Introduction
Objective
Introduction to the guidelines
Target audience
Diagnosis and organization
Occurrence
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150 adults and 30 children with bothersome migraines.
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65 adults and 10 children with daily headaches and a large proportion of these have medication overuse headache.
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One patient with cluster headache.
Type | Probable diagnosis | Description |
---|---|---|
Acute headache | Subarachnoid haemorrhage, and others. | Acute onset, severe headache +/− neurological symptoms |
Episodic Headache | Migraine +/− aura | Pulsating headache, aggravation by physical activity with nausea, phono- and photophobia |
Tension-type headache | Pressure headache without associated symptoms | |
Cluster headache and others | Unilateral headache with ipsilateral autonomic facial symptoms | |
Trigeminal neuralgia | Seconds lasting unilateral severe | |
Chronic headache | Chronic tension-type headache | Pressure headache without associated symptoms or medication overuse headache |
Medication-overuse headache | Use of acute pain medication more than 10–15 days per month | |
Intracranial hypertension, incl. Brain tumor headache | Frequent and increasing headache with nausea and neurological symptoms |
Taking a medical history on headache or facial pain
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New onset headache
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Thunderclap headache (sudden onset of severe headache)
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Sudden headache occurring during strenuous physical or sexual activity
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Headache with atypical aura (lasts over 1 h or includes motor outcomes)
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Headache with aura developed while using birth control pills
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New onset of headache in a patient with cancer or HIV infection
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Headache accompanied by fever
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Headache accompanied by neurological outcomes phrased migraine aura
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Progressive headache over weeks
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New onset headache in patients under 10 years of age or over 40 years of age
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Headache, which is position-dependent
Useful questions | |
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How many different types of headache/facial pain do you have? Separate history must be taken for each type! | |
Time course | When did the headache start? How frequent is the headache (episodic, daily and/or constant)? Duration of each attack (seconds/minutes/hours/days)? |
Character | Pain intensity? Pain quality and type? Where is the pain located and does the pain move? Associated symptoms? |
Reasons | Trigger factors and/or dispositions? Aggravating or soothing factors? Family dispositions for headache / facial pain? |
Ictal behavior | What do you do during the attack? How does the attack affect your activity level? Medication intake, if yes: which and what dose? |
General health state interictally | With or without any symptoms between attacks? Worries of anxiety for new attacks and/or their reasons? |
Physical examination of a headache/facial pain patient
Diagnostic diary and calendar
Organization of treatment in Denmark
Migraine with and without aura
Diagnosis
1.1 [G43.0] Migraine without aura | |
A. At least five attacks fulfilling criteria B-D. | |
B. Headache attacks lasting 4–72 h (untreated or unsuccessfully treated) | |
C. Headache at least two of the following four characteristics: | |
1. unilateral location | |
2. pulsating quality | |
3. moderate or severe pain intensity | |
4. aggravation by or causing avoidance of routine physical activity (e.g. walking or climbing stairs) | |
D. During headache at least one of the following: | |
1. nausea and/or vomiting | |
2. photophobia and phonophobia | |
Not better accounted for by another ICDH-3 diagnosis | |
1.1 [G43.1] Migraine with aura | |
A. At least two attacks fulfilling criteria B-C | |
B. One or more of the following fully reversible aura symptoms: | |
1. visual | |
2. sensory | |
3. speech and/or language | |
4. motor | |
5. brainstem | |
6. retinal | |
C. At least three of following six characteristics: | |
1. at least one aura symptom spreads gradually over ≥5 min | |
2. two or more aura symptoms occur in succession 3. each individual aura symptom lasts 5–60 min | |
4. at least one aura symptom is unilateral | |
5. at least one aura symptom is positive | |
6. the aura is accompanied, or followed within 60 min, by headache | |
D. Not better accounted for by another ICDH-3 diagnosis. |
1.3 [G43.3] Chronic Migraine | |
A. Headache (migraine-like or tension-type-like) on ≥15 days for > 3 months, and fulfilling criteria B and C | |
B. Occurring in a patient who has had at least five attacks fulfilling criteria B-D for Migraine without aura and/or criteria B-C for Migraine with aura | |
C. On ≥8 days/month for > 3 months, fulfilling any of the following: | |
1. criteria C and D for Migraine without aura | |
2. criteria B and C for Migraine with aura | |
3. believed by the patient to be migraine at onset and relieved by a triptan or ergot derivate | |
D. Not better accounted for by another ICDH-3 diagnosis |
Migraine | Tension-type headache | |
---|---|---|
Time pattern | Attacks lasting 4–72 h | Varies, from episodes lasting 30 min to a continuous headache |
Headache characteristics | Frequently unilateral and pulsating. Aggrevation by physical activity | Frequently bilateral and pressing. Normally no aggravation by physical activity |
Intensity | Tyically moderate to servere | Typically mild to moderate |
Accompanying symptoms | Frequent nausea and/or vomiting, photophobia or phonophobia | None or only mild nausea, photophobia or phonophobia |
Background
Clinical assessment and special assessment program
Non-pharmacological treatment
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Biofeedback therapy has a documented effect on migraine [6].
