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Critical / Killer Headaches

General Approach:

• Emergency department evaluation relies on excluding life-threatening causes of Headache (killers), specifically SAH and meningitis
• Then consider other serious causes of headache that can debilitate the patient (the maimers)
• When these have been excluded, we can then diagnose primary headache syndromes.

Testing

• Question 1: Does this patient need a CT? (ACEP Guidelines Ann Emerg Med 2008;52:407)
  • Neuro exam-new abnormal findings (focal deficit, Altered mental status) (ACEP Level B)
  • New, sudden, severe Headache
  • HIV + with new Headache
  • Patients older than 50 with new type of Headache (ACEP Level C)

• Question 2: Does this patient need an Lumbar Punction?
  • R/O SAH: Sudden-onset, severe headache + CT Head (-) → LP to exclude SAH (ACEP Level B)
  • Concern for Meningits (Cell count, OP, gram stain)
  • Concern for Idiopathic Intracranial Hypertension (Opening pressure)

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1. Subarachnoid Hemorrhage (SAH)

Clinical presentation

• Onset: sudden (thunderclap)
• Maximum severity is reached instantaneously in 50% and within 1-5 minutes in 19% of patients with SAH. (J Neurol Neurosurg Psychiatry 1998;65:791-3)
• Sudden severe Headache: (Lancet 1994:344:590-3)
  • SAH in 25%
  • Benign thunderclap headache in 40%
  • Remaining will have another primary or secondary HA
• Therefore, all patients with sudden severe Headache need to be worked-up for SAH

• Other qualities
  • Quality: qualitatively different headache from previous
  • Severity: +/- worst headache of life
  • Associated sx: Nausea / Vomitus, syncope, Altered Level of Consciousness, diplopia
  • Risk factors: HTN, EtOH (alcohol), smoking, Family History
• Physical Examination:
  • Focal neuro deficit
  • Meningismus (70%)
  • Retinal/vitreal bleed
• Clinical Presentation of SAH lies on a spectrum (Emerg Med Clin N Am 2003;21:73-87)
  • The classic (middle of the spectrum) presentation is that of sudden onset and distinct HA, worst of their life associated with neck pain, Nausea / Vomitus
  • On the extreme end, they can present with focal neuro deficit and Altered Level of Consciousness / Altered mental status
  • On the subtle end 1/3 present with only Headache without neuro deficits, and are likely to be misdiagnosed

SAH Red Flags

□ Sudden onset
□ Maximal at onset
□ Different from previous Headaches

Work-up

• CT Head
  • CT sensitivity is extremely high early but rapidly diminishes with time
  • Misses about 2% of SAH within 12 hours and 7% by 24 hours
  • Therefore anyone with suspected SAH and normal CT requires an Lumbar Puncture
  • Journal Club (EM:RAP 3/14; 12/13)
    • CT within 6 hours (BMJ 2011;343:d4277)
      • Overall CT sensitivity is 93% (Sensitivity after 6h is 86%)
      • CT within 6 hours of HA onset → Sensitivity 100%, Specificity 100%, NPV 100%
      • Prospective cohort study
    • CT within 6 hours (Stroke 2012;43:2115)
      • CT within6 hours of HA onset → Sensitivity 100% with 2 caveats
      • Caveat1: <6h rule only applies to pts with HA (not neck pain)
      • Caveat2: Experienced neuroradiologists needed to interpret CT
    • Editorial (Stroke 2012;43:2031)
      • … we believe that practice should change. Neurologically intact patients who present with thunderclap headache and undergo CT scan within 6 hours of symptom onset no longer need an LP to exclude SAH if the CT scan is negative.”
• Lumbar Puncture
  • Timing: Results depends on timing of HA (J Emerg Med 2002;23:67-74)
  • <12h:
    • Xanthochromia may/may not be present; large RBCs should be present
    • Incompletely clearing RBCs ?? (if suspected traumatic tap → repeat at different interspace)
  • 12h-2weeks:
    • Xanthochromia highly suggestive of SAH; large RBC +/- present 2weeks: Both may be absent.

