The Paroxysmal Hemicranias

CHAPTER 12 THE PAROXYSMAL HEMICRANIAS



Lawrence C. Newman, MD, Richard B. Lipton, MD




1. What are the paroxysmal hemicranias?


The paroxysmal hemicranias are a group of rare, benign headache disorders that resemble cluster headache in most ways but do not respond to anticluster medications. The headaches are characterized by severe, excruciating, throbbing, boring, or pulsatile pain affecting the orbital, supraorbital, and temporal regions. These pains are associated with at least one of the following signs or symptoms ipsilateral to the painful side:



image Conjunctival injection


image Lacrimation


image Nasal congestion


image Rhinorrhea


image Ptosis


image Eyelid edema


Attacks occur from 1 to 40 times daily, usually exceeding eight attacks in a 24-hour period. Duration is typically 2 to 30 minutes, but on rare occasions attacks last as long as 2 hours. Headaches may occur any time during the day or night, and there is often a predisposition to nocturnal attacks, in which the patient is awakened from a sound sleep by an incapacitating headache.



2. Are there different clinical variations of the paroxysmal hemicranias?


Yes. Although there has been controversy regarding the nomenclature of the paroxysmal hemicranias, there appear to be three related forms:



image Chronic paroxysmal hemicrania (CPH), in which multiple headaches occur daily for years on end without remission or with remission periods of less than 1 month


image Episodic paroxysmal hemicrania (EPH), in which there are discrete phases characterized by frequent daily attacks separated by long-term, pain-free remissions


image Pre-CPH, in which an initially episodic form of these headaches ultimately evolves into the chronic unremitting form


Some authors prefer alternative nomenclature. At present, only CPH and EPH are recognized in the International Headache Society’s diagnostic system as outlined in the Second Edition of the International Classification of Headache Disorders (ICHD II).



3. What distinguishes the paroxysmal hemicranias from cluster headache?


The major distinguishing features of the paroxysmal hemicranias and cluster headache lie in the frequency of the attack, the duration of the attack, and the response to treatment. In addition, the paroxysmal hemicranias do not show the striking preponderance among males that characterizes cluster headache. In cluster headache, attacks are less frequent but of longer duration—one or two a day with a typical duration of 30 minutes to 2 hours. Attacks in the paroxysmal hemicranias exceed five a day and last 2 to 25 minutes each.



4. Do the paroxysmal hemicranias differ pathophysiologically from cluster headache?

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Jun 14, 2016 | Posted by in PAIN MEDICINE | Comments Off on The Paroxysmal Hemicranias

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