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Acupuncture in addition to symptomatic treatment has in a meta-analysis shown to reduce the number of headache days corresponding to preventive medical treatment, but there is only a small difference compared to placebo [7].
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There is consensus, but not clear scientific evidence, that physiotherapy should primarily focus on instruction in relaxation, correct working postures, posture correction and instruction in active home exercises [8].
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Physical exercise can be beneficial to reduce the headache duration, but not frequency or intensity [8].
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Behavioural therapy and cognitive therapy (stress and pain management) may be effective, but are only offered to a limited extent in Denmark.
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Information about the causes of migraine and the possibilities for treatment, thorough physical examination, as well as simply taking the patient seriously, can have a beneficial effect in some patients.
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Identify and reduce as much as possible, predisposing factors such as stress, depression or anxiety.
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Identify and eliminate triggers, e.g. irregular lifestyle (e.g. poor sleep patterns or irregular food intake). Intake of provocative food items such as red wine, dark chocolate and certain cheeses may in some patients have an effect, although there is no clear evidence for this.
Pharmacological treatment
Acute treatment
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No definite difference has been demonstrated in the effect between simple analgesics (paracetamol, NSAIDs and acetylsalicylic acid) alone or in combination with antiemetics and triptans [9]. Simple analgesics, if necessary combined with antiemetics, are therefore the first choice [10]. Many of the patients who have insufficient effect of simple analgesics have good effect from triptans [10].
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Step-wise treatment is recommended in which each step comprises three treatments before proceeding to the next step. Hereby, the most effective and inexpensive treatment is achieved [10].
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The first step consists of simple analgesics and an antiemetic if needed.
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The second stage consists of triptans.
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Ergot alkaloids (ergotamine derivate) are rarely used due to the risk of serious side effects. These should only be used by specialists.
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Treatment should be initiated as early as possible. In migraine with aura, however, triptans should be taken only when the headache starts.
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Analgesics often has a better effect when combined with rest and, possibly, sleep. If the patient has difficulty calming down, benzodiazepine may be given e.g. 5 mg diazepam.
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Pay attention to medication overuse headaches (see Section “Medication overuse headaches”).
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For concomitant nausea, simple analgesics may be combined with antiemetics to treat the nausea, but there is no conclusive evidence that antiemetics improve the absorption of common analgesics [11]. Metoclopramide tablet 10 mg or tablet domperidone 10 mg can be used (the latter used especially for young people due to less risk of extrapyramidal side effects). See Table 6.
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Simple analgesics should be used for a maximum of 14 days per month to avoid medication overuse headache.
Analgesics | Initial Dose | Antiemetics | Initial Dose |
---|---|---|---|
Ibuprofen | 400–600 mg | Metoclopramide | 10 mg |
Naproxen | 500 mg | Domperidone | 10 mg |
Paracetamol | 1.000 mg | ||
Aspirin/caffeine | 500/50 mg |
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There are clinically relevant differences between the seven oral triptans in terms of efficacy and side effects (Table 7). In addition, there can be significant differences in how well the individual patient responds to the different triptans, therefore the choice of triptan should be individualized.
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Patients who have no effect of one particular triptan may benefit from another triptan. Before the effect of any triptan is ruled out, the patient should, as a rule of thumb, have tried three different triptans, each during three attacks.
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There are significant price differences between the triptans.
