Hemicrania continua
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting, but rare cases of remission have been documented. Hemicrania continua is considered a primary headache disorder, meaning that it's not caused by another condition.
Diagnostic criteria
International Headache Society's International Classification of Headache Disorders 2nd Edition,[1] establishes the following diagnostic criteria for hemicrania continua:
- Headache for more than 3 months fulfilling other 3 criteria:
- All of the following characteristics:
- Unilateral pain without side-shift
- Daily and continuous, without pain-free periods
- Moderate intensity, but with exacerbations of severe pain
- At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side of pain:
- Conjunctival injection and/or lacrimation
- Nasal congestion and/or rhinorrhea
- Ptosis and/or miosis
- Complete response to therapeutic doses of indomethacin
A variant on hemicrania continua has also been described, in which the attacks may shift sides, although meeting the above criteria in all other respects.[1][1][1][1]
Epidemiology
Hemicrania continua was first described in 1981,[1] at that time around 130 cases were described in the literature.[1] However, rising awareness of the condition has led to increasingly frequent diagnosis in headache clinics, and it seems that it is not as rare as these figures would imply. The condition occurs more often in women than men and tends to first present in adulthood, although it has also been reported in children as young as 5 years old.[1]
Cause and diagnosis
The cause of hemicrania continua is unknown. There is no definitive diagnostic test for hemicrania continua. Diagnostic tests such as imaging studies may be ordered to rule out other causes for the headache. When the symptoms of hemicrania continua are present, it's considered "diagnostic" if they respond completely to indomethacin.
The factor that allows hemicrania continua and its exacerbations to be differentiated from migraine and cluster headache is that hemicrania continua is completely responsive to idomethacin. Triptans and other abortive medications do not affect hemicrania continua.
Symptoms
In addition to persistent daily headache of HC, which is usually mild to moderate, HC can present other symptoms.[1] These additional symptoms of HC can be divided into three main categories:
- Autonomic symptoms:
- conjunctival injection
- tearing
- rhinorrhea
- nasal stuffiness
- eyelid edema
- forehead sweating
- Stabbing headaches:
- Short, "jabbing" headaches superimposed over the persistent daily headache.
- Usually lasting less than one minute.
- Migrainous features:
- throbbing pain
- nausea and/or vomiting
- phonophobia
- photophobia
Differential Diagnosis of Hemicrania continua
| Cardiovascular | No underlying causes |
| Chemical / poisoning | No underlying causes |
| Dermatologic | No underlying causes |
| Drug Side Effect | No underlying causes |
| Ear Nose Throat | No underlying causes |
| Endocrine | No underlying causes |
| Environmental | No underlying causes |
| Gastroenterologic | No underlying causes |
| Genetic | No underlying causes |
| Hematologic | No underlying causes |
| Iatrogenic | No underlying causes |
| Infectious Disease | No underlying causes |
| Musculoskeletal / Ortho | No underlying causes |
| Neurologic | No underlying causes |
| Nutritional / Metabolic | No underlying causes |
| Oncologic | No underlying causes |
| Opthalmologic | No underlying causes |
| Overdose / Toxicity | No underlying causes |
| Psychiatric | No underlying causes |
| Pulmonary | No underlying causes |
| Renal / Electrolyte | No underlying causes |
| Rheum / Immune / Allergy | No underlying causes |
| Trauma | No underlying causes |
| Miscellaneous | No underlying causes |
Treatment
Hemicrania continua generally responds only to indomethacin 25-300 mg daily, which must be continued long term. Unfortunately, gastrointestinal side effects are a common problem with indomethacin, which may require additional acid-suppression therapy to control.[1]
In patients who are unable to tolerate indomethacin, the use of celecoxib 400-800 mg per day (Celebrex) and rofecoxib 50 mg per day (Vioxx - no longer available) have both been shown to be effective and are likely to be associated with fewer GI side effects.[1] There have also been reports of two patients who were successfully managed with topiramate 100-200 mg per day (Topamax) although side effects with this treatment can also prove problematic.[1][1]
References
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
