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Evaluation of the Sudden and Severe Headache: Diagnosis and Management Michael Gerardi, MD, FAAP, FA

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Title: Evaluation of the Sudden and Severe Headache: Diagnosis and Management Michael Gerardi, MD, FAAP, FA


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
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.

3
The Case (Continued)
  • 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.

4
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
Objectives
  • What is the differential of a thunderclap
    headache?
  • What is the sensitivity of neuroimaging in
    subarachnoid hemorrhage (SAH)?
  • What constitutes a positive lumbar puncture in
    SAH and when should it be performed?
  • Do patients with suspected SAH who have a
    negative CT and lumbar puncture require
    additional imaging to rule-out expanded but
    unruptured aneurysm?

6
Headache
  • 1 of 10 top presenting complaints
  • 1 to 2 of visits to ED
  • 18 million outpatient visits
  • 638 million days of work lost per 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

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

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

9
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
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
Differential Diagnosis of Headache
  • Onset
  • Location
  • Associated symptoms
  • Pain characteristics
  • Duration
  • Prior history
  • Diagnostic tests
  • Physical exam

12
Medical Conditions That Present With Headache
  • Pheochromocytoma
  • Hyperthyroidism
  • SLE
  • Giant Cell Arteritis
  • Fibromyalgia

13
Types of Headaches in the ED
  • Final Diagnosis Percentage
  • Infection - not intracranial 39.3
  • Tension HA 19.3
  • Miscellaneous 14.9
  • Post-traumatic 9.3
  • Hypertension related 4.8
  • Vascular (Migraine) 4.5
  • No diagnosis 6.0
  • SAH 0.9
  • Meningitis 0.6

14
Ped HA Compared to Literature Serious Conditions
  • Author Age Tumor Bleed Meningitis
  • Burton 288 2-18 0 0 0.3
  • Fodden 106 0-90 4.7 8.5 0
  • Leicht 485 15-89 2.7 1.0 0.8
  • Dopeshi 872 2-92 0.1 0.9 0.6
  • Dickman 124 16-65 0 0 0

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

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

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

18
Headache Danger Signals Associated With
Neurologic Change
  • Memory impairment
  • Ataxia
  • Drowsiness
  • Sensory loss
  • Signs of meningeal irritation

19
Headache Danger Signals Associated With
Neurologic Change
  • Progressive visual or neurologic change
  • Confusion
  • Weakness
  • Loss of coordination
  • Asymmetry of pupils, DTRs

20
Headache Danger Signals Abnormal Medical
Evaluation
  • Fever
  • Chronic malaise
  • Arthralgia
  • HTN
  • Myalgia
  • Wt loss
  • Tender, poorly pulsatile temporal arteries

21
Subarachnoid Hemorrhage
  • 10 of all acute CVAs
  • 30,000 persons/year
  • 10 -16/100,000
  • 1 of all ED patients with acute cephalgia

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

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

24
Subarachnoid Hemorrhage
  • Warning leaks in 50
  • CT misses up to 10 small leaks
  • Suspect if
  • 35 years
  • no previous HA
  • no fading of HA
  • came on with exertion
  • altered LOC or neuro deficits
  • stiff neck

25
Subarachnoid Hemorrhage Neurologic Findings
  • Sudden HA without localizing findings
  • Altered mentation
  • Confusion, lethargy
  • Bilateral extensor plantar reflex
  • Unusual to find focal deficits

26
Causes of Non-Traumatic Subarachnoid Hemorrhage
  • Berry aneurysms
  • AVM
  • Cerebral angiomas
  • Mycotic aneurysm
  • Extension from parenchymatous hemorrhage
  • Anticoagulation therapy

27
Causes of Non-Traumatic Subarachnoid Hemorrhage
  • Systemic bleeding diathesis
  • Hemorrhagic encephalitis
  • Hemorrhagic cerebral vasculitis
  • Hemorrhage into CNS tumors or metastases
  • Unknown

28
Intracranial Aneurysms
  • Women men 3 2
  • 4 million Americans
  • 20 multiple aneurysms
  • Increase in mid-20s
  • Peak incidence of 12 by age 60
  • Risk of spontaneous rupture 1 to 3/yr
  • Peak 40 to 60 years

29
Arteriovenous Malformations
  • 10-15 of SAH
  • Spontaneous hemorrhage
  • Any age but usually
  • Incidence 3 per year
  • Incidence of major neurologic deficit or
    mortality 50

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

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

32
Thunderclap Headache
  • 25 associated with SAH
  • Warning headache
  • followed by SAH in 5 to 60
  • Expansion or dissection of unruptured aneurysm
  • Cerebral venous thrombosis
  • Exertional / coital headache

33
Subarachnoid Hemorrhage Morbidity and Mortality
  • 28,000
  • ruptured aneurysms
  • 10,000 18,000
  • dead/disabled available for Rx
  • 3,000 7,000 8,000 10,000
  • died rapidly misdiagnosed dead or functional
  • no warning or missed disabled survivors

34
Misdiagnosis of Symptomatic Cerebral Aneurysm
Mayer 1996
  • 217 patients with symptomatic SAH
  • 54 / 217 misdiagnosed
  • 46 / 217 minimal findings
  • viral meningitis 15
  • migraine 13
  • uncertain etiology 13
  • Failure to consider SAH

