1. Acute Neurological Emergencies: Headache1 1 1

2. Brad Bunney, MD Associate Professor Dept of Emergency Medicine
University of Illinois College of Medicine
Chicago, IL

3. Michael Gerardi, MD, FAAP, FACEP Vice-Chairman, Department of Emergency Medicine Morristown Memorial Hospital Morristown, New Jersey Nina T. Gentile, MD Associate Professor Division of Emergency Medicine Temple University School of Medicine Philadelphia, PA Daniel G. Murphy, MD, FACEP Vice Chair & Medical Director Maimonides Medical Center Brooklyn, New York 1 1 1

4. 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.

5. 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.

6. 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 6 5 6

7. 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? 2 2 2

8. 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

9. 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

10. “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

11. 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

12. 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

13. Medical Conditions That Present With Headache
Pheochromocytoma
Hyperthyroidism
SLE
Giant Cell Arteritis
Fibromyalgia

14. Types of Headaches in the ED
Final Diagnosis Percentage
Infection - not intracranial
Tension HA
Miscellaneous
Post-traumatic
Hypertension related
Vascular (Migraine)
No diagnosis
SAH
Meningitis

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
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

22. Time of Death Following SAH by Cause

23. Current Problems in Management of Subarachnoid Hemorrhage
Errors and delays in diagnosis
Treatment of acute effects
Prevention of recurrent hemorrhage
Prevention or treatment of vasospasm or cerebral ischemia

24. Classic Symptoms of Subarachnoid Hemorrhage
Sudden, unusually severe or “thunderclap” headache
Loss of consciousness
Pain in neck, back, eye or face
Nausea, vomiting, photophobia, phonophobia

25. Classic Signs of Subarachnoid Hemorrhage
Abnormal vital signs
Respiratory changes, hypertension, cardiac arrhythmias
Meningismus
Focal neurologic signs may be present
III nerve palsy – IC/PCA aneurysm
Paraparesis – ACA aneurysm
Hemiparesis, aphasia – MCA aneurysm
Ocular hemorrhages

26. 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

27. 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

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

29. Causes of Non-Traumatic Subarachnoid Hemorrhage
“Berry” aneurysms
AVM
Cerebral angiomas
Mycotic aneurysm
Extension from parenchymatous hemorrhage
Anticoagulation therapy

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

31. Warning Headache unusually severe distinct “Thunderclap” headache
% 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. Misdiagnosis of SAH 217 patients from 4 institutions
54 (25%) were initially misdiagnosed
121 patients initially presented in good clinical condition
46 (38%) were initially misdiagnosed
Stroke 1996;27:

34. Misdiagnosis of SAH
Outcome of Patients with Good Initial Presentation in Misdiagnosed and Correctly Diagnosed Patients With SAH
Outcome Misdiagnosis (n=45) Correct Diagnosis (n=75)
Excellent/good (53)* 68 (91)*
Fair (11) ( 5)
Poor/vegetative/dead 16 (36)* ( 4)*
Values are number (%) in each clinical grade category.
P<.001
Stroke 1996;27:

35. Misdiagnosis of SAH
Rebleeds and Deteriorations Before Treatment in Misdiagnosed and Correctly Diagnosed Patients With SAH
Misdiagnosis (n=54) Correct Diagnosis (n=163)
Rebleeds 21* 4
Deteriorations
Total
*12/21 of misdiagnosed and 3/4 of correctly diagnosed patients rebled within 5 days of presentation.
Stroke 1996;27:

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

38. 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

39. 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 < 10: blood isodense
Sensitivity decreases over time from onset of symptoms

41. SAH CT Findings High density hemorrhage injury
(1) Interhemospheric fissure
(2) Inferior frontal sulci
(3) Third ventricle
(4) Ambient cistern
(5)Sylvian fissure

43. SAH: CT Sensitivity Sames: Acad Emerg Med Jan 1996
181 patients; aged with SAH
Sensitivity %
pain < 24 hrs %
pain > 24 hrs %
LP 100% sensitive if neg CT
“A normal NGCT does not reliably exclude the need for LP”

44. SAH Diagnosis: LP Needed Sidman: Acad Emerg Med Sep 1996
140 patients; aged 10-88
Sensitivity of CT
< 12 hrs 80/ %
> 12 hrs 49/ %
Overall, 11/140 had (-) CT and (+) LP
overall sensitivity %

45. 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

46. 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, 0.3%to 8.8%)
Modern CT is sufficient to exclude 98% of SAH in patients

47. Morgenstern, et al: Ann Emerg Med 1998 (107 “Worst HA’s)
Variables CT-/LP CT+ CT-/LP+
Photophobia
Stiff neck
Nausea
Lethargy
Time < 24 h
Migraine
Headache

48. CT is Normal: Do LP?
Yes!

49. 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

50. 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

51. 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 don’t undergo lysis in vitro, causing xanthochromia from oxyhemoglobin

52. Xanthochromia vs. Erythrocytes
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%

53. 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

54. 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?

