Basics
Description
- Pain in the cranium, orbits, or upper neck
- Pain within the skull is projected to the surface:
- Intracranial:
- Arteries, veins, dura, meninges
- Extracranial:
- Skin, scalp, fascia, muscles
- Mucosal linings of the sinuses
- Arteries
- Temporomandibular joints, teeth
- Pain is transmitted via the V cranial nerve.
- May be caused by a number of mechanisms:
- Nerve irritation
- Traction on pain-sensitive vessels
- Vasodilatation of pain-sensitive vessels
- Hypoxia, hypercapnia, fever, histamine injection, nitroglycerin ingestion
- Complaint in 2-4% of all ED visits:
- 95% have a benign etiology (lower in patients older than 50 yr)
- Life-threatening etiologies are rare and can be difficult to diagnose.
Etiology
- Migraine:
- Intra/extracranial vasodilatation and constriction of pain-sensitive blood vessels
- May also involve cortical depression
- Throbbing headache
- Tension:
- Requires ≥10 attacks of a similar nature
- Unknown etiology (possibly serotonin imbalance, decreased endorphins, spasm)
- Most common type of recurring headache
- Triggered by poor posture, stress, anxiety, depression, cervical osteoarthritis
- Bilateral, nonpulsatile, band like
- Mild to moderate intensity
- 4-13 hr duration
- Cluster headaches:
- Triggered by alcohol, certain foods, altered sleep habits, strong emotions
- May involve vasospasm near cranial nerves
- Intracranial (traction, pressure):
- Mass lesions
- Idiopathic intracranial hypertension
- Extracranial (compression):
- Pathology causing pain in a peripheral nerve of the head and neck
- Inflammation:
- Temporal arteritis
- Cerebral vasculitis
- Thrombosis:
- Cerebral venous sinus thrombosis (CVST)
- Impaired vascular autoregulation/endothelial dysfunction:
- Posterior reversible leukoencephalopathy syndrome (PRES)
- Reversible cerebral vasoconstriction syndrome (RCVS)
Serious causes of headache in children are rare but those who come to the ED for this complaint should all have follow-up with a pediatrician.
Older patients with new headache have a higher likelihood of a serious etiology and should have more thorough evaluation with a low threshold for imaging.
In addition to all other causes of headache, pregnant women (and recently postpartum women) are at increased risk for CVST, eclampsia, PRES, and RCVS.
Diagnosis
Signs and Symptoms
History
- Attributes of the pain-PQRST:
- Provocative and palliative features:
- Position of the head, coughing or straining (increase suggests elevated ICP), and movement
- Quality:
- Throbbing or continuous
- Deep or superficial
- Change compared to prior headaches
- Region
- Severity
- Worst headache of life?
- Timing
- Sudden or gradual?
- Associated findings:
- Visual symptoms, dizziness, nausea, vomiting
- Historical factors indicating additional testing:
- New onset:
- Age >50
- HIV, transplant, or cancer patient?
- Trauma or falls (even without headstrike)
- Persistent vomiting
- Any new focal neurologic or visual symptoms
- Risk factors for cerebral sinus thrombosis:
- Malignancy
- Pregnancy (or postpartum)
- Protein S or protein C deficiency
- Oral contraceptive
- Ulcerative colitis
- Beh §et syndrome
Physical Exam
- Complete neuro exam including cranial nerves, motor, sensation, deep tendon reflexes, gait
- Examine for papilledema.
- Evaluate skin for rashes:
- Palpate temporal arteries
Essential Workup
- Detailed history, CNS, HEENT, and neck exam
- Factors indicating testing beyond the history and physical exam:
- Severely elevated diastolic BP
- Fever
- Altered level of consciousness
- Papilledema
- Abnormal neurologic exam or meningismus
Diagnosis Tests & Interpretation
Lab
- CSF:
- Essential in suspected meningitis, subarachnoid hemorrhage (SAH)
- ESR:
- If temporal arteritis or other inflammatory disorders suspected:
Imaging
- Head CT scan:
- Indications:
- Uncertain diagnosis based on history and physical exam (leaving open the possibility of serious causes)
- Signs of increased ICP
- "First or worst" headache
- Abrupt onset
- New focal neurologic abnormalities
- Papilledema
- Recurrent morning headache
- Persistent vomiting
- Associated with fever, rash, and nausea
- Trauma with loss of consciousness, focal deficits, or lethargy
- Altered mental status, meningismus
- Definitive test for SAH if performed within 6 hr of onset and read by an attending radiologist
- Within 24 hr, >95% sensitive (sensitivity falls rapidly with time and is 50% at 7 days out)
- Sinus imaging may show acute sinusitis; chronic sinusitis rarely causes acute headache.
