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Guideline Summary
Guideline Title
Treatment of miscellaneous idiopathic headache disorders (group 4 of the IHS classification) — report of an EFNS task force.
Bibliographic Source(s)
Evers S, Goadsby P, Jensen R, May A, Pascual J, Sixt G, EFNS task force. Treatment of miscellaneous idiopathic headache disorders (Group 4 of the IHS classification) -- report of an EFNS task force. Eur J Neurol. 2011 Jun;18(6):803-12. [150 references] PubMed External Web Site Policy
Guideline Status

This is the current release of the guideline.

FDA Warning/Regulatory Alert

Note from the National Guideline Clearinghouse: This guideline references a drug(s) for which important revised regulatory information has been released.

  • August 1, 2013 – Acetaminophen External Web Site Policy: The U.S. Food and Drug Administration (FDA) notified healthcare professionals and patients that acetaminophen has been associated with a risk of rare but serious skin reactions. Acetaminophen is a common active ingredient to treat pain and reduce fever; it is included in many prescription and over-the-counter (OTC) products. These skin reactions, known as Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP), can be fatal. These reactions can occur with first-time use of acetaminophen or at any time while it is being taken. Other drugs used to treat fever and pain/body aches (e.g., non-steroidal anti-inflammatory drugs, or NSAIDS, such as ibuprofen and naproxen) also carry the risk of causing serious skin reactions, which is already described in the warnings section of their drug labels.

Scope

Disease/Condition(s)

Headache disorders

Guideline Category
Management
Prevention
Treatment
Clinical Specialty
Family Practice
Internal Medicine
Neurology
Pediatrics
Intended Users
Advanced Practice Nurses
Nurses
Physician Assistants
Physicians
Guideline Objective(s)
  • To give recommendations for the treatment of headache disorders classified as the so-called group 4 headaches in the second edition of the International Classification of Headache Disorders (ICHD-II)
  • To give drug and nondrug treatment recommendations for these headache disorders for both acute and prophylactic treatment
Target Population

Patients with headache disorders classified as the so-called group 4 headaches in the second edition of the International Classification of Headache Disorders (ICHD-II)

Interventions and Practices Considered
  1. Drug treatment as appropriate
  2. Lumbar puncture
  3. Avoidance of physical activity during heat or at high altitudes
  4. Regular training and slow increase in sports activities
  5. Maintenance of normal body mass index
  6. Avoidance of strong physical activity during sexual activity
  7. Caffeine before sleeping
  8. Computed tomography/magnetic resonance imaging/magnetic resonance angiography to rule out subarachnoid haemorrhage and dissection
Major Outcomes Considered
  • Efficacy of treatments
  • Degree of pain
  • Incidence of headache
  • Rate of remission
  • Quality of life

Methodology

Methods Used to Collect/Select the Evidence
Hand-searches of Published Literature (Secondary Sources)
Searches of Electronic Databases
Description of Methods Used to Collect/Select the Evidence

A literature search was performed using the reference databases MedLine, Science Citation Index, and the Cochrane Library; the key words used were 'headache' together with the respective description of the eight different headache types (last search in April 2010). All articles published in English, German, or French were considered when they described a controlled trial or a case report or series on the treatment of one of these headache disorders. In addition, a review book and the German treatment recommendations for these headache disorders were considered.

Number of Source Documents

Not stated

Methods Used to Assess the Quality and Strength of the Evidence
Weighting According to a Rating Scheme (Scheme Given)
Rating Scheme for the Strength of the Evidence

Evidence Classification Scheme for a Therapeutic Intervention

Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:

  1. Randomization concealment
  2. Primary outcome(s) is/are clearly defined
  3. Exclusion/inclusion criteria are clearly defined
  4. Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
  5. Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences

Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e

Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment

Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion

Methods Used to Analyze the Evidence
Review of Published Meta-Analyses
Systematic Review
Description of the Methods Used to Analyze the Evidence

Not stated

Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations

The first draft of the manuscript was written by the chairman of the task force. All other members of the task force read the first draft and discussed changes by email. A second draft was then written by the chairman, which was again discussed by email. All recommendations had to be agreed to by all members of the task force unanimously. The background of the research strategy and of reaching consensus and the definitions of the recommendation levels used in this study have been described in the "Description of Methods Used to Collect/Select the Evidence" and the "Rating Scheme for the Strength of the Recommendations" fields.

The recommendations are based on the scientific evidence from clinical trials, from case reports, and on the expert consensus by the respective task force of the European Federation of Neurological Societies (EFNS).

