Primary Cough, Exertional, and Sex Headaches: Differential Diagnosis and Management
Critical First Step: Rule Out Secondary Causes
Every patient with cough headache requires cranio-cervical MRI, as approximately 50% are secondary to structural lesions, most commonly Chiari type I malformation 1, 2. For exercise and sexual headaches, obtain CT followed by brain MRI with MRA or angioCT to exclude space-occupying lesions or subarachnoid hemorrhage 1.
Red Flags Requiring Urgent Neuroimaging
- Cough headache: Age <50 years, occipital pain lasting >1 minute, associated neurological symptoms/signs, no response to indomethacin 1
- Exercise/sexual headache: Female sex, single episode, age >40 years, duration >24 hours, associated symptoms/signs 1
- All types: Abrupt onset of severe headache, marked change in pattern, awakening from sleep, focal neurologic signs 3
Distinguishing Primary from Secondary Forms
Primary Cough Headache Characteristics
- Age: Typically >50 years 1
- Location: Non-occipital predominance 1
- Duration: Seconds only 1
- Associated features: None 1
- Indomethacin response: Positive 1
Secondary Cough Headache Characteristics
- Age: Younger patients (average 40 years) 2
- Location: Posterior/occipital 1, 2
- Duration: >1 minute, condition persists longer (5 years vs 11 months) 2
- Associated features: Posterior fossa symptoms/signs 2
- Indomethacin response: Negative 1, 2
- Etiology: Chiari type I malformation most common; also consider ACE inhibitor-induced (7 cases in one series) 2
Exercise and Sexual Headaches
These are clinical variants of the same entity 2. Primary forms share:
- Age of onset: Average 40 years 2
- Character: Bilateral, pulsating 2
- Secondary causes: Rare, but subarachnoid hemorrhage is most common when present 2
Management Algorithm
For Primary Cough Headache
First-line treatment: Indomethacin 1, 4, 5, 6
- Provides significant relief in primary cases 4
- Lack of response suggests secondary etiology 1, 2
- Consider discontinuing ACE inhibitors if present, as they can cause cough headache 2
For Primary Exertional and Sexual Headaches
First-line treatment: Indomethacin or beta-blockers 4, 2, 5, 6
- Both headache types respond to beta-blockers 2
- Indomethacin is reported effective for both syndromes 5, 6
- These medications can be used prophylactically before anticipated triggers 4
For Secondary Forms
Treatment depends on underlying etiology 2:
- Chiari malformation: Posterior fossa decompression surgery; dynamic cerebrospinal fluid MRI shows impaired CSF circulation at foramen magnum, with preoperative plateau waves that resolve after surgical reconstruction 2
- Subarachnoid hemorrhage: Requires neurosurgical intervention 1, 4
- Space-occupying lesions: Treat underlying pathology 1
Clinical Pearls and Common Pitfalls
Key Diagnostic Distinctions
Cough headache is fundamentally different from exercise/sexual headaches 2:
- Cough headache has higher rate of secondary causes (58.8% vs rare) 2
- Cough headache requires different imaging protocol (cranio-cervical MRI with dynamic CSF studies if secondary) 2
- Exercise and sexual headaches are variants of the same condition, not separate entities 2
Critical Mistakes to Avoid
- Never skip neuroimaging in cough headache patients—nearly half have structural lesions 1, 2
- Don't assume single episodes of exercise/sexual headache are benign—these warrant urgent evaluation for subarachnoid hemorrhage 1
- Don't rely on headache characteristics alone—systematic imaging is essential 1
- Consider medication history—ACE inhibitors can cause cough headache 2