Headache: The Ominous Causes

Introduction

Most headaches are benign and do not require a specific workup. Here are the ominous ones that require a specific workup and management.

From the Patient History

• Sudden, severe, and maximal at onset, especially in an older patient without a prior history of headaches → subarachnoid hemorrhage → get a head CT without contrast → CT angiogram or cerebral angiogram.
• Systemic symptoms
  • Weight loss → cancer, infection
  • Fevers, chills → meningitis, encephalitis
• New onset headache in an older adult (e.g., > age 55) →suspect giant cell arteritis (“temporal arteritis”)
  • Local signs and symptoms
    • Unilateral vision loss
    • Jaw claudication: pain on chewing (secondary to masseter muscle ischemia)
    • Non-pulsatile, tender or indurated temporal artery
  • Systemic signs and symptoms
    • fever, weight loss, proximal muscle aches
    • concomitant polymyalgia rheumatica is common (↑ ESR, ↑ WBC, ↓ Hb/Hct)
  • Treat immediately with high dose steroids, followed by a biopsy of the temporal artery.
• Progressively worsening headache or vomiting (in terms of severity and/or frequency) → expanding intracranial lesion with mass effect (e.g., hematoma or tumor), increasing intracranial pressure.
• Postural headaches
  •  Worse when in upright position, relieved with recumbency (orthostatic headache) → intracranial hypotension.
  • Worse with bending over or prolonged recumbency (e.g., early morning headache) → intracranial hypertension (from tumor or idiopathic) → image brain.
    • Also worse with coughing, sneezing and Valsalva.
• With acute, unilateral retro-orbital headache or neck pain → cervical artery dissection → get CT or MR angiogram of the head and neck.
• During pregnancy or peripartum period. As with non-pregnant patients, most are benign. The worrisome ones are:
  • Preeclampsia → look for new hypertension, proteinuria
  • Idiopathic intracranial hypertension (“pseudotumor cerebri”). Need:
    • Evidence of intracranial hypertension:
      • Ophthalmoscopy → papilledema
      • Lumbar puncture → increased opening pressure
    • Proof of idiopathic nature:
      • MRI/MRV → no masses or cerebral venous sinus thrombosis
      • Lumbar puncture → normal CSF composition
      • Exclusion of secondary causes of intracranial hypertension (e.g., hypervitaminosis A, adrenal insufficiency, hypoparathyroidism)
  • Cerebral venous sinus thrombosis → papilledema, especially in a patient with seizures and a family history of thrombophilia → get an MRI/MRV of the brain or look for the empty delta sign on CT venogram of the brain.
  • Carotid artery dissection → CTA or MRA of the head and neck
  • Postpartum infarction of anterior pituitary gland (Sheehan syndrome) → headache and inability to lactate in the setting of a delivery complicated by hypotension → ✓ serum prolactin level and image the brain
• Ongoing systemic infection (e.g., endocarditis) → septic emboli.
• History of malignancy → brain metastasis.
• HIV and other immunocompromised states → opportunistic infections (e.g. cerebral toxoplasmosis, cryptococcal meningitis), CNS lymphoma → image the brain.
• Headache, especially frontal, with involvement of household members or other cohorts (including sick pets!!), especially in the setting of a source of incomplete combustion → carbon monoxide poisoning → ✓ carboxyhemoglobin level → administer 100% oxygen via non-rebreather mask.

The Physical Examination

• Very high blood pressure (e.g., > 180) → hypertensive encephalopathy.
  • The triad of paroxysmal headaches, sweating and tachycardia should suggest the possibility of a pheochromocytoma → ✓ Plasma free metanephrines
• Any new neurological problem (e.g., seizure, confusion, focal deficits, personality changes) → investigation required.
• Papilledema → intracranial hypertension → image the brain → lumbar puncture if brain imaging is unrevealing.
• Meningeal irritation (headache, photophobia, nuchal rigidity, positive Kernig sign) → meningitis or subarachnoid hemorrhage → needs a lumbar puncture, unless the question can be answered with brain imaging along (e.g., subarachnoid hemorrhage).
• Blurry vision, red eye +/- fixed at midpoint or sluggish pupil, cloudy cornea, increased intraocular pressure → acute angle-closure glaucoma →
  • beta blockers (e.g., timolol),
  • alpha2 agonists (apraclonidine),
  • carbonic anhydrase inhibitors (e.g., acetazolamide),
  • prostaglandins (e.g., bimatoprost)
  • urgent ophthalmology referral.
• Painful, grouped vesicular rash in dermatomal distribution → herpes zoster virus → treat with an antiherpes drug (e.g., acyclovir, ganciclovir).
• Bilateral proptosis, ecchymosis, ocular bruit → carotid-cavernous sinus fistula → get a CT angiogram of the head and neck → neurosurgery consult.

Workup

Be especially vigilant about serious causes of headache in which brain imaging can be negative:

• Meningitis
• Subarachnoid hemorrhage (“sentinel bleed”)
• Giant cell arteritis
• Idiopathic intracranial hypertension
• Intracranial hypotension
• Carbon monoxide poisoning