Immediate Lumbar Puncture to Rule Out Spontaneous Intracranial Hypotension
This patient requires an urgent lumbar puncture with opening pressure measurement to evaluate for spontaneous intracranial hypotension (SIH), which is the most likely diagnosis given the positional headache pattern (worse when bending over or laying down) and failure to respond to standard migraine therapy. 1
Critical Red Flags Present
This case demonstrates several features that distinguish it from typical migraine:
- Positional worsening with bending over or laying down is the opposite of classic orthostatic headache but can occur in SIH variants, particularly when there is venous engorgement or intracranial pressure fluctuations 1
- Complete refractoriness to both oral and IV migraine medications for 5 consecutive days suggests this is not a typical migraine attack 1
- Progressive worsening over 7 months with associated joint pain raises concern for secondary headache disorders 1
- Normal CT and MRI do not exclude SIH, as early or mild cases may not show typical findings like brain sagging, pachymeningeal enhancement, or subdural collections 1
Diagnostic Approach
Perform lumbar puncture with opening pressure measurement immediately:
- Opening pressure <6 cm H2O strongly suggests SIH 1
- Send CSF for cell count, protein, glucose to exclude infectious or inflammatory causes 1
- Even if opening pressure is normal, SIH can still be present due to intermittent CSF leaks 1
If LP opening pressure is low or clinical suspicion remains high despite normal pressure:
- Order MRI brain and spine with gadolinium specifically looking for pachymeningeal enhancement, venous engorgement, brain sagging, and CSF leak 1
- Consider CT myelography or dynamic MR myelography to identify the leak site 1
Why Standard Migraine Treatment Failed
The complete lack of response to standard acute migraine therapy (NSAIDs, triptans, antiemetics, IV metoclopramide/ketorolac combinations) over 5 days strongly argues against this being typical migraine 1. True intractable migraine typically shows at least partial response to IV therapy, particularly the combination of metoclopramide 10 mg IV plus ketorolac 30 mg IV 1.
Joint Pain Connection
The 4-month history of joint pain concurrent with headache progression raises additional diagnostic considerations:
- Connective tissue disorders (particularly Ehlers-Danlos syndrome) are associated with spontaneous CSF leaks and SIH 1
- While autoimmune workup was negative, consider testing for specific connective tissue markers if not already done 1
- Joint hypermobility examination should be performed 1
Treatment Algorithm Based on Findings
If SIH is confirmed:
- Conservative management first: strict bed rest, aggressive hydration (3-4 L/day), caffeine 300-500 mg daily 1
- If no improvement in 48-72 hours: epidural blood patch (EBP) is definitive treatment with 70-90% success rate 1
- Targeted EBP at leak site (if identified) has higher success than blind lumbar EBP 1
If SIH is excluded and this is truly refractory migraine:
- Initiate preventive therapy immediately with propranolol 80-240 mg/day or topiramate, as this patient clearly meets criteria for prevention (>2 attacks/month causing disability >3 days) 1
- Consider admission for IV dihydroergotamine (DHE) protocol: DHE 0.5-1 mg IV every 8 hours for 3 days, with metoclopramide 10 mg IV given 30 minutes before each DHE dose 1
- Strictly avoid opioids, which worsen outcomes and lead to medication overuse headache 1
Critical Pitfall to Avoid
Do not continue escalating acute migraine medications without investigating the underlying cause. The pattern of positional worsening, complete treatment refractoriness, and progressive course over months demands investigation for secondary causes before assuming this is simply difficult-to-treat migraine 1. Continuing to treat empirically as migraine delays diagnosis of potentially treatable conditions like SIH, and risks developing medication overuse headache if acute treatments are used >2 days/week 1.