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Chronic Paroxysmal Hemicrania (CPH)

Trigeminal Autonomic Cephalalgia with Absolute Indomethacin Response

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Chronic Paroxysmal Hemicrania (CPH)?

Chronic paroxysmal hemicrania (CPH) is a rare primary headache disorder classified as a trigeminal autonomic cephalalgia (TAC), occurring 0.07% in headache clinic populations.

Diagnostic criteria (ICHD-3): at least 20 attacks of severe unilateral orbital, supraorbital or temporal pain lasting 2–30 minutes, with frequency >5 per day for more than half the time, accompanied by ipsilateral cranial autonomic features.

Defining feature is absolute response to therapeutic doses of indomethacin (typically 75–225 mg/day), which both confirms diagnosis and provides effective treatment.

Episodic and chronic forms exist; chronic form (CPH) features attacks for more than one year without remission or with remissions <3 months.

Symptoms

Severe unilateral pain in orbital, supraorbital, or temporal region (rarely changes side between attacks within an individual)
Brief attack duration: 2–30 minutes (averaging 10–20 minutes), more frequent than cluster headache
High frequency: typically 5–40 attacks daily during active phase
Cranial autonomic features ipsilateral to pain: conjunctival injection, lacrimation, nasal congestion or rhinorrhea, eyelid edema, forehead/facial sweating, ptosis, miosis
Restlessness or agitation during attacks (less commonly than cluster headache)
Triggers: neck movements, alcohol, stress in some patients; many attacks occur unprovoked
Female predominance (female-to-male ratio approximately 2:1 to 3:1)

Risk Factors

Adults aged 20–40 years at onset (some pediatric cases reported)
Female predominance (in contrast to cluster headache male predominance)
Less consistent association with smoking and alcohol than cluster headache
No clear genetic or family predisposition established
Possible secondary CPH from pituitary tumors, posterior cranial fossa lesions, or vascular abnormalities — warrants brain MRI in atypical presentations
No clear circadian or seasonal pattern in many patients (unlike cluster headache)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Frequent severe unilateral headaches with autonomic features, especially with response to indomethacin
  • Suspected cluster headache that occurs more than 8 times per day or with shorter duration than typical cluster
  • New-onset paroxysmal hemicrania, especially with atypical features (warranting MRI to exclude secondary cause)
  • Known CPH with breakthrough symptoms despite indomethacin therapy
  • Adverse effects from chronic indomethacin therapy (gastrointestinal, renal, cardiovascular)

Treatment Methods

01
Indomethacin trial: starting dose 25 mg three times daily, escalating to 75 mg three times daily over 7–14 days; maintenance dose typically 75–150 mg/day, occasionally up to 225 mg/day
02
Indomethacin 'indotest' for diagnostic confirmation: complete pain relief within 1–7 days at adequate dose; partial response should prompt reassessment
03
Gastric protection during indomethacin therapy: proton pump inhibitor (omeprazole 20–40 mg daily) and monitoring of renal and cardiovascular status
04
Dose adjustment to lowest effective maintenance dose; periodic attempts at dose reduction or discontinuation in patients with prolonged remission
05
Alternative treatments for indomethacin-intolerant patients (less effective): COX-2 inhibitors (celecoxib, etoricoxib), gabapentin, topiramate, verapamil, melatonin
06
Refractory cases: occipital nerve blocks, sphenopalatine ganglion blocks, occipital nerve stimulation in selected centers; greater occipital nerve injection
07
Brain MRI with pituitary protocol to exclude secondary causes, especially if atypical features (older onset, side switching, lack of response, neurologic deficits)
08
Patient education on chronic medication therapy, importance of consistent dosing, monitoring for side effects, and avoiding triggers when identified

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.