Rating Scheme for the Strength of the Recommendations

Rating of Recommendations for a Therapeutic Intervention

Level A (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.

Level B (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.

Level C (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.

Cost Analysis

A formal cost analysis was not performed and published cost analyses were not reviewed.

Method of Guideline Validation
Peer Review
Description of Method of Guideline Validation

The guidelines were validated according to the European Federation of Neurological Societies (EFNS) criteria (see the "Availability of Companion Documents" field).

Recommendations

Major Recommendations

The levels of evidence (class I-IV) supporting the recommendations and ratings of recommendations (A-C) are defined at the end of the "Major Recommendations" field.

Pragmatic Recommendations for the Treatment of Headache Disorders Group 4 of the International Headache Society (IHS) Classification

Primary Stabbing Headache

  • Indomethacin 25–50 mg twice a day (bid) (B)
  • Choice: melatonin 3–12 mg in the evening or gabapentin 400 mg bid (C)

Primary Cough Headache

  • Indomethacin 25–200 mg per day (B)
  • Acetazolamide 125 mg three times a day (tid) (up to 200 tid) (B)
  • Lumbar puncture with decrease in cerebrospinal fluid pressure (B)
  • Methysergide 2 mg, naproxen 550 mg per day (C)

Primary Exertional Headache

  • Avoidance of physical activity during heat or in high altitude
  • Regular training and slow increase in activity in sports
  • Normal body mass index
  • Indomethacin 50–100 mg as short-term prophylaxis long-term prophylaxis (C)
    1. Choice: indomethacin 25–50 mg tid (B)
    2. Choice: propranolol 20–80 mg tid, flunarizine 10 mg per day (C)

Primary Headache Associated with Sexual Activity

  • Avoidance of strong physical activity during sexual activity
  • Indomethacin 50 to 75 mg as short-term prophylaxis (B)
  • Propranolol (B)

Hypnic Headache

  • Caffeine before sleeping (B)
    1. Choice: lithium 300–600 mg per day (B)
    2. Choice: indomethacin 100–150 mg per day, flunarizine 10 mg per day (C)

Primary Thunderclap Headache

  • Acute phase: exclusion of subarachnoid haemorrhage and dissection by computed tomography (CT)/magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) scan, and lumbar puncture
  • In the acute phase pain treatment with 500 mg paracetamol tid, metamizole 500 mg tid, or tramadol 200 mg tid (or similar opioid) after the acute phase in relapsing cases treatment with nimodipine 30–60 mg every 4 h over 14 days (C)

Hemicrania Continua

  • Indomethacin 25 mg tid up to 200 mg per day (A)

New Daily-Persistent Headache

  • Valproic acid 600–900 mg per day
  • Amitriptyline up to 150 mg per day

Definitions:

Evidence Classification Scheme for a Therapeutic Intervention

Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:

  1. Randomization concealment
  2. Primary outcome(s) is/are clearly defined
  3. Exclusion/inclusion criteria are clearly defined
  4. Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
  5. Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences

Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e

Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment

Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion

Rating of Recommendations for a Therapeutic Intervention

Level A (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.

Level B (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.

Level C (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.

Clinical Algorithm(s)

None provided

Evidence Supporting the Recommendations

Type of Evidence Supporting the Recommendations

The type of supporting evidence is identified and graded for selected recommendations (see the "Major Recommendations" field).

The treatment recommendations presented in this article are not based on controlled trials but most often based on case reports or series and on expert consensus.

Benefits/Harms of Implementing the Guideline Recommendations

Potential Benefits

Appropriate treatment of miscellaneous idiopathic headache disorders (the so-called group 4 headaches in the second edition of the International Classification of Headache Disorders [ICHD-II])

Potential Harms
  • In some patients taking indomethacin, agents to protect the stomach are required such as antacid drugs, histamine-2 (H2) antagonists, or proton pump inhibitor.
  • Controls of the thyroid and renal functions are necessary in patients taking lithium.

Qualifying Statements

Qualifying Statements

This guideline provides the view of an expert task force appointed by the Scientific Committee of the European Federation of Neurological Societies (EFNS). It represents a peer-reviewed statement of minimum desirable standards for the guidance of practice based on the best available evidence. It is not intended to have legally binding implications in individual cases.

Implementation of the Guideline

Description of Implementation Strategy

The European Federation of Neurological Societies (EFNS) has a mailing list and all guideline papers go to national societies, national ministries of health, World Health Organisation, European Union, and a number of other destinations. Corporate support is recruited to buy large numbers of reprints of the guideline papers and permission is given to sponsoring companies to distribute the guideline papers from their commercial channels, provided there is no advertising attached.