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There is no evidence that the effect of orally disintegrating tablets or rapidly soluble tablets is any quicker than that of standard tablets. Nasal spray and subcutaneous injection act more rapidly than tablets.
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A combination of triptan and NSAIDs may be more effective in some patients than each drug alone [14].
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Oral triptans may, in case of nausea/vomiting, be combined with the antiemetic metoclopramide [15] or domperidone; in some cases non-oral administration is an advantage (nasal spray or subcutaneous injection).
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Approximately 20–50% of patients experience recurrence of migraine within 48 h. An additional dose of triptan is usually effective in these cases. Recurrence of migraine can also be treated with NSAIDs.
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In the absence of effect of a triptan, repeating the treatment with triptan in the same attack is usually ineffective.
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Triptans should be used for a maximum of 9 days per month to avoid headache medication overuse.
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Common side effects are a sensation of pressure on the chest, nausea, distal paraesthesia and fatigue.
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Triptans have not been systematically tested in hemiplegic migraine or migraine with brainstem aura and is not recommended for use in these patients in some countries. However, studies in hemiplegic migraine and migraine with brainstem aura patients have not shown risk of using triptans [16, 17]. If these patients do not have previous clinical stroke, uncontrolled hypertension, ischemic heart disease or peripheral vascular disease, triptans can be used. Of note, experimental studies in humans have shown that sumatriptan do not constrict intracerebral vessels [18, 19].
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Triptans are generally contraindicated in patients with previous stroke. Some migraine patients can have accidental findings of white matter changes or infarct-like changes on brain imaging. If these patients do not present history of clinical stroke, uncontrolled hypertension, IHD or peripheral vascular disease, triptans can be used.
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Triptans are, among others, contraindicated in uncontrolled hypertension, ischemic heart disease, previous cerebral infarction and peripheral vascular diseases. Caution should be exercised when treating patients < 18 years and > 65 years. However, Sumatriptan 10 mg nasal spray is approved for adolescents aged 12–17 years. See www.promedicin.dk for a detailed list.
Triptan | Formulation | Therapeutic gain (pain freedom after 2 h) | Comments |
---|---|---|---|
Sumatriptan | Tablet 50 mg | 17% | |
Tablet 100 mg | 21% | ||
Nasal spray 10 & 20 mg | 21% (20 mg) | ||
Subcutaneous injection 6 mg | 44% | ||
Zolmitriptan | Tablet 2.5 mg Soluble tablet 2.5 mg | 20% | Soluble tablets have same efficacy as tablets |
Naratriptan | Tablet 2.5 mg | 15% | Side effects equals placebo |
Rizatriptan | Tablet & soluble tablets 5 and 10 mg | 30% | 5 mg if concomitant treatment with propranolol |
Almotriptan | Tablet 12,5 mg | 23% | Side effects equal placebo |
Eletriptan | Tablet 40 mg | 28% | 80 mg suggested, if 40 mg does not work |
Frovatriptan | Tablet 2.5 mg | 8% | Probable slower onset of effect but longer lasting compared to sumatriptan |
Preventive treatment
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Preventive treatment is offered to reduce the frequency or severity of attacks.
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Preventive treatment should be considered [10] if
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The number of migraine days per month is four or higher.
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attack medications have poor effect.
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the patient’s quality of life is reduced considerably due to the migraine.
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frequent or very long-lasting cases of aura occur.
-
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Thorough information to the patient about the purpose, side effects and realistic expectations about treatment effect is important.
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Preventive treatment is generally considered successful if the frequency or severity of migraine can be halved without the occurrence of bothersome side effects [10].
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Choose preventive medication based on scientific evidence for efficacy, side effect profile and competing disorders.
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Use slow titration to minimize side effects.
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Use a headache calendar to document the effect.
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The prevention treatment should attempted for a minimum of 2–3 months at full dose, before it may be finally assessed whether there is an effect (unless it is not tolerated due to side effects).
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In case of effect, the preventive drug should be discontinued every 6–12 months to ensure that there is still a need for and effect of the medication.
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Lack of effect of one type of preventive does not preclude effect of other types of prevention.
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There is no evidence of an effect when combining several forms of preventive.
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At ≥15 headache days per month, medication overuse should be ruled out.
