Unilateral Headache with Neck Radiation and Positional Worsening
This presentation demands urgent evaluation for spontaneous intracranial hypotension (SIH) or subarachnoid hemorrhage (SAH), as the combination of unilateral headache radiating to the neck with worsening when lying flat represents a critical red flag that requires immediate neuroimaging and potentially lumbar puncture.
Critical Life-Threatening Diagnoses to Exclude First
Subarachnoid Hemorrhage
- Neck pain or stiffness combined with severe headache is a criterion in the Ottawa SAH Rule that mandates further investigation for SAH 1
- Obtain non-contrast head CT immediately if presenting within 6 hours of symptom onset (sensitivity 98.7% when interpreted by fellowship-trained neuroradiologists) 1
- If CT is negative or patient presents >6 hours after onset, perform lumbar puncture for xanthochromia evaluation (sensitivity 100%, specificity 95.2% by spectrophotometric analysis) 1
- CTA has sensitivity of only 61% for ruptured aneurysms <3mm, making LP essential when clinical suspicion remains high 1
Spontaneous Intracranial Hypotension
- The positional component (worse lying flat) is atypical for classic SIH but the neck pain with unilateral headache pattern warrants consideration 2
- SIH classically presents with orthostatic headache (better lying down, worse upright), but atypical presentations exist 2
- First-line imaging: MRI brain with IV contrast showing diffuse pachymeningeal enhancement, plus MRI complete spine to localize CSF leak source 2
- Do not exclude SIH based on normal CSF opening pressure alone—clinical presentation and imaging findings are more important 2
Primary Differential Diagnoses
Cervicogenic Headache (Most Likely if Life-Threatening Causes Excluded)
- Cervicogenic headache characteristically presents as unilateral pain starting in the neck and spreading to the ipsilateral oculo-fronto-temporal region, with pain provoked by cervical movements 3
- Key diagnostic features include: cervical spine tenderness, paraspinal and suboccipital muscle tenderness, limitation of cervical motion, and pain with cervical movement 3
- Digital pressure over triggering areas at the upper nuchal region reproduces the spontaneous pain pattern 4
- Affects approximately 4% of the general population and up to 20% of patients with chronic headaches 3
Note on positional worsening: The worsening when lying flat is somewhat atypical for cervicogenic headache, which is typically provoked by movement rather than posture 3. This unusual feature elevates concern for other diagnoses.
Vestibular Migraine or Migraine Variant
- Unilateral location is a diagnostic criterion for migraine without aura 1
- Migraine can present with neck pain due to shared trigeminal-cervical pathways 1
- Consider if patient has history of recurrent attacks (≥5 attacks for migraine without aura) lasting 4-72 hours with pulsating quality, moderate-severe intensity, and associated nausea/photophobia/phonophobia 1
Other Critical Considerations
- Vascular dissection must be excluded in the setting of unilateral headache and neck pain 3
- Postural orthostatic tachycardia syndrome (POTS): increase in heart rate >30 beats/minute during standing test 3
- Orthostatic hypotension: drop in systolic BP >20 mmHg and/or diastolic BP >10 mmHg 3
Diagnostic Algorithm
Immediate Workup (Emergency Department)
Detailed history focusing on:
Physical examination:
Imaging based on presentation timing:
Outpatient Workup (If No Red Flags)
- MRI is the imaging modality of choice for cervicogenic headache assessment, though imaging is not diagnostic given lack of definitive criteria and high frequency of abnormal findings in asymptomatic patients 3
- Physical therapy evaluation is appropriate as first-line diagnostic and therapeutic approach 3
Treatment Approach
If Cervicogenic Headache Confirmed
- Physical therapy with cervical spine mobilization and stabilization is the first-line treatment recommended by the American College of Physicians 3
- Combined program of manual therapy and motor control exercises represents the most effective intervention with long-term maintained results 3
- Greater occipital nerve block can provide short-term relief and serve diagnostic/therapeutic purposes 3
- Percutaneous interventions (facet joint injections, cervical epidural steroid injections) reserved for refractory cases 3
If SIH Confirmed
- Early epidural blood patch (EBP) is first-line treatment and should be performed as soon as possible after diagnosis 2
- Non-targeted EBP appropriate initially; myelography reserved for failed cases 2
Critical Pitfalls to Avoid
Do not dismiss the atypical positional component (worse lying flat)—this is opposite to typical cervicogenic headache and demands consideration of SIH or increased intracranial pressure 1, 3, 2
Do not rely on imaging alone for cervicogenic headache diagnosis—clinical examination findings are paramount 3
Do not skip lumbar puncture if SAH remains in differential after negative CT, especially if >6 hours from onset 1
Do not exclude SIH based on normal opening pressure alone 2
In patients with neck pain and headache, always consider cervical artery dissection, particularly if there is history of trauma or sudden onset 3