Hemicrania continua: Difference between revisions

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{{Infobox medical condition (new)
 
{{Infobox medical condition
| name            = Hemicrania continua
| name            = Hemicrania continua
| synonyms        =  
| synonyms        =  
| image          =
| field          = [[Neurology]]
| caption        =
| symptoms        = [[Unilateral headache]], [[autonomic symptoms]]
| pronounce      =
| field          =  
| symptoms        =  
| complications  =
| onset          =  
| onset          =  
| duration        =  
| duration        =  
| types          =  
| types          =  
| causes          =  
| causes          = Unknown
| risks          =  
| risks          =  
| diagnosis      =  
| diagnosis      = Clinical evaluation, response to [[indomethacin]]
| differential    =  
| differential    = [[Migraine]], [[cluster headache]], [[tension-type headache]]
| prevention      =
| treatment      = [[Indomethacin]]
| treatment      =  
| medication      =  
| medication      =  
| prognosis      =
| frequency      =  
| frequency      =  
| deaths          =  
| deaths          =  
}}
}}
'''Hemicrania continua''' ('''HC''') is a persistent [[unilateral (disambiguation)|unilateral]] [[headache]] that responds to [[indomethacin]]. It is usually unremitting, but rare cases of remission have been documented.<ref>Mark Thompson, [http://www.hemicraniacontinua.com/ "Hemicrania Continua"]</ref> Hemicrania continua is considered a [[primary headache disorder]], meaning that it is not caused by another condition.
{{Short description|A primary headache disorder characterized by continuous, unilateral pain}}
 
{{Use dmy dates|date=October 2023}}
== Symptoms ==
'''Hemicrania continua''' is a rare [[primary headache disorder]] characterized by continuous, unilateral [[headache]] that varies in intensity but does not resolve completely. It is classified under the group of [[trigeminal autonomic cephalalgias]] and is known for its responsiveness to [[indomethacin]], a nonsteroidal anti-inflammatory drug.
In addition to persistent daily headache of HC, which is usually mild to moderate (and frequently severe), HC can present other symptoms.<ref name="isbn1-55009-265-0">{{cite book |vauthors=Goadsby P, Silberstein S, Dodick D |title=Chronic Daily Headache for clinicians |publisher=B C Decker Inc |location= |year=205 |isbn=978-1-55009-265-3 |oclc= |doi= |page=220}}</ref> These additional symptoms of HC can be divided into three main categories:
==Clinical Features==
 
Hemicrania continua is defined by its persistent nature, with patients experiencing a continuous headache on one side of the head. The pain can fluctuate in intensity, with periods of exacerbation that may include additional symptoms such as:
# Autonomic symptoms:
* [[Nausea]]
#* conjunctival injection
* [[Photophobia]] (sensitivity to light)
#* tearing
* [[Phonophobia]] (sensitivity to sound)
#* rhinorrhea
* [[Autonomic symptoms]] such as tearing, nasal congestion, or ptosis on the affected side
#* nasal stuffiness
The condition is unique in its absolute response to indomethacin, which is a key diagnostic criterion.
#* eyelid edema
==Diagnosis==
#* forehead sweating
The diagnosis of hemicrania continua is primarily clinical, based on the characteristic symptoms and the patient's response to indomethacin. The International Classification of Headache Disorders (ICHD) provides criteria that include:
# Stabbing headaches:
* Unilateral headache for more than three months
#* Short, "jabbing" headaches superimposed over the persistent daily headache.
* Complete resolution of headache with indomethacin
#* Usually lasting less than one minute.
* Presence of at least one autonomic symptom during exacerbations
# Migrainous features:
==Pathophysiology==
#* throbbing pain
The exact pathophysiology of hemicrania continua is not well understood. It is believed to involve dysfunction in the [[trigeminal nerve]] pathways and possibly the [[hypothalamus]], similar to other trigeminal autonomic cephalalgias. The role of indomethacin suggests an inflammatory component, although the precise mechanisms remain unclear.
#* nausea and/or vomiting
==Treatment==
#* [[phonophobia]]
The hallmark of hemicrania continua treatment is the use of indomethacin, which typically provides complete relief of symptoms. The dosage of indomethacin may vary, and it is often started at a low dose and gradually increased until the headache is controlled. Long-term use of indomethacin can have side effects, so patients are monitored closely.
#* [[photophobia]]
In cases where indomethacin is not tolerated, alternative treatments may include other nonsteroidal anti-inflammatory drugs, [[gabapentin]], or [[topiramate]], although these are generally less effective.
== Cause ==
==Prognosis==
The cause of hemicrania continua is unknown.{{cn|date=November 2019}}
With appropriate treatment, the prognosis for individuals with hemicrania continua is generally good. Most patients experience significant relief from symptoms with indomethacin. However, the condition is chronic and may require long-term management.
 