Drug | Daily dose |
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Metoprolol/propranolol | 50–200 mg/40–240 mg |
Candesartancilexetil | 16 (24–32) mg |
Topiramate | 25–100 (200) mg |
Amitriptyline | 10–100 mg |
Flunarizin | 5–10 mg |
Valproate | 500–1800 mg |
Lisinopril | 20 mg |
Pitzotifen | 1.5–3 mg |
Riboflavin | 400 mg |
Magnesium | 360 mg |
Drug | Daily dose |
---|---|
Beta-blockers: | |
Metoprolol | 50–200 mg |
Propranolol | 40–240 mg |
Candesartancilexetil | 16 (24–32) mg |
Topiramate | 50–100 (200) mg |
Amitriptyline | 10–100 mg |
Botulinum type a toxin | 155–195 units i.m. Every 12 weeks |
CGRP-antibodies: | |
Erenumab | 70–140 mg s.c. every 4 weeks |
Fremanezumab | 225 mg s.c. every month or 675 mg s.c. every 3 months |
Galcanezumab | Start dose 240 mg s.c. followed by 120 mg s.c. every month. |
Eptinezumab | 100–300 mg i.v. every 3 months |
Tension-type headache
Diagnosis
2.1 [G44.2] Infrequent episodic tension-type headache | |
A. At least 10 episodes of headache occurring on < 1 day/month on average (< 12 days/year) and fulfilling criteria B-D | |
B. Lasting from 30 min to 7 days | |
C. At least two of the following four characteristics: | |
1. bilateral location | |
2. pressing or tightening (non-pulsating) quality | |
3. mild or moderate intensity | |
4. not aggravated by routine physical activity such as walking or climbing stairs | |
D. Both of the following: | |
1. no nausea or vomiting | |
2. no more than one of photophobia or phonophobia | |
E. Not better accounted for by another ICHD-3 diagnosis. | |
2.2 [G44.2] Frequent episodic tension-type headache | |
As 2.1 apart from: | |
A. A minimum of 10 episodes of headache occurring on ≥1 day/month but < 15 days/month on average (≥12 and < 180 days/year) and fulfilling criteria B-D | |
2.3 [G44.2] Chronic tension-type headache | |
As 2.1 apart from: | |
Headache occurring on ≥15 days/month on average for > 3 months (≥180 days/year), fulfilling criteria B-D | |
A. A minimum of 10 episodes of headache occurring on ≥1 day/month but < 15 days/month on average (≥12 and < 180 days/year) and fulfilling criteria B-D | |
B. Lasting hours to days, or unremitting | |
C. Both of the following: | |
1. no more than one of photophobia, phonophobia or mild nausea | |
2. neither moderate or severe nausea nor vomiting |
Background
Clinical assessment and special assessment program
Non-pharmacological treatment (see Table 11)
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Treatment of tension-type headaches is based primarily on non-pharmacological interventions. There is little or no scientific evidence for this, so the following recommendations are based on “expert opinion” [27].
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Identify and eliminate, to the extent possible, triggers, e.g. stress or unphysiological work postures. Physical activity may be beneficial.
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Provide information about the causes of tension-type headaches. It may be explained that each headache episode can be due to muscle tension or stress, while in chronic headache there may be a disturbance in the pain-regulating centres of the brain.
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Physiotherapy should primarily be aimed at instruction in correct working postures, posture correction and instruction in active home exercises aimed at reducing musculoskeletal tensions [27]. Passive physiotherapy has no lasting effect and is not recommended.
-
Behavioural therapy and cognitive therapy (stress and pain management) are typically handled by psychologists. The treatment involves instruction in relaxation, biofeedback (electromyography (EMG) and temperature) and cognitive techniques (including restructuring of negative thoughts). The focus is on managing pain and stress [27]. This type of treatment is currently only offered in a few places in Denmark. Biofeedback therapy has a documented effect on tension headaches [6].
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Acupuncture has a documented effect [28]. However, only a minimal effect following active treatment was found compared to sham treatment.
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Manipulation of the cervical spine and blockage of the greater occipital nerve have not had an effect in the few controlled studies performed.