Implementation Tools
Staff Training/Competency Material
For information about availability, see the Availability of Companion Documents and Patient Resources fields below.

Institute of Medicine (IOM) National Healthcare Quality Report Categories

IOM Care Need
Getting Better
Living with Illness
Staying Healthy
IOM Domain
Effectiveness

Identifying Information and Availability

Bibliographic Source(s)
Evers S, Goadsby P, Jensen R, May A, Pascual J, Sixt G, EFNS task force. Treatment of miscellaneous idiopathic headache disorders (Group 4 of the IHS classification) -- report of an EFNS task force. Eur J Neurol. 2011 Jun;18(6):803-12. [150 references] PubMed External Web Site Policy
Adaptation

Not applicable: The guideline was not adapted from another source.

Date Released
2011 Jun
Guideline Developer(s)
European Federation of Neurological Societies - Medical Specialty Society
Source(s) of Funding

The present guidelines were developed without external financial support.

Guideline Committee

European Federation of Neurological Societies Task Force on Treatment of Miscellaneous Idiopathic Headache Disorders

Composition of Group That Authored the Guideline

Task Force Members: S. Evers, Department of Neurology, University of Münster, Münster, Germany; P. Goadsby, Headache Group, Department of Neurology, University of California, San Francisco CA, USA and UCL, Institute of Neurology, Queen Square, London, UK; R. Jensen, Danish Headache Center, Department of Neurology, Glostrup Hospital, University of Copenhagen, Copenhagen, Denmark; A. May, Department of Systems Neuroscience, University of Hamburg, Hamburg, Germany; J. Pascual, Area of Clinical Neuroscience, Service of Neurology, University Hospital "Central de Asturias", Oviedo, Spain; and G. Sixt, Department of Neurology, Regional Hospital, Bolzano, Italy

Financial Disclosures/Conflicts of Interest

The authors report the following financial supports: Stefan Evers: Salary by the University of Münster; honoraria and research grants by Addex Pharm, AGA Medical, Allergan, Almirall, AstraZeneca, Berlin Chemie, Boehringer, CoLucid, Desitin, Eisai, GlaxoSmithKline, Ipsen Pharma, Janssen Cilag, MSD, Novartis, Pfizer, Pharm Allergan, Pierre Fabre, Reckitt-Benckiser, UCB. Peter J. Goadsby: Salary from the University of California, San Francisco. The author has no relationships that are relevant to this review. Rigmor Jensen: Salary by the University of Copenhagen and Glostrup Hospital; honoraria and research grants by Berlin Chemie, Allergan Norden, Medotech, Norpharma, MSD. Arne May: Salary by the University of Hamburg; research grants by DFG and BMBF; grants and honoraria from several pharmaceutical companies. Julio Pascual: Salary by the Spanish Health Service; honoraria by Allergan, Almirall, Janssen-Cilag, Juste, MSD. Gabriele Sixt: none.

Guideline Status

This is the current release of the guideline.

Guideline Availability

Electronic copies: Available to registered users from the European Federation of Neurological Societies Web site External Web Site Policy.

Availability of Companion Documents

The following are available:

  • Brainin M, Barnes M, Baron JC, Gilhus NE, Hughes R, Selmaj K, Waldemar G; Guideline Standards Subcommittee of the EFNS Scientific Committee. Guidance for the preparation of neurological management guidelines by EFNS scientific task forces – revised recommendations 2004. Eur J Neurol. 2004 Sep;11(9):577-81. Electronic copies: Available in Portable Document Format (PDF) from the European Federation of Neurological Societies (EFNS) Web site External Web Site Policy.
  • Continuing Medical Education questions are available to registered users from the EFNS Web site External Web Site Policy.
Patient Resources

None available

NGC Status

This NGC summary was completed by ECRI Institute on November 20, 2012. The information was verified by the guideline developer on January 30, 2013. This summary was updated by ECRI Institute on July 10, 2013 following the U.S. Food and Drug Administration advisory on Valproate. This summary was updated by ECRI Institute on October 28, 2013 following the U.S. Food and Drug Administration advisory on Acetaminophen.

Copyright Statement

This NGC summary is based on the original guideline, which is subject to the Wiley Online Library copyright restrictions.

Disclaimer

NGC Disclaimer

The National Guideline Clearinghouse™ (NGC) does not develop, produce, approve, or endorse the guidelines represented on this site.

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