==Related Pages==
== Diagnosis==
* [[Trigeminal autonomic cephalalgias]]
The following diagnostic criteria are given for hemicrania continua:<ref name=ICHD2/>
* [[Indomethacin]]
 
* [[Chronic daily headache]]
# Headache for more than 3 months fulfilling other 3 criteria:
* [[Cluster headache]]
# 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 (eyelid)|Ptosis]] and/or [[miosis]]
# Complete response to therapeutic doses of indomethacin, although cases of hemicrania continua that do not resolve with indomethacin treatment have been documented.<ref>{{Cite journal | pmid = 19096758| pmc = 3451756| year = 2009| author1 = Prakash| first1 = S| title = Hemicrania continua unresponsive or partially responsive to indomethacin: Does it exist? A diagnostic and therapeutic dilemma| journal = The Journal of Headache and Pain| volume = 10| issue = 1| pages = 59–63| last2 = Shah| first2 = N. D.| last3 = Bhanvadia| first3 = R. J.| doi = 10.1007/s10194-008-0088-9}}</ref>
 
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.<ref>{{cite journal |vauthors=Newman LC, Lipton RB, Russell M, Solomon S |title=Hemicrania continua: attacks may alternate sides |journal=Headache |volume=32 |issue=5 |pages=237–8 |year=1992 |pmid=1628961 |doi=10.1111/j.1526-4610.1992.hed3205237.x}}</ref><ref>{{cite journal |vauthors=Marano E, Giampiero V, Gennaro DR, di Stasio E, Bonusa S, Sorge F |title="Hemicrania continua": a possible case with alternating sides |journal=Cephalalgia |volume=14 |issue=4 |pages=307–8 |year=1994 |pmid=7954766 |doi=10.1046/j.1468-2982.1994.1404305-4.x}}</ref><ref>{{cite journal |vauthors=Newman LC, Spears RC, Lay CL |title=Hemicrania continua: a third case in which attacks alternate sides |journal=Headache |volume=44 |issue=8 |pages=821–3 |year=2004 |pmid=15330832 |doi=10.1111/j.1526-4610.2004.04153.x}}</ref><ref name="pmid16472344">{{cite journal |vauthors=Matharu MS, Bradbury P, Swash M |title=Hemicrania continua: side alternation and response to topiramate |journal=Cephalalgia |volume=26 |issue=3 |pages=341–4 |year=2006 |pmid=16472344 |doi=10.1111/j.1468-2982.2005.01034.x}}</ref>
 
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 efficacy of indomethacin may not be long term for all patients, as can eventually become ineffective.
===Differential diagnosis===
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 indomethacin. [[Triptans]] and other abortive medications do not affect hemicrania continua.
=== Classification ===
The [[International Headache Society]]'s International Classification of Headache Disorders classifies hemicrania continua as a primary headache disorder.<ref name=ICHD2>{{cite web |url= http://www.ihs-headache.org/upload/ct_clas/ihc_II_main_no_print.pdf |title=The International Classification of Headache Disorders 2nd Edition (ICHD-2) |work=the Headache Classification Subcommittee of the International Headache Society |accessdate=2012-09-22}}</ref>
 
