Immediate Diagnostic Workup for Suspected Spontaneous Intracranial Hypotension
This patient requires urgent MRI brain with IV contrast AND MRI complete spine to diagnose spontaneous intracranial hypotension (SIH), as headache worsening when lying down or bending over is the hallmark orthostatic pattern of CSF leak. 1, 2
Critical Diagnostic Criteria
The orthostatic headache pattern must meet specific timing requirements to confirm SIH 2:
- Absent or only mild headache upon waking or after prolonged lying flat
- Onset within 2 hours of becoming upright
- Improvement by >50% within 2 hours of lying flat
- Consistent timing pattern across episodes
Associated Symptoms That Increase Diagnostic Certainty
Actively assess for these accompanying features 1, 2:
- Nausea and vomiting (present in 28% of SIH cases)
- Neck pain or stiffness
- Tinnitus or hearing changes (11% of cases; also predicts recurrence risk)
- Photophobia
- Visual disturbances (3% of cases)
- Vertigo (8% of cases)
Mandatory Differential Diagnoses to Exclude First
Before confirming SIH, you must rule out 2:
Postural Orthostatic Tachycardia Syndrome (PoTS):
- Perform formal standing test documenting heart rate
- Diagnostic if heart rate increases >30 beats per minute on standing
- Note: Negative standing test does not exclude PoTS if clinical suspicion remains high
Orthostatic Hypotension:
- Document blood pressure drop >20 mmHg systolic and/or >10 mmHg diastolic on standing
Cervicogenic Headache:
- Headache provoked by cervical movement rather than posture
- Reduced cervical range of motion
- Associated myofascial tenderness
Imaging Protocol - Must Be Done Immediately
Order both studies simultaneously 1, 2:
Brain MRI with IV Contrast
Look for these confirmatory findings 1, 2:
- Diffuse pachymeningeal (dural) enhancement (97% of cases)
- Venous sinus engorgement
- Midbrain descent
- Pituitary enlargement (convex superior surface)
- Ventricular collapse (predicts higher recurrence risk)
- Posterior fossa crowding (predicts higher recurrence risk)
Complete Spine MRI
Identifies the leak source 1:
- Epidural fluid collections (direct evidence of CSF leak)
- CSF-venous fistula
- Dilated epidural venous plexus (indirect sign)
- Subdural hygromas (indirect sign)
- Dural enhancement (indirect sign)
Critical Pitfall to Avoid
Do NOT rely on CSF pressure measurement - CSF pressure can be normal in patients with SIH, and absence of low CSF pressure should not exclude this diagnosis 1. The diagnosis is clinical and radiographic, not manometric.
Treatment Pathway After Diagnosis Confirmation
Epidural blood patch (EBP) is first-line treatment and should be performed as soon as possible after diagnosis 1, 2:
Post-EBP Management Protocol 1:
- Monitor in recovery area with vital signs (heart rate, blood pressure, pulse oximetry) and spinal observations
- 2-24 hours bed rest and observation period
- Supine or Trendelenburg position for non-targeted patches
- Supine with head elevated as comfortable for targeted patches
- Thromboprophylaxis during immobilization per institutional VTE policy
Post-Discharge Instructions 1:
- Lie flat as much as possible for 1-3 days
- Minimize for 4-6 weeks: bending, straining, stretching, twisting, closed-mouth coughing, sneezing, heavy lifting, strenuous exercise, constipation
Emergency Return Precautions 1
Instruct patient to seek urgent medical attention for:
- New-onset severe back or leg pain
- Lower limb motor weakness or sensory disturbance
- Urinary or fecal incontinence
- Urinary retention
- Perineal sensory disturbance
- Nausea and vomiting
- Fever
Recognizing Rebound Headache vs. Treatment Failure
Rebound headaches occur in 25% of patients 1-2 days post-EBP 1:
Characteristic Features of Rebound Headache 1:
- Reversal of orthostatic pattern: relief upright, worse lying down
- Headache location change: from occipital to frontal/periorbital/retroorbital
- Associated symptoms: nausea, emesis, blurry vision
- Self-limited: usually resolves without intervention
Management of Rebound Headache 1:
- Conservative management first - do NOT repeat imaging or EBP
- Acetazolamide to lower CSF production
- CSF drainage via lumbar puncture or lumbar drain only for severe refractory cases
High-Risk Features for Recurrence
Identify patients at higher risk requiring closer follow-up 2:
- Ventricular collapse on brain MRI
- Posterior fossa crowding
- Longer delay between symptom onset and treatment
- Persistent tinnitus
Life-Threatening Complication to Monitor
Cerebral venous thrombosis (CVT) complicates SIH in rare cases 1:
- Occurs in 86% of documented SIH-CVT cases, often initially unrecognized
- Order CT or MR venography for any sudden change in headache pattern or new neurological findings
- EBP should be prioritized as initial treatment even with CVT present
- Consider anticoagulation balancing risks and benefits
Why Previous Treatment Failed
The patient received metoclopramide and ketorolac, which are migraine-specific treatments that do not address CSF leak 1, 3. While metoclopramide is effective for migraine (though with slower onset than other agents) 4, 5, and ketorolac shows good efficacy for acute migraine 3, 6, 7, neither treats the underlying pathophysiology of SIH. The orthostatic pattern (worse lying down/bending) distinguishes this from migraine, where headache is typically provoked by movement rather than posture 2.