55. Differentiate Between
Traumatic LP and SAH
CSF characteristic
Traumatic tap
True SAH
Color gets lighter with subsequent tubes
yes no
RBC count in first & last tube
count decrease
stay constant
Clotting of blood in CSF
yes no
present withi4 hrs of SAH, max at 1 wk, persists for about 3 weeks
Xanthochromia in supernatant
rare with RBC count less than 200,000

56. 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 dx’d with migraine or tension HA
Markus 1991; Linn 1994; Harling 1989
117 patients
no SAH, no sudden deaths

57. Vasospasm Occurs in 70% of patients with SAH
Appears after 3-4 days, peaks at 7-10 days, and resolves over 2-4 weeks
Can be localized or involve several arteries
Caused by factors released at time of bleeding that induce vasoconstriction and reduced blood flow

58. Calcium Channel Blocking Drugs
Drugs that limit transmembrane fluxes
Vascular smooth muscle contraction
Cell ischemia
Platelet aggregation
Selective cerebrovascular effects
Cross blood-brain barrier
Limited cardiovascular effects

59. Clinical Trials of Nimodipine
Nimodipine improves survival and functional recovery after SAH
Benefits are due to its antihypertensive and neuronal protective effects

60. The Case (Continued)
The patient had labs drawn, was given 5mg of morphine and sent off to CT scan.
The CT scan revealed an acute SAH. The neurosurgeon ordered an angiogram which revealed an aneurysm.
The patient went to the OR that day and was release with a normal neurological status 4 days later.

61. Case #2

62. First ED Visit: Late Friday Night
24 yo female with headache for 2 weeks, worse over the last 2 days
104/76, 80, 18, 98.1F
Right frontal forehead, sharp, non-radiating, constant but waxing/waning, worse when she moved.
(+) nausea
(-) fever, photophobia, neck pain or visual changes

63. Past Medical/Social History
No recent trauma
Smoker 1 PPD
Social drinker
No hx of headaches, except for last 2 weeks
No allergies
No meds except ibuprofen and acetaminophen recently – not helpful
Worked as a part-time sales clerk

64. Exam: First Visit
Alert, oriented, looked well except for discomfort of headache
Face normal, Perrl, EOMI, fundi normal, TMs normal, mastoids non-tender, neck supple, motor neuro exam normal, normal gait, mental status normal

65. ED Therapy and Work Up
Prochlorperazine 10 mg, by vein Acetaminophen 325/Oxycodone 5, orally
CBC, Chem 7, UCG, CT Head without contrast

66. ED Diagnostic Results: Visit 1
WBC count 12.4K
CT head reviewed by ED attending and radiology resident as negative

68. ED Disposition: Visit 1 Patient’s pain responded to medications
Patient discharged with prescription for acetaminophen/butalbital/caffeine = Fioricet

69. Radiology Over-Read: Monday AM (2.5 days since 1st ED visit)
Opacification of the right ethmoid and right sphenoid sinuses with expansion of the sphenoid septations toward the left.
No intracranial disease

71. ED Discrepancy Procedure
Patient was contacted by phone and informed of sinus problem on CT
Patient went to her PMD that afternoon
PMD discharged her with prescription for levofloxacin

72. 2nd ED Visit: Tuesday Morning (3.5 days after 1st ED visit)
New onset swelling and severe pain around left eye
Continued, worsening right-sided headache
Slept poorly, confused, hallucinating?
100/80, 96, 18, 101.9F

73. Morning Exam: 2nd Visit
Left peri-orbital edema, erythema, proptosis, chemosis, severe pain with EOMs. Left pupil reacted to light.
Ambulated in with normal gait. No obvious motor deficits.
Awake. Followed simple commands, but mildly confused, answering slowly or incorrectly, with difficulty concentrating.
(+) Nuchal rigidity

74. ED Therapy & Work Up 2 grams ceftriaxone by vein after cultures
Repeat CT of brain and sinuses with contrast LP
ID and ENT consults; vancomycin and metronidazole given by vein
Admitted to MICU

77. Afternoon Exam: 2nd Visit
Deteriorating mental status.
Mild left sided weakness left upper and left lower extremities.