Non-pharmacological treatment of tension-type headache | |
• Physical and neurological examination and reassurance. | |
• Rule out other underlying disorder e.g. depression or oromandibular dysfunction. | |
• Rule out overuse of analgesics | |
• Inform the patient about pain mechanisms | |
• Minimize, as possible, provoking factors, e.g. stress or non-physiological work posture | |
• Physiotherapy (daily exercises and posture correction) | |
• Biofeedback | |
• Stress- og pain management | |
• Acupuncture |
Pharmacological treatment (see Table 12)
Attack treatment
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Ibuprofen 400 mg (200–600 mg)
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Aspirin 1000 mg (500–1000 mg)
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Naproxen 500 mg (250–500 mg)
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Paracetamol 1000 mg
Pharmacological treatment of tension-type headache | |
• Treatment of the individual episode (NSAID, paracetamol). | |
• Avoid overuse of analgesics. | |
• Avoid opioids. | |
• Prophylactic treatment should be considered for chronic tension-type headache when there is insufficient effect of non-pharmacological treatment. | |
• Amitriptyline, mirtazapine and venlafaxine may have preventive effects. | |
• Remember to inform that antidepressants are given to increase the concentration of pain-inhibitory neurotransmitters in the central nervous system and not to treat depression. | |
• Use a headache calendar to monitor the treatment effect | |
• Prophylactic medication should be discontinued after 6–12 months to see if there is still a need for the medication. |
Preventive treatment
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Inform thoroughly about the mechanisms of action (especially that antidepressants are not given on the indication depression) and side effects.
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Slow escalation to minimize side effects.
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Use a sufficiently high dose.
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Monitor effect using a headache calendar (see Fig. 2, can be downloaded at dhos.dk or downloaded as an app for smartphone).
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Asses the effect after 1–2 months on the final dose.
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Attempt discontinuation every 6–12 month.
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ECG should be checked before starting treatment and again at doses above 40 mg / day.
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10 mg × 1, increasing by 10 mg per week until an effect is achieved or significant side effects occur.
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The maintenance dose where the best balance between effect and side effects is typically is 30–75 mg.
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The full dose is given 1–2 h before bedtime to improve sleep and minimize fatigue the next day.
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Typical side effects include dry mouth, fatigue, dizziness and weight gain.
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Nortriptyline can be considered as an alternative used in the same way and with the same doses as amitriptyline. It probably improves sleep less than amitriptyline but also causes less sedation [30].
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15 mg × 1, increasing to 30 mg × 1 after one week.
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Administered approximately one hour before bedtime.
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Typical side effects include fatigue, weight gain and dizziness.
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75 mg × 1, increasing to 150 mg × 1 after one week.
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Administered approximately one hour before bedtime.
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Typical side effects include fatigue, abdominal pain, nausea and dizziness.
Summary
Cluster headache
Diagnosis
3.1 [G44.0/N90] Cluster headache [2] | |
A. At least five attacks fulfilling criteria B-D | |
B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15–180 min (when untreated)1 | |
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 rhinorrhoea | |
- Eyelid oedema | |
- Forehead and facial sweating | |
- miosis and/or ptosis | |
2. a sense of restlessness or agitation | |
D. Occurring with a frequency between one every other day and 8 per day2 | |
E. Not better accounted for by another ICHD-3 diagnosis | |
3.1. [G43.01/N89] Episodic cluster headache | |
A. Attacks fulfilling criteria 3.1 and occurring in bouts (cluster periods) | |
B. At least two bouts lasting from 7 days to 1 year (when untreated) and separated by pain-free remission periods of ≥3 months | |
3.1.2 [G43.02/N89] Chronic cluster headache | |
A. Attacks fulfilling criteria 3.1 and occurring in bouts (cluster periods) | |
B. Occurring without a remission period, or with remission lasting < 3 months, for at least 1 year |
Background
Clinical evaluation
Cluster headache | Paroxysmal hemicrania | SUNCT | |
---|---|---|---|
Epidemiology | |||
Gender ratio (M:F) | 2–4:1 | 1:2–3 | 8–12:1 |
Prevalence | 0,9% | 0,02% | Very rare |
Typical age of onset | 20–40 years of age | 20–40 years of age | 20–50 years of age |
Pain | |||
Character | Drilling/stabbing/squeezing | Drilling | Stabbing |
Intensity | Very severe | Severe | Severe |
Localization | Periorbital | Orbital, temporal | Orbital, temporal |
Attack duration | 15–180 min | 2–30 min | 1–600 s. |
Attack frequency | 1–8 per day | 1–40 per day | 3–200 per day |
Autonomic symptoms | Yes | Yes | Yes |
Effect of indomethacin | No | Yes | No |
Attack treatment | Oxygen 12–15 l/min Inj. Sumatriptan Nasal spray sumatriptan | None | None |
Prophylactic treatment | Verapamil, prednisone | Indomethacin | Lamotrigine, Topiramate, Gabapentin |
Non-pharmacological treatment
Pharmacological treatment (Table 15)
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If cluster headache is suspected, patients should be referred sub-acutely to a private neurologist or a department of neurology to facilitate correct diagnosis and thereby correct treatment.
