Chronic Paroxysmal Hemicrania

Chronic paroxysmal hemicrania (CPH) is a primary headache syndrome characterized by recurrent unilateral episodes of headache associated with cranial autonomic symptoms. Headaches are sharp and stabbing in nature and occur greater than 5 times per day, up to 40 times per day in some cases. Associated cranial autonomic features include ipsilateral lacrimation, conjunctival injection, nasal congestion, rhinorrhea, facial flushing, eyelid edema, miosis or mydriasis, diaphoresis or aural fullness. The mean duration attack is 26 minutes, with a range of 2 minutes to nearly two hours. Attacks occur both daytime and nighttime in most cases. CPH occurs on the same side in greater than 95% of patients. CPH differs from episodic paroxysmal hemicrania in that there is no remission or remission that lasts less than three months. Paroxysmal hemicrania responds well to indomethacin, with complete resolution in most patients. CPH is in a family of headache syndromes called the trigeminal autonomic cephalgias (TACs). TACs are characterized by unilateral trigeminal nerve distribution pain that occurs in tandem with ipsilateral cranial nerve autonomic symptoms. The TACs include paroxysmal hemicrania, cluster headache, hemicrania continua, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The International Classification of Headache Disorders 3rd Edition defines chronic paroxysmal hemicrania as follows: A. At least 20 attacks fulfilling criteria B to E without a remission period, or with remissions lasting less than 3 months, for at least one year: B. Severe unilateral orbital, supraorbital and/or temporal pain lasting 2 to 30 minutes C. Either or both of the following: 1. At least one of the following symptoms or signs, ipsilateral to the headache: Conjunctival injection and/or lacrimation Nasal congestion and/or rhinorrhea Eyelid edema Forehead and facial sweating Miosis and/or ptosis 2. A sense of restlessness or agitation D. Occurring with a frequency greater than 5 per day E. Prevented absolutely by therapeutic doses of indomethacin F. Not better accounted for by another ICHD-3 (International Classification of Headache 3rd edition) disorder