Treatment (See ICH Algorithm)

• ABC/Resus/Airway
  • Treat like ICH patient
  • Intubate if Altered mental status, not protecting airway → document good neurologic exam prior
• Emergent Neurosurgical consultation
• Vasospasm prevention
  • Nimodipine 60 mg po/NG q4h x 21d

2. Meningitis

Read Also : Diagnosing and Treating Meningitis

Etiology

• Bacterial
  • Meningococcus (Neisseria meningitidis)
  • Streptococcus pneumoniae
  • Haemophilus influenzae
  • Listeria monocytogenes
• Viral
  • Enteroviruses (≈ 85% of meningitis)
  • Arboviruses (West Nile, etc…)
  • Herpes viruses
  • Others (Mumps, LCMV etc…)

Meningitis symptoms

Fever 85%
Stiff neck 70%
Altered mental status 67%
HeadAche 50%
Focal neuro 23%
Rash 22%

Clinical Presentation

• Classic triad
  • All 3: fever, neck stiffness and Altered mental status present in only 44%
    • Absence of all 3 virtually eliminates a diagnosis of meningitis
  • 95% patients had 2 out of 4 of: (NEJM 2004;351:1849-59)
    • Headache
    • Fever
    • Neck stiffness
    • AMS
• Meningismus
  • Jolt accentuation (JAMA 1999;282(2):175-81)
    • Procedure: Ask patient to turn head right/left, 2-3 rotations per second
    • Positive test: Worsening of headache
    • Absence of jolt accentuation has a sensitivity of 100% for ruling out meningitis
  • Kernig’s sign (inability to straighten the leg when the hip is flexed to 90 degrees)
  • Brudzinski’s sign (forced flexion of the neck elicits a reflex flexion of the hips)
    • Kernig/Brudzinski both have very low sensitivity ≈ 5%
• Other symptoms:
  • Abnormal neurologic exam
  • Leg pain, refusal to walk (Meningococcus)
  • Photophobia, N/V, lethargy
  • Petechial rash (Meningococcus)

Bottom Line

• Triad: Absence of all of the triad (fever, Altered mental status, neck stiffness) → eliminates a diagnosis of meningitis
• Meningismus: Kernig/Brudzinski are not helpful in ruling out meningitis (low sensitivity)
  • If they are positive → can rule in meningitis (high specificity)
• Jolt accentuation negative essentially eliminates a diagnosis of meningitis

Work-up

• Lumbar puncture
  • Tube 1&4: Cell count and diff
  • Tube 2: glucose and protein
  • Tube 3: Gram stain, culture, HSV PCR
• Do I need a CT before LP? (2 studies say not always) MAY omit if:
  • Age<60, no immunocompromised/CNS disease/seizure within 1 week and has a nonfocal neuro exam (NEJM 2001;345:1727-1733)
  • Absence of AMS, focal neuro exam, papilledema and favorable clinical impression by doctor (Ann Int Med 1999;159:2681-2685)
• CSF analysis (predictive of bacterial meningitis) (JAMA. 1989;262(19):2700)
  • ↑ WBC > 2000
    • Multiple studies show bacterial meningitis incidence of 5-19% in patients with CSF WBC < 100!
    • No single variable can r/o meningitis
  • Neutrophil > 1180
  • CSF glucose <34mg/dL
  • CSF protein > 220 mg/dL
  • Gram stain +

Treatment

• Antibiotics
  • Timing
    • Administer as soon as possible-before Lumbar puncture
    • Multiple studies have shown an association between time of antibiotic administration and poor outcome (greater than 4-6 hours)
  • Empiric antibiotics
    • Ceftriaxone 2gm IV or Cefotaxime 2gm IV immediately +
    • Vancomycin 1gm IV (15-20mg/kg) +
    • Ampicillin 2gm IV (adults > 50yo)
• Dexamethasone
  • Dose: 10mg IV q 6 hour x 4 days; start 15 minutes prior to, or with first dose of antibiotics
  • Journal club (Cochrane Database 2010;9:CD00405)
    • No mortality benefit but helps prevent hearing loss and short-term neurologic sequelae
    • Benefits seen mostly in patients with S. pneumoniae
• Acyclovir
  • Dose: 10mg/kg q 8h for HSV

Special Case: HA and HIV (CD4 < 200?)