- MRI:
- Indicated to assess for etiologies that are missed by CT scan and LP:
- Posterior fossa lesion
- Pituitary apoplexy
- CVST
- MRA:
- Indicated if SAH suspected, CT is negative, and unable to perform lumbar puncture
- Suspicion of carotid or vertebral dissection (e.g., recent neck manipulation or trauma)
- Nonmigrainous vascular cause suspected (e.g., RCVS)
Diagnostic Procedures/Surgery
Lumbar puncture:
- Perform CT 1st if:
- New focal neurologic finding
- Papilledema
- Abnormal mental status
- HIV positive or immunosuppressed
- Detect intracranial and meningeal infections
- Detect blood not evident on CT scan:
- There is no specific threshold number of red cells below which SAH is excluded - the RBC count is a function of time from onset.
- Opening pressure:
- Essential to diagnose pseudotumor cerebri and CVST
- Can distinguish traumatic tap vs. true hemorrhage.
- Xanthochromia:
- Should be visible by 12 hr after onset of a SAH
- Visual inspection is the most commonly used method - spectrometry (is more sensitive but has a high false-positive rate).
Differential Diagnosis
- Note: There can be significant overlap in these groupings.
- Acute single headache:
- SAH
- Meningitis
- Vascular:
- Acute intracerebral hemorrhage
- Hypertensive encephalopathy
- Cranial artery dissection
- CVST
- Cerebellar stroke
- Ocular:
- Acute narrow-angle glaucoma
- Pituitary apoplexy
- Temporal neuritis
- Traumatic
- Acute sinusitis
- Toxic/metabolic:
- Fever
- Hypoglycemia
- High-altitude disease
- Carbon monoxide poisoning
- Narcotic, alcohol, or benzodiazepine withdrawal
- Post lumbar puncture
- Cold stimulus headache
- Acute recurrent headache:
- Presenting within days to weeks of onset
- CVST
- Pseudotumor cerebri
- Temporal arteritis
- SAH (rebleed)
- Migraine, cluster, tension
- Hypoxic
- Trigeminal neuralgia
- Postherpetic neuralgia
- Coital and exertional headache
- Subacute headache:
- Within weeks to months of onset
- Chronic subdural hematoma
- Brain tumor
- Brain abscess
- Chronic sinusitis
- Temporomandibular joint syndrome
- Chronic post-traumatic headache
- Pseudotumor cerebri (idiopathic intracranial HTN)
- Temporal arteritis
- Chronic headache:
- Months to years since onset
- Chronic tension headache
- Analgesic abuse/rebound
- Depression
- Extracranial:
- Trigeminal neuralgia: Transient, shock like facial pain
- Temporal arteritis: Elderly, severe, scalp artery tenderness/swelling
- Metabolic: Severe anemia
- Acute glaucoma: Nausea, eye pain, conjunctival injection, increased IOP
- Cervical: Spondylosis, trauma, arthritis
Treatment
Initial Stabilization/Therapy
- ABCs if altered mental status
- Empiric antibiotics if bacterial meningitis is suspected, acyclovir if immunocompromised
Ed Treatment/Procedures
- Migraine (See Headache, Migraine)
- Tension:
- Aspirin
- Acetaminophen
- NSAID
- Nonpharmacologic (meditation, massage, biofeedback)
- Cluster (See Headache, Cluster)
- Temporal arteritis (See Giant Cell Arteritis)
- Intracranial infection (See Meningitis)
- Intracranial hemorrhage (See Subarachnoid Hemorrhage)
Follow-Up
Disposition
Admission Criteria
- Headache secondary to suspected organic disease
- Intractable vomiting and dehydration
- Pain refractory to outpatient management
- Consider ICU admission:
- Suspected symptomatic aneurysm
- Acute subdural hematoma
- SAH
- Stroke
- Increased ICP
- Intracranial infection
Discharge Criteria
- Most migraine, cluster, and tension headaches after pain relief
- Local or minor systemic infections (e.g., URI)
Issues for Referral
Patients with recurrent headaches should have follow-up with a neurologist or PCP.