78. ED Admitting Diagnoses
Orbital Cellulitis
Meningitis
Rule out Cavernous Sinus Thrombosis

79. Septic Dural Sinus Thrombosis Suppurative Intracranial Thrombophlebitis
Infected venous thrombosis of cortical veins or sinuses
From meningitis, subdural empyema, epidural abscess, infection in the skin of the face, paranasal sinuses, middle ear, mastoid, maxillary teeth or neck.
Iatrogenic cases have been associated with rhinoplasty, hip surgery and oral/dental surgery.

80. Non-Septic Dural Sinus Thrombosis
Dehydration from vomiting
Hypercoagulable states
Immunologic abnormalities, including the presence of circulating antiphospholipid antibodies

81. Septic Dural Sinus Thrombosis
Rare; 155 reported cases since 1940
Cavernous Sinus Thrombosis (CST) is the predominant subset (62%?)
Fulminant, aggressive disease: mortality CST =30%, superior sagittal sinus thrombosis =78%
Morbidity CST: 50% cranial nerve deficit; 17% visually impaired

85. Schematic axial projection of the cavernous sinus and its venous anastomoses.

89. Coronal view of the cavernous sinus
Coronal view of the cavernous sinus. Cranial nerve III is the most superiorly situated nerve in the cavernous sinus. Cranial nerves III, IV, and the first two divisions of the trigeminal nerve, V1 and V2, travel in their respective nerve sheaths laterally in the cavernous sinus. Cranial nerve VI travels more medially within the cavernous sinus adjacent to the cavernous carotid artery. PG = Pituitary gland; S = sphenoid sinus; C = cavernous internal carotid artery.

90. Infected Thrombus Pathogens
CST: Staphylococcus aureus, other gram-positive organisms, and anaerobes.
Lateral Sinus (otitis media and/or mastoid infection) Proteus species, Escherichia coli, S. aureus, and anaerobes.
Superior Sagittal Sinus (meningitis or air sinus infection) - Streptococcus pneumoniae, S. aureus, other streptococci, and Klebsiella.

91. ED Presentation: Superior Sagittal Sinus Thrombosis
Headache, nausea and vomiting, confusion, and focal or generalized seizures.
Rapid development of stupor and coma.
Weakness of the lower extremities with bilateral Babinski signs or hemiparesis is often present.

92. ED Presentation: Transverse Sinus Thrombosis
Headache and earache.
Gradinego's syndrome: otitis media, sixth nerve palsy, and retro-orbital or facial pain.
Sigmoid sinus and internal jugular vein thrombosis may present with neck pain.

93. ED Presentation: Cavernous Sinus Thrombosis
Sinusitis, midface infection for 5-10 days.
Fever, headache, malaise, retro-orbital pain and diplopia, which generally precede…..
Ptosis, proptosis, chemosis, eyelid edema, peri-orbital edema and extraocular dysmotility due to deficits of cranial nerves III, IV, and VI.
Hypo- or hyperesthesia of the ophthalmic and maxillary divisions of V, decreased corneal reflex. dilated, tortuous retinal veins and papilledema.
Meningeal signs: nuchal rigidity, Kernig and Brudzinski signs.

94. Diagnostic Studies CBC, diff, cultures
Sinus Films, CT, MR, MR Venography, Venous phase cerebral angiogram LP

100. ED Management
Antibiotics: S aureus is the usual cause, broad-spectrum coverage for gram-positive, gram-negative, and anaerobic organisms also, pending cultures.
Drain primary source of infection, if feasible (eg, sphenoid sinusitis, facial abscess).
Anticoagulation in carefully selected cases of septic cavernous-sinus thrombosis, not other forms of septic dural-sinus thrombosis.
Urokinase or rtPA?
Corticosteroids?

101. Consults
ENT
Neurology ID
Intensive Care

102. Outcome of Case Day 1: Seizure, worsening deficit, intubated
Day 2: Heparinized, transient neuro improvement then relapse.
Day 5: Sinuses drained
Day 6: Brain dead
Day 19: Demise

103. Acute Headache
Questions ?