== Treatment ==
Hemicrania continua generally responds only to [[indomethacin]] 25–300&nbsp;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.<ref>{{cite journal |vauthors=Pareja JA, Caminero AB, Franco E, Casado JL, Pascual J, Sánchez del Río M |title=Dose, efficacy and tolerability of long-term indomethacin treatment of chronic paroxysmal hemicrania and hemicrania continua |journal=Cephalalgia: An International Journal of Headache |volume=21 |issue=9 |pages=906–10 |year=2001 |pmid=11903285 |doi=10.1046/j.1468-2982.2001.00287.x}}</ref>
 
In patients who are unable to tolerate indomethacin, the use of [[celecoxib]] 400–800&nbsp;mg per day (Celebrex) and [[rofecoxib]] 50&nbsp;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.<ref>{{cite journal |vauthors=Peres MF, Silberstein SD |title=Hemicrania continua responds to cyclooxygenase-2 inhibitors |journal=Headache |volume=42 |issue=6 |pages=530–1 |year=2002 |pmid=12167145 |doi=10.1046/j.1526-4610.2002.02131.x}}</ref> There have also been reports of two patients who were successfully managed with [[topiramate]] 100–200&nbsp;mg per day (Topamax) although side effects with this treatment can also prove problematic.<ref name="pmid16472344"/><ref name="pmid17371364">{{cite journal |vauthors=Brighina F, Palermo A, Cosentino G, Fierro B |title=Prophylaxis of hemicrania continua: two new cases effectively treated with topiramate |journal=Headache |volume=47 |issue=3 |pages=441–3 |year=2007 |pmid=17371364 |doi=10.1111/j.1526-4610.2007.00733.x}}</ref>
 
[[Greater occipital nerve]] (GON) block comprising 40&nbsp;mg Depomedrone and 10 mls of 1% Lignocaine injected into the affected nerve is effective, up to a period of approximately three months.  Changing the 'cocktail' to include (for example) 10 mls of .5% Marcaine and changing to 2% Lignocaine, whilst in theory should increase the longevity, renders the injection completely ineffective. See 4.2 Posology and method of administration (flocculation).<ref>[https://www.drugs.com/uk/pdf/leaflet/261720.pdf DEPO-MEDRONE WITH LIDOCAINE INJECTION]</ref><ref>{{cite journal|title=Expert Consensus Recommendations for the Performance of Peripheral Nerve Blocks for Headaches – A Narrative Review|first1=Andrew|last1=Blumenfeld|first2=Avi|last2=Ashkenazi|first3=Uri|last3=Napchan|first4=Steven D.|last4=Bender|first5=Brad C.|last5=Klein|first6=Randall|last6=Berliner|first7=Jessica|last7=Ailani|first8=Jack|last8=Schim|first9=Deborah I.|last9=Friedman|first10=Larry|last10=Charleston|first11=William B.|last11=Young|first12=Carrie E.|last12=Robertson|first13=David W.|last13=Dodick|first14=Stephen D.|last14=Silberstein|first15=Matthew S.|last15=Robbins|date=1 March 2013|journal=Headache: The Journal of Head and Face Pain|volume=53|issue=3|pages=437–446|doi=10.1111/head.12053|pmid = 23406160}}</ref>
 
[[Occipital nerve stimulation]] may be highly effective when other treatments fail to relieve the intractable pain.<ref>{{cite web|url=http://www.neurologyreviews.com/the-publication/past-issue-sngle-view/occipital-nerve-stimulation-for-headache/7ef763329e4d7dd532baab0ce8087da9.html|title=- MDedge - Neurology Reviews|website=www.neurologyreviews.com}}</ref>
 
== Epidemiology ==
Hemicrania was mentioned in 1881 in The Therapeutic Gazette Vol. 2, by G.S.Davis,<ref>{{cite book|last=Davis|first=G.S.|title=The Therapeutic Gazette, Volume 2|year=1881|page=54}}</ref> and the incident has been cited in King's American Dispensatory (1898 and later editions)<ref>{{cite book|last=King; Felter; Lloyd|first=John; Harvey Wickes; John Uri|title=King's American Dispensatory|year=1898|publisher=Ohio Valley Co|location=Cincinnati|page=1511}}</ref> in the description of the strong analgesic Jamaican Dogwood, a relatively low dose of which reportedly produced convulsions and prolonged respiratory depression over six hours in an elderly woman with this condition.
 