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Patients should immediately receive acute treatment for the attacks and preventive treatment aiming to reduce attack frequency and pain intensity.
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The dosage of the preventive treatment should be increased as fast as possible.
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The dosage of the preventive treatment should be gradually reduced if patients are attack-free for 14 days (please be aware that patients may experience milder attacks and/or autonomic symptoms indicating that the bout is still active) or when patients sense that the bout has ended.
Type | Dose |
---|---|
Attack treatment: | |
Inhalation of 100% oxygen | 12–15 l/min via O2ptimask [36] w. 3 l reservoir or DVO mask |
Inj. sumatriptan | 6 mg |
Sumatriptan nasal spray | 20 mg |
Preventive treatment | |
Tablet verapamil retard | Initially 100 mg × 2 for 3 days, hereafter 200 mg × 2. Possible further increase to 400–600 mg daily. Rarely up to 1000 mg |
Transitional treatment | |
Occipital nerve block (GON) / tablet prednisone | Please refer to text |
Acute treatment
Preventive treatment
-
A greater occipital nerve block (GON-block) with 2 ml of betamethasone and 0.5 ml of lidocaine 20 mg/ml injected in the neck. The block should be injected halfway between protuberantia occipitalis externa and processus mastoideus, where the greater occipital nerve can be palpated. It is not complicated to perform the block and it is not necessarily a specialist task. If efficient, the effect typically lasts three to 4 weeks. Three months must pass between blocks to avoid tissue necrosis and alopecia.
-
Prednisone tablets 75 mg once a day for 5 days hereafter reducing the dose with 12.5 mg a day, is also a very efficient treatment.
Summary
Medication overuse headache
Diagnosis
A. Headache occurring on ≥15 days/month in a patient with a pre-existing headache disorder | |
B. Regular overuse for > 3 months of one or more drugs that can be taken for acute and/or symptomatic treatment of headache: | |
1. Simple analgesics (paracetamol or NSAIDs) for ≥15 days/month | |
2. Ergotamines, triptans, opioids, combination analgesics or any combination of the above mentioned ≥10 days/month | |
C. Not better accounted for by another ICHD-3 diagnosis. |
Background
Clinical assessment
Treatment of MOH
Non-pharmacological treatment
Elements of treatment | |
• Complete stop of all kinds of short-term medication, including analgesics and acute migraine medication for 2 months. Alternatively, reduced intake of short-term medication to maximum 2 days a week, but this approach works less effectively. | |
• Detailed information to patients, relatives and health care professionals. | |
• Support, information and treatment of withdrawal symptoms. | |
• In-patient care at a neurology department when substantial co-morbidities or high risk of severe withdrawal symptoms. | |
• Recommendation of 2–3 weeks of sick leave. | |
• Close follow-up during the first year after withdrawal. |
Pharmacological treatment during withdrawal therapy
Rescue medication in week 1–3 of the withdrawal may be needed: | |
• Tablet levomepromazine 12.5–25 mg as needed max. 75 mg/day, or tablet promethazine 25 mg as needed max. 75 mg/day. | |
• Tablet metoclopramide 10 mg or tablet domperidone 10 mg against nausea as needed max. 30 mg/day. | |
• In case of opioid- or barbiturate overuse: Tablet methadone, e.g. 20 mg, dosage decreased over next 4 days (only in-patient care). | |
• Early start of preventive headache medication simultaneously with start of withdrawal should be considered | |
After 2 months of withdrawal | |
• If not started earlier, start of preventive headache medication should be started. | |
• Detailed information to the patient about correct use of short-term medication, preventive medication, and headache calendar to prevent relapse. | |
• Treatments that previously were ineffective due to medication overuse, may become effective after withdrawal. |
Preventive medication for MOH
Summary
Secondary types of headache