35
Missed Cerebral Aneurysms Mayer 1996
  • 9 / 43 (21) CTs initially read as neg.
  • 6 of these 9 () SAH
  • 48 re-bleed or deteriorated (vs. 2)
  • Good or excellent outcomes
  • 91 initially correct
  • 53 if misdiagnosed

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

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

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

39
International Headache Society
  • A first episode of severe headache cannot be
    classified as migraine
  • more than 4 episodes
  • nor as tension-type headache
  • more than 9 episodes
  • First or worst headache requires evaluation
  • as do qualitatively different headaches

40
Can a CT Scan Safely Rule Out SAH?
  • First diagnostic study
  • Thin cuts ( 3 mm) through base of brain
  • Blood on CT function of Hgb
  • Hgb
  • Sensitivity decreases over time from onset of
    symptoms

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

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

43
CT Subarachnoid Hemorrhage Sames et al 1996
  • Sensitivity of NGCT
  • Group 1 (symptoms
  • Group 2 (symptoms 24 hrs) 83.8
  • A normal NGCT does not reliably exclude the need
    for LP

44
SAH CT Sensitivity Sames Acad Emerg Med Jan 1996
  • 181 patients aged 13-86 with SAH
  • Sensitivity 91.2
  • pain
  • pain 24 hrs 83.8
  • LP 100 sensitive if neg CT
  • A normal NGCT does not reliably exclude the need
    for LP

45
SAH Diagnosis LP Needed Sidman Acad Emerg Med
Sep 1996
  • 140 patients aged 10-88
  • Sensitivity of CT
  • 12 hrs 49/60 81.7
  • Overall, 11/140 had (-) CT and () LP
  • overall sensitivity 92.1

46
Morgenstern LB, et al Worst headache and SAH
Prospective, modern CT and spinal fluid analysis.
Ann Emerg Med Sept 1998.
  • 38,730 patients over 16 months, prospectively
    screened for worst HA
  • Blinded neuroradiologists
  • Neg CT LP
  • cell count x 2
  • visual and spectrophotometric detection of
    xanthochromia
  • CSF D-dimer assay

47
Morgenstern, et al Ann Emerg Med 1998
  • 455 headaches 107 worst headache
  • CT 18 of 107 (17) () SAH
  • (-) CT/ () SAH Only 2 (2.5)
  • (95 CI, 0.3to 8.8)
  • Modern CT is sufficient to exclude 98 of SAH in
    patients

48
Morgenstern, et al Ann Emerg Med 1998 (107
Worst HAs)
  • Variables CT-/LP- CT
    CT-/LP
  • Photophobia 45 28 50
  • Stiff neck 26 37 100
  • Nausea 65 36 100
  • Lethargy 17 40 50
  • Time
  • Migraine 20 11 0
  • Headache 48 27 0

49
CT is Normal Do LP?
  • Yes!

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

51
LP First? Schull Acad Emerg Med 1999
  • CT sensitivity 86
  • LP after 12 hours 100
  • Mathematical modeling for 100 patients
  • 9 more LPs
  • 81 fewer CT scans

52
Traumatic Taps
  • 20 of LPs
  • 0.5 and 6 has incidental intracranial aneurysm
  • Impression or 3-tube method not reliable in
    detecting traumatic tap
  • Erythrocytes disseminate rapidly
  • Released Hgb oxyhemoglobin
  • xanthochromia bilirubin

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

54
Xanthochromia vs. Erythrocytes
  • Xanthochromia
  • primary criterion for SAH if neg CT
  • advocates spectrophotometry
  • Erythrocytes
  • considered more accurate by some
  • used visual inspection which can miss
    discoloration in up to 50

55
Timing the Tap
  • With spectrophotometry, and waiting 12 hours
    after onset of headache very accurate
  • traumatic tap done earlier does not lead to
    xanthochromia and confusion
  • Waiting
  • prolongation of ED stay
  • risk ultra-early rebleeding

56
Normal CT Persistently Bloody CSF ???
  • Not prudent to delay LP
  • Without xanthochromia and clinical suspicion is
    high?
  • Vascular imaging
  • Xanthochromia present and clinical suspicion is
    high?
  • Vascular imaging

57
Thunderclap Headache NL CT NL LP - Vascular
Imaging?
  • Unruptured cerebral aneurysm
  • Day and Raskin 1 patient - clipped
  • Raps et al 7 patients
  • Witham 1 patient - very thin aneurysm dome
    clipped

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

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

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

61
Predisposing Factors for Aneurysmal Rupture
  • Pregnancy and childbirth
  • Poorly controlled HTN
  • Valsalva maneuver
  • Coitus
  • Heavy ETOH consumption
  • Cocaine abuse
  • Amphetamines

62
HA Cough, Exertional, Sex Pascual Neurology
1996
  • 72 patients
  • Intracranial lesions on neuroimaging
  • cough-induced 17 / 30 42
  • exertional 12 / 28 43
  • sex 1 / 14 7

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

64
HA Cough, Exertional, Sex Pascual Neurology
1996
  • ALL patients with SAH
  • single HA
  • prolonged
  • severe generally accompanied by
  • nausea
  • vomiting
  • photophobia

65
Subarachnoid Hemorrhage Morbidity and Mortality
  • 28,000
  • ruptured aneurysms
  • 10,000 18,000
  • dead/disabled available for Rx
  • 3,000 7,000 8,000 10,000
  • died rapidly misdiagnosed dead or functional
  • no warning or missed disabled survivors

66
Acute Headache
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