In newer times, Hemicrania continua was described in 1981;<ref name="pmid7305699">{{cite journal |vauthors=Medina JL, Diamond S |title=Cluster headache variant. Spectrum of a new headache syndrome |journal=Arch. Neurol. |volume=38 |issue=11 |pages=705–9 |year=1981 |pmid=7305699 |doi=10.1001/archneur.1981.00510110065010}}</ref> at that time around 130 cases were described in the literature.<ref name="pmid7305699"/>  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 present first in adulthood, although it has also been reported in children as young as 5 years old.<ref>{{cite journal  |vauthors=Peres MF, Silberstein SD, Nahmias S, etal |title=Hemicrania continua is not that rare |journal=Neurology |volume=57 |issue=6 |pages=948–51 |year=2001 |pmid=11577748 |doi=10.1212/wnl.57.6.948}}</ref>
 
== References ==
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  footnotes using the <ref>, </ref> and  <reference /> tags
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{{Reflist}}
== External links ==
{{Medical resources
|  DiseasesDB    = 
|  ICD10          = {{ICD10|G44.8}}
|  ICD9          = {{ICD9|339.41}}
|  ICDO          = 
|  OMIM          = 
|  MedlinePlus    = 
|  eMedicineSubj  = 
|  eMedicineTopic = 
|  MeshID        =
}}
{{Headache}}
 
[[Category:Headaches]]
[[Category:Headaches]]
[[Category:Neurological disorders]]

Latest revision as of 23:58, 3 April 2025


Hemicrania continua
Synonyms
Pronounce N/A
Specialty N/A
Symptoms Unilateral headache, autonomic symptoms
Complications N/A
Onset
Duration
Types
Causes Unknown
Risks
Diagnosis Clinical evaluation, response to indomethacin
Differential diagnosis Migraine, cluster headache, tension-type headache
Prevention N/A
Treatment Indomethacin
Medication
Prognosis N/A
Frequency
Deaths


A primary headache disorder characterized by continuous, unilateral pain


Hemicrania continua is a rare primary headache disorder characterized by continuous, unilateral headache that varies in intensity but does not resolve completely. It is classified under the group of trigeminal autonomic cephalalgias and is known for its responsiveness to indomethacin, a nonsteroidal anti-inflammatory drug.

Clinical Features[edit]

Hemicrania continua is defined by its persistent nature, with patients experiencing a continuous headache on one side of the head. The pain can fluctuate in intensity, with periods of exacerbation that may include additional symptoms such as:

The condition is unique in its absolute response to indomethacin, which is a key diagnostic criterion.

Diagnosis[edit]

The diagnosis of hemicrania continua is primarily clinical, based on the characteristic symptoms and the patient's response to indomethacin. The International Classification of Headache Disorders (ICHD) provides criteria that include:

  • Unilateral headache for more than three months
  • Complete resolution of headache with indomethacin
  • Presence of at least one autonomic symptom during exacerbations

Pathophysiology[edit]

The exact pathophysiology of hemicrania continua is not well understood. It is believed to involve dysfunction in the trigeminal nerve pathways and possibly the hypothalamus, similar to other trigeminal autonomic cephalalgias. The role of indomethacin suggests an inflammatory component, although the precise mechanisms remain unclear.

Treatment[edit]

The hallmark of hemicrania continua treatment is the use of indomethacin, which typically provides complete relief of symptoms. The dosage of indomethacin may vary, and it is often started at a low dose and gradually increased until the headache is controlled. Long-term use of indomethacin can have side effects, so patients are monitored closely. In cases where indomethacin is not tolerated, alternative treatments may include other nonsteroidal anti-inflammatory drugs, gabapentin, or topiramate, although these are generally less effective.

Prognosis[edit]

With appropriate treatment, the prognosis for individuals with hemicrania continua is generally good. Most patients experience significant relief from symptoms with indomethacin. However, the condition is chronic and may require long-term management.

Related Pages[edit]