Diagnostic Considerations
Differential diagnosis should include other headaches with major autonomic features (eg, SUNCT syndrome) and persistent headaches with milder autonomic features (eg, hemicrania continua [HC]).
HC is a rare disorder characterized by continuous, unilateral, indomethacin-responsive hemicrania of moderate severity. These headaches last from minutes to several days and sometimes are associated with autonomic features. A noncontinuous form also has been described.
Along with HC, several other paroxysmal, indomethacin-responsive headache syndromes have been described, including the following:
- Jabs-and-jolts syndrome
- Icepick headache
- Hypnic headache syndrome
- Benign cough headache syndrome
- Benign exertional headache
- Coital headache
- Thunderclap headache
Cervicogenic headache, facial neuralgia, and other rare disorders, such as red ear syndrome, also are considered in the differential diagnosis.
Simultaneous occurrences of CPH and other disorders, including CH, trigeminal neuralgia (CPH-tic syndrome), and migraine, also have been reported. The crucial factor in the differential diagnosis is the absolute response to indomethacin.
Cluster headache
The trigeminal-autonomic cephalgias include cluster headache (CH) and paroxysmal hemicranias in which head pain and cranial autonomic symptoms are prominent. CH is the most important differential diagnosis.
CH has male preponderance, unlike CPH, which is more common in females. In CPH, the frequency of attacks is higher, usually more than 15 in 24 hours, whereas CH has an attack frequency of 1-4 (maximum 8) in 24 hours. The duration of headaches is shorter in CPH (2-25 min) than in CH (15-60 min). Finally, CPH can be distinguished by its response to indomethacin therapy, which is not seen in CH.
SUNCT syndrome
SUNCT syndrome is a rare, distinctive condition with a male preponderance; it is characterized by less severe pain but also by marked autonomic activation during attacks. The pain, usually in the frontal and periocular area, is intractable to medications, including indomethacin.
LASH
A unique, stereotypical, episodic headache disorder marked by long-lasting autonomic symptoms with associated hemicrania (LASH) has been reported. The autonomic symptoms clearly overshadow the headache as the major component of the syndrome. Both types of symptoms respond to indomethacin.
Other differentials
Other disorders that may mimic CPH include the following[12] :
- Infarcts
- Arteriovenous malformation
- Brain tumor - Eg, frontal lobe tumor, meningioma of the roof of the cavernous sinus, pathologic process in the region of the cavernous sinus, gangliocytoma near the sella turcica, and parasellar pituitary microadenoma
- Maxillary cyst
- Collagen vascular disorders
- Cervical radiculopathy
- Disk herniation
- Carotidynia
Differential Diagnoses
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