Treatment
Medication
- Chlorpromazine: 25-50 mg IM/IV (peds: 0.5-1 mg/kg/dose IM/IV/PO) q4-6h
- Dexamethasone: 10-24 mg IV once
- Dihydroergotamine: 1 mg IM/IV, repeat q1h; max. dose 3 mg
- Ergotamine: 2 mg PO/SL at onset, then 1 mg PO q30min; max. dose 10 mg/wk
- Ketorolac: 30-60 mg IM; 15-30 mg IV once, then 15-30 mg q6h (peds: 1 mg/kg IV q6h)
- Lidocaine 4%: 1 mL intranasal on same side as symptoms
- Metoclopramide: 5-10 mg IM/IV/PO q6-8h
- Morphine: 2.5-20 mg (peds: 0.1-0.2 mg/kg/dose) IM/IV/SQ q2-6h
- Prochlorperazine: 5-10 mg IM/IM/PO TID-QID; max. 40 mg/d
- Sumatriptan: 6 mg SQ, repeat in 1 hr, up to 12 mg/24h
DO NOT use the response to any medication to indicate a benign cause of a headache.
Pearls and Pitfalls
- The sensitivity for detecting SAH on CT scan falls rapidly after 24 hr. LP remains essential for all patients with suspected SAH presenting after 6 hr of symptom onset.
- Neurology consultation should not delay urgent imaging in patients with high-risk features.
- Use dopamine antagonists with caution in patients with QT prolongation or electrolyte abnormalities. Use ergotamines and triptans carefully in patients with a documented history of CAD.
- Patients with chronic headaches and multiple visits benefit from consistent protocols for pain management; however, be alert to significant changes in their symptoms
- Do not wait for LP results to empirically treat cases of suspected meningitis.
Additional Reading
- Edlow JA, Caplan LR, O'Brien K, et al. Diagnosis of acute neurological emergencies in pregnant and post-partum women. Lancet Neurol. 2013;12:175-185.
- Perry JJ, Stiell IG, Sivilotti ML, et al. Sensitivity of computed tomography performed within six hours of onset of headache for diagnosis of subarachnoid haemorrhage: Prospective cohort study. BMJ. 2011;343:d4277.
- Pope JV, Edlow JA. Avoiding misdiagnosis in patients with neurological emergencies. Emerg Med Int. 2012;2012:949275.
- Swadron, SP. Pitfalls in the management of headache in the emergency department. Emerg Med Clin North Am. 2010;28(1):127-147.
Codes
ICD9
- 339.00 Cluster headache syndrome, unspecified
- 346.90 Migraine, unspecified, without mention of intractable migraine without mention of status migrainosus
- 784.0 Headache
- 339.00 Cluster headache syndrome, unspecified
- 339.02 Chronic cluster headache
- 346.10 Migraine without aura, without mention of intractable migraine without mention of status migrainosus
- 339.01 Episodic cluster headache
- 346.00 Migraine with aura, without mention of intractable migraine without mention of status migrainosus
- 346.90 Migraine, unspecified, without mention of intractable migraine without mention of status migrainosus
- 346.30 Hemiplegic migraine, without mention of intractable migraine without mention of status migrainosus
- 350.2 Atypical face pain
- 437.4 Cerebral arteritis
- 446.5 Giant cell arteritis
ICD10
- G43.909 Migraine, unsp, not intractable, without status migrainosus
- G44.009 Cluster headache syndrome, unspecified, not intractable
- R51 Headache
- G43.009 Migraine w/o aura, not intractable, w/o status migrainosus
- G44.009 Cluster headache syndrome, unspecified, not intractable
- G44.029 Chronic cluster headache, not intractable
- G43.109 Migraine with aura, not intractable, w/o status migrainosus
- G43.909 Migraine, unsp, not intractable, without status migrainosus
- G44.019 Episodic cluster headache, not intractable
- G43.409 Hemiplegic migraine, not intractable, w/o status migrainosus
- G44.001 Cluster headache syndrome, unspecified, intractable
- G50.1 Atypical facial pain
- G44.00 Cluster headache syndrome, unspecified
- G44.011 Episodic cluster headache, intractable
- G44.01 Episodic cluster headache
- G44.021 Chronic cluster headache, intractable
- G44.02 Chronic cluster headache
- G93.2 Benign intracranial hypertension
- I67.7 Cerebral arteritis, not elsewhere classified
- M31.6 Other giant cell arteritis
SNOMED
- 193031009 Cluster headache syndrome (disorder)
- 25064002 Headache (finding)
- 37796009 Migraine (disorder)
- 193031009 Cluster headache syndrome (disorder)
- 230473009 Chronic cluster headache
- 56097005 Migraine without aura
- 230472004 Episodic cluster headache
- 37796009 Migraine (disorder)
- 4473006 Migraine with aura (disorder)
- 59292006 Hemiplegic migraine (disorder)
- 71303008 Atypical facial pain (finding)
- 400130008 Temporal arteritis (disorder)
- 427020007 cerebral vasculitis (disorder)
- 68267002 Benign intracranial hypertension (disorder)
- 79267007 Retinal migraine (disorder)
- 83351003 Basilar migraine (disorder)