• CT w/wo contrast:
  • R/O mass lesion, toxo, lymphoma
• Serum
  • If serum crypto Ag(-) → likely do not need Lumbar puncture (patient very unlikely to have crypto infection)
• Lumbar puncture
  • Use: r/o cryptococcal meningitis
  • India ink: round encapsulated yeast (75% patients)
  • CSF crypto Ag: Sensitivity 93 to 100% and specificities 93 to 98%
  • CSF culture: definitive diagnosis
• Treatment: Amphotericin + Fluconazole

3. Cerebral Venous Thrombosis

General

• CVT = DVT of the brain = blood clots in brain (venous)
• CVT = Dural sinus thrombosis, (superior, sagittal, inferior) sinus thrombosis, cortical vein thrombosis

Risk factors

• Thrombophilia/Hypercoagulable state
• Pregnancy/Puerperium
• Medications (Oral contraceptive pills, Doxycycline)
• Cancer/Inflammatory/Hematologic disease

Clinical

• Presentation spectrum: Headache → Seizure → Stroke
• Headache
  • Headache type: No characteristic history, multiple types of headache symptoms
  • Physical Exam – Papilledema present in most cases (fundoscopic, pan-optic or US)
• Seizure
• Stroke
  • Bilateral neuro deficit
  • Non-arterial distribution

Diagnosis

• D-dimer – low sensitivity, not useful
• CT Head – normal in 30% of cases
  • Highly suggestive:
    • Dense triangle sign (thrombosed sup sag sinus posteriorly)
    • Cord sign (thrombosed cortical vein)
    • Bilateral edema/ICH
  • Diagnostic (CT w/ contrast-venogram): Empty delta sign (flow defect in sup sag sinus)
• MR Brain with venography (study of choice)

Complications

• Intracranial Hypertension (ICH) → Coma → Death
• PE (pulmonary embolism)

Treatment

• Neurosurgical consultation
• Anticoagulation
  • Heparin safe, even with hemorrhagic infarcts
• Journal Club-Heparin anticoagulation
  • Cochrane Review of Heparin anticoagulation (Cochrane Database Syst Rev 2001;(4))
    • RR of death 0.33 (95% CI 0.08-1.21) → not statistically significant
    • Conclusion: CVT is a rare and fatal disease and no other treatment exists
  • ISCVT-International Study on Cerebral vein and Dural Venous Thrombosis (Stroke 2004;35:664-70)
    • Prospective observational study 624 patients → 80% received anticoagulation
    • 6.8% mortality

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Emergent Diagnoses / Emergent Causes of Headache

1. Brain Mass Lesion

Etiology

• Tumor, abscess, chronic subdural hematoma (SDH)

Clinical Presentation

• Progressive, exertional headache, morning headache
• New onset seizure, nausea/vomiting
• H/O HIV (cryptococcus, toxoplasmosis, lymphoma, and tuberculosis)
• Physical Exam : Focal neuro exam, papilledema, aphasia

Work-up

• CT Head w/wo contrast, MRI

Treatment

• Neurosurgical consultation, steroids, ventriculostomy

Vasogenic edema

• Etiology: tumor related disruption of BBB
• Treatment:
  • Glucocorticoids – indicated in all patients with peri-tumor edema
  • Dexamethasone 10mg IV x 1 then 4mg IV q6h

HYDROCEPHALUS

• Cause: CNS infection, hemorrhage, tumor
• Types
  • Communicating hydrocephalus
    • Symmetric dilation of all 4 ventricles
    • Extraventricular obstruction or impaired CSF absorption, usually in neonates/infants;
    • Adult causes: pseudotumor cerebri
  • Non-communicating (Obstructive) hydrocephalus
    • Asymmetric dilation of ventricles
• Treatment
  • Third Ventriculostomy – only effective for obstructive hydrocephalus
  • Shunt (VP-Shunt) – can be used to treat either cause of hydrocephalus

SHUNT COMPLICATIONS

• Shunt Infection (Ventriculitis)
  • 5 to 15 percent of procedures, most occurring in first 6 months
  • Persistent fever → tap shunt
  • Antibiotics, shunt may need removal
• Shunt mechanical failure (obstruction at the ventricular catheter)
  • CT: Hydrocephalus
• Overdrainage
  • Presentation: neurological symptoms → postural headache and nausea
  • CT: Slit ventricle syndrome (small or slit-like ventricles)

2. Idiopathic Intracranial Hypertension (IIH)

General

• AKA Idiopathic Intracranial Hypertension, Pseudotumor cerebri

Presentation

• Obese, young female
• Headache x weeks/months
• Visual disturbances
• Papilledema
  • Not all patients with IIH have papilledema

Diagnosis

• Diagnostic criteria for IIH: (Neurology 2002;59:1492-95)
  • S/Sx of elevated intracranial pressure
  • Non focal neuro exam (except abducens nerve paresis)
  • Normal neuroimaging study (CT w/ con or MRI/MRV)
  • Increased CSF pressure (nonobese>200; obese>250 mm water)
  • No other cause of increased intracranial pressure (CNS tumor, encephalitis, right heart failure)
• Vision loss secondary to papilledema is the only serious complication of IIH, is avoidable with appropriate treatment
• MRI/MRV to exclude CVT