Diagnostic criteria
Background
Special investigation program
Clinical assessment
-
New onset headache
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Thunderclap headache (sudden onset of severe headache)
-
Sudden headache occurring during strenuous physical or sexual activity
-
Headache with atypical aura (lasts over 1 h or includes motor outcomes)
-
Headache with aura developed while using birth control pills
-
New onset of headache in a patient with cancer or HIV infection
-
Headache accompanied by fever
-
Headache accompanied by focal neurological signs except transient attributed to migraine aura
-
Progressive headache over weeks
-
New onset headache in patients under 10 years of age and over 40 years of age
-
Headache that is position dependent
Chronic post-traumatic headache
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In the vast majority of cases, the headache will be of the migraine or tension-type headache type
-
Frequent complaints of difficulty concentrating, memory problems, fatigue, hypersensitivity to light and sounds, visual disturbances, dizziness and irritability
Medication overuse headache
Idiopathic intracranial hypertension (IIH)
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By far most common in obese people
-
Papilledema is the most prominent feature
-
The headache may worsen in the supine position and be worst in the morning
-
In addition to headaches, there may be neck pain / back pain, visual field defects, transient visual obscurations, abduction paresis and pulsating tinnitus
-
Suspected cases require acute hospitalization (important differential diagnosis: sinus vein thrombosis) and neuroradiological examination, possibly measurement of the cerebrospinal pressure, which will be elevated more than 25 cm H2O
-
Untreated intracranial hypertension can lead to permanent visual impairment or blindness
Low-pressure headaches
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Typically seen after lumbar puncture but can be seen spontaneously.
-
Typical deterioration in an upright position and rapid improvement in a supine position.
Subarachnoid haemorrhage (SAH)
-
May be initiated by generalized seizures
-
May be followed by changes in the level of consciousness
Giant cell arteritis (arteritis temporalis)
-
Headache and general symptoms (e.g. fatigue, fever, night sweats, and weight loss)
-
Tenderness on palpation of the temporal artery
-
Chewing claudication (up to 40% have this)
-
Amaurosis fugax (approximately 10%), which may lead to blindness if left untreated.
-
Elevated CRP or erythrocyte sedimentation rate (in rare cases, however, these may be normal)
-
Arteria temporalis biopsy positive in 50% of cases. Treatment should be started on clinical suspicion before a possible biopsy response. Contact on-duty doctor in internal medicine or rheumatology in case of suspicion.
Primary glaucoma
-
Rarely occurring before the age of 50.
-
Risk factors: familial disposition, woman and myopia.
-
The condition may manifest as acute ocular hypertension.
-
Painful red eye.
-
Medium dilated pupil without light reaction.
-
Accompanied by nausea and vomiting.
-
Complains of blurred vision and coloured rings around light objects.
Cerebral venous thrombosis
-
Supranuclear palsy (60%)
-
Papilledema (30–60%)
-
Meningism (25–30%)
-
Decreased level of consciousness (60%)
-
Epileptic seizures, possibly with Todd’s palsy (40–50%)
Arterial dissection
Brain tumour
-
New onset of headache (tension-type character),
-
Worsening of pre-existing headache,
-
Headache worst in the morning and accompanied by nausea,
-
Possibly with epileptic seizures,
-
Cognitive change or personality change,
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Speech disorders and / or hemiparesis.
Neuroinfection
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May present with cognitive impairment, photophobia or petechiae
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May present with seizures
Sinusitis
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Pain localization above, behind and under the eyes, aggravation by forward bending.
