases over time from onset of symptoms

41 Michael Gerardi, MD Acute HA of Recent Onset Leido A. Headache 1994 l 27 patients; 24 - 77 yo l 1 hr to 13 days after HA onset l no previous similar HA l no focal neurologic signs l all had CT; LP if CT neg

42 Michael Gerardi, MD Acute HA of Recent Onset Leido A. Headache 1994 l 9 of 27 (33%) : SAH v 4 (+) CT v 5 normal CT, (+) LP l 2 of 19 LPs: meningitis l CT scanning should be done with first severe acute headache

43 Michael Gerardi, MD CT & Subarachnoid Hemorrhage: Sames et al: 1996 Sensitivity of NGCT: Group 1 (symptoms < 24 hrs)93.1% Group 2 (symptoms > 24 hrs)83.8% A normal NGCT does not reliably exclude the need for LP

44 Michael Gerardi, MD SAH: CT Sensitivity Sames: Acad Emerg Med Jan 1996 l 181 patients; aged 13-86 with SAH v Sensitivity 91.2% v pain < 24 hrs 93.1% v pain > 24 hrs 83.8% l LP 100% sensitive if neg CT l A normal NGCT does not reliably exclude the need for LP

45 Michael Gerardi, MD SAH Diagnosis: LP Needed Sidman: Acad Emerg Med Sep 1996 l 140 patients; aged 10-88 l Sensitivity of CT v < 12 hrs80/80100% v > 12 hrs49/6081.7% l Overall, 11/140 had (-) CT and (+) LP v overall sensitivity92.1%

46 Michael Gerardi, MD Morgenstern LB, et al: Worst headache and SAH: Prospective, modern CT and spinal fluid analysis. Ann Emerg Med Sept 1998. l 38,730 patients over 16 months, prospectively screened for worst HA l Blinded neuroradiologists vNeg CTLP vcell count x 2 vvisual and spectrophotometric detection of xanthochromia vCSF D-dimer assay

47 Michael Gerardi, MD Morgenstern, et al: Ann Emerg Med 1998 l 455 headaches & 107 worst headache l CT: 18 of 107 (17%): (+) SAH l (-) CT/ (+) SAH: Only 2 (2.5%) v (95% CI, 0.3%to 8.8%) l Modern CT is sufficient to exclude 98% of SAH in patients

48 Michael Gerardi, MD Morgenstern, et al: Ann Emerg Med 1998 (107 Worst HAs) Variables CT-/LP- CT+ CT-/LP+ Photophobia4528 50 Stiff neck2637 100 Nausea6536 100 Lethargy174050 Time < 24 h587550 Migraine2011 0 Headache4827 0

49 Michael Gerardi, MD CT is Normal: Do LP? Yes!

50 Michael Gerardi, MD What about LP First? l Duffy et al; 1982: 55 patients who underwent LP as initial w/u v Condition deteriorated immediately in 7 patients l Hillman et al; 1986: 4 alert patients with SAH who deteriorated after lumbar puncture l Both studies: v clots on CT or a dilated pupil

51 Michael Gerardi, MD LP First? Schull: Acad Emerg Med 1999 l CT sensitivity: 86% l LP after 12 hours: 100% l Mathematical modeling for 100 patients v 9 more LPs v81 fewer CT scans

52 Michael Gerardi, MD Traumatic Taps l 20% of LPs l 0.5% and 6% has incidental intracranial aneurysm l Impression or 3-tube method not reliable in detecting traumatic tap l Erythrocytes disseminate rapidly l Released Hgb oxyhemoglobin xanthochromiabilirubin

53 Michael Gerardi, MDXanthochromia l Bilirubin, enzyme-dependent process, is diagnostically more reliable but: v takes up to 12 hours l Timing is important l CSF should be centrifuged and examined promptly so RBCs dont undergo lysis in vitro, causing xanthochromia from oxyhemoglobin

54 Michael Gerardi, MD Xanthochromia vs. Erythrocytes l Xanthochromia v primary criterion for SAH if neg CT v advocates: spectrophotometry l Erythrocytes v considered more accurate by some v used visual inspection which can miss discoloration in up to 50%

55 Michael Gerardi, MD Timing the Tap l With spectrophotometry, and waiting 12 hours after onset of headache: very accurate v traumatic tap done earlier does not lead to xanthochromia and confusion l Waiting: vprolongation of ED stay v risk ultra-early rebleeding

56 Michael Gerardi, MD Normal CT & Persistently Bloody CSF ??? l Not prudent to delay LP l Without xanthochromia and clinical suspicion is high? v Vascular imaging l Xanthochromia present and clinical suspicion is high? v Vascular imaging

57 Michael Gerardi, MD Thunderclap Headache: NL CT & NL LP - Vascular Imaging? l Unruptured cerebral aneurysm v Day and Raskin: 1 patient - clipped v Raps et al: 7 patients v Witham: 1 patient - very thin aneurysm dome; clipped

58 Michael Gerardi, MD Thunderclap Headache: NL CT & NL LP Vascular Imaging? l Wijdicks et al; Lancet, 1988 v Retrospective evaluation 71 patients v no SAH in 3.3 years f/u v Half dxd with migraine or tension HA l Markus 1991; Linn 1994; Harling 1989 v 117 patients v no SAH, no sudden deaths

59 Michael Gerardi, MD SAH High Risk Factors Clinical History l Onset of HA: abrupt, maximal at onset, thunderclap headache l Severity of headache: usually the worst of life or very severe l Quality: First of this intensity; unique or different l Associated signs / sxs: LOC, diplopia, seizure, focal neurologic signs

60 Michael Gerardi, MD SAH High Risk Factors: Epidemiologic l Cigarette smoking l Hypertension l Alcohol consumption (binge?) l Personal or family history l Polycystic kidney disease l Heritable connective tissue diseases l Sickle Cell Anemia l Pregnancy and childbirth l Valsalva maneuver l Coitus l Cocaine abuse l Amphetamines

61 Michael Gerardi, MD Predisposing Factors for Aneurysmal Rupture l Pregnancy and childbirth l Poorly controlled HTN l Valsalva maneuver l Coitus l Heavy ETOH consumption l Cocaine abuse l Amphetamines

62 Michael Gerardi, MD HA: Cough, Exertional, & Sex Pascual: Neurology 1996 l 72 patients l Intracranial lesions on neuroimaging v cough-induced17 / 30 42% v exertional12 / 28 43% v sex1 / 14 7%

63 Michael Gerardi, MD HA: Cough, Exertional, & Sex Pascual: Neurology 1996 l Cough-induced: underlying lesion was always Chiari type I malformation l Indomethacin- effective in benign but not with underlying lesions l SAH v 10 / 12 : exercise - induced v 1/ 14 : sexual activity

64 Michael Gerardi, MD HA: Cough, Exertional, & Sex Pascual: Neurology 1996 l ALL patients with SAH: v single HA v prolonged v severe generally accompanied by v nausea v vomiting v photophobia

65 Michael Gerardi, MD Subarachnoid Hemorrhage: Morbidity and Mortality 28,000 ruptured aneurysms 10,00018,000 dead/disabledavailable for Rx 3,000 7,000 8,000 10,000 died rapidly misdiagnoseddead orfunctional no warning or misseddisabled survivors

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