Treatment

• Indications for treatment: Vision loss (emergent tx), headache (therapeutic)
• Weight loss
• Medications
  • Acetazolamide (carbonic anhydrase inhibitor) reduces CSF pressure
  • Topiramate (partial CAI) reduces CSF pressure
• Surgical
  • VP Shunt (vision loss and HA refractory to treatment)
  • Optic nerve sheath fenestration
  • Venous stenting

3. Temporal Arteritis

Clinical Presentation

• New headache
• Temporal artery tender / decrease pulse
• Jaw/tongue claudication
• Vision change

Diagnosis (need 3/5)

• Age >50
• New headache
• Temporal artery tender / decrease pulse
• ESR > 50
• Biopsy: vasculitis/granuloma

Treatment

• No ischemic organ damage (eg, visual loss) → begin empiric prednisone 40-60mg if medium or high suspicion until ESR or biopsy results return.
• Ischemic organ damage (AION) – Vision loss → Methylprednisolone 1000mg IV daily x 3 days

4. Acute Glaucoma

Clinical Presentation

• Eye pain, Nausea / Vomitus, blurred vision
• Conjunctival hyperemia
• Corneal edema, mid-dilated, non reactive pupil
• Elevated IOP (>20)

Treatment

• Emergent Ophthalmology consultation
• Decrease IOP (intra ocular pressure)
  • 0.5% timolol maleate (Timoptic)
  • 1% apraclonidine (Iopidine)
  • 2% pilocarpine (Isopto Carpine)
  • Acetazolamide 500mg IV then 500mg po

5. Cervical Artery Dissection

General

• Cervical artery dissection = carotid artery dissection + vertebral artery dissection
• Common cause of pediatric strokes (1 in 5)
• Cause:
  • Major trauma: penetrating or blunt
  • Minor trauma: coughing, whiplash, cervical seatbelt signs, rotational routine neck movement, chiropractor manipulation

Presentation

• Stroke syndromes:
  • Carotid artery dissection: MCA (Middle cerebral artery), anterior circulation stroke syndromes (hemiplegia)
  • Vertebral artery dissection: posterior fossa symptoms (ataxia, vertigo, dysmetria)
  • Wallenberg Syndrome: ipsilateral facial numbness, contalateral body numbness
  • Horner’s Syndrome: miosis, ptosis, anhydrosis (vertebral & carotid artery dissection)
• Facial (carotid), neck (vertebral) pain
• SAH like acute onset
• Cranial nerve abnormalities

Diagnosis

• Carotid duplex – poor sensitivity
• CT Angiography
• MRI/MRA
• Angiography

Treatment

• Neurological/Neurosurgery consultation
• Anticoagulation
  • Contraindicated if concomitant cerebral hemorrhage
  • Used to decrease risk of thrombus formation and embolization

6. Carbon Monoxide Poisoning

History and Physical

• Exposure ?
• Multiple patients / similar location /similar symptoms
• Headache with N/V/ syncope
• Altered mental status
• Cherry-red mucous membranes
• Cerebellar ataxia

Action

• COHb level
• O2 (100% NRM vs HBO)

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Urgent Headaches

• Acute Sinusitis
• Obstructive hydrocephalus
• Benign Exertional HA
• Post-Traumatic
• Drug/Food Related
• Cranial Neuralgias

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Primary Headaches

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1. Migraine Headache

Presentation

• Common migraine
  • Severe, throbbing, unilateral HA
  • Photo/Phonophobia
  • Hours to days
• Classic migraine
  • Preceded by aura
  • +/- GI distress
• POUNDing mnemonic
  • Pulsating, duration 4-72 hOurs, Unilateral, Nausea, Disabling
  • Highly predictive of migraine headache (JAMA 2006;296:1274-83)

Treatment

• IV Dopamine antagonist (Compazine, Reglan)
• IVF (Normal Saline)
• Opiates
• Triptans/NSAIDS
• Dexamethasone

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2. Cluster Headache

Presentation

• Middle aged men
• Sudden onset Headache
• Unilateral blur vision / nasal congest / lacrimate / salivate / sweat
• Lasts hours, recur same time of day
• Precipitated by exertion / stress

Treatment

• O2
• Symptomatic relief
• Intranasal lidocaine
• Neurology referral

3. Tension Headache

Presentation

• Frontal/occipital/band-like
• No preceding event

Treatment

• Symptomatic relief (NSAIDS, Tylenol)