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Tightness in the nose
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Can present with fever and malaise
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Some patients may experience blurred vision
Other reasons
Trigeminal neuralgia
Diagnosis
• Trigeminal neuralgia (TN) is a unilateral disorder of short-lasting stabbing pain paroxysms. | |
• The painful area typically involves the 2nd and/or 3rd trigeminal branch. | |
• The mean age of onset is 52 years, but the range of onset is wide (8–90 years). | |
• Pain attacks are evoked by light sensory stimuli such as chewing, touching the face, talking, tooth brushing, shaving and cold wind. There can also be spontaneous pain. | |
• Trigger zones are often located around the nasal wing and the lateral part of the upper and lower lip. | |
• Natural history of TN is unpredictable. There may be severe exacerbations of pain and there may be periods of complete pain remission lasting for weeks and months – in some cases even years. | |
• Symptomatic TN is caused by a brainstem plaque from multiple sclerosis or by a space-occupying lesion in the cerebellopontine angle cistern. At clinical presentation, it can be indistinguishable from primary TN. |
A. Recurrent paroxysms of unilateral facial pain in the distribution(s) of one or more divisions of the trigeminal nerve, with no radiation beyond, and fulfilling criteria B, C and D | |
B. Pain has all of the following characteristics: | |
1. lasting from a fraction of a second to 2 min | |
2. servere intensity | |
3. electric shock-like, shooting, stabbing or sharp in quality | |
C. Precipitated by innocuous stimuli within the affected trigeminal distribution | |
D. Not better accounted for by another ICHD-3 diagnosis |
Background
Clinical assessment
Non-pharmacological treatment
Pharmacological treatment
Acute treatment
Preventive treatment
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Initial dose is 100–200 mg BID. Titrate 100 mg every 3rd day until pain freedom or unacceptable side effects
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Typical maintenance dose is 100–600 mg BID
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Daily doses of 1800 mg or more may be necessary
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Initial dose is 150–300 mg BID. Titrate 150 mg every 3rd day until pain freedom or unacceptable side effects
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Typical maintenance dose is 150–900 mg BID
-
Daily doses of 2700 mg or more may be necessary
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Initial dose is 300 mg daily titrating with 300 mg every 3rd day until efficacy or unacceptable side effects. Maximum daily dose is 3600 mg divided in three doses.
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Typical maintenance dose is between 300 mg BID up to 1200 mg TID.
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Initial dose is 75 mg BID titrating with 150 mg every 7th day until efficacy or unacceptable side effects. Maximum daily dose is 600 mg divided in two doses.
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Typical maintenance dose is 75 mg - 300 mg BID.
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Initial dose is 25 mg daily for 2 weeks, then 50 mg daily for 2 weeks and hereafter titrating with 50 mg per week until 50 mg BID. Observe efficacy. The drug can be further titrated with 50 mg per week until efficacy or unacceptable side effects.
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Typical maintenance dose is 50 mg - 200 mg BID.
Neurosurgical treatment
Microvascular decompression
Percutaneous procedures
Summary
Hormones and migraines
Diagnosis
Background
Specific risk factors
Choice of contraception
Migraine with aura
Migraine without aura
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Use of oestrogen-containing contraceptive pills, where there is no contraceptive pill break through several cycles, e.g. by taking birth control pills continuously for 9 weeks (instead of the usual 3 weeks) followed by a 7 day pill-free period. If breakthrough bleeding occurs earlier, there is a pause at the time of breakthrough bleeding.
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Use of mini-pills (containing desogestrel 75 μg / day only).
Treatment of menstrual migraines
Pregnancy
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As far as possible non-pharmacological with calm, bed rest, ice packs and the like.
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If drug treatment is needed, paracetamol is the first choice.
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NSAIDs should be avoided.
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Sumatriptan can be used if necessary. The other triptans are not recommended due to sparse data.
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Metoclopramide can be used. Domperidone is not recommended.
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Ergotamine is contraindicated due to the uterine contracting effect [82].
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As far as possible non-pharmacological: regular lifestyle, incl. Regular nutritious meals, sleep, physical activity and tranquillity.
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Beta-blockers can be used in the lowest possible dosage, but there is a risk of side effects in the newborn such as bradycardia, hypotension and hypoglycaemia.
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Antiepileptic drugs and antidepressants are not recommended [82].
Breastfeeding
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As far as possible non-pharmacological with calm, bed rest, ice packs and the like.
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If the above has been tried and is insufficient, paracetamol can be used.
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NSAIDs can be used; ibuprofen is preferable (due to short half-life, no active metabolites and low concentration in breast milk).
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Sumatriptan and eletriptan can be used if necessary. Breast-feeding is not recommended for 12 h after ingestion of other triptans.
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Caution with metoclopramide as it is absorbed into breast milk.
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Avoid acetylsalicylic acid, benzodiazepines and ergotamine.
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Avoid drug prevention as much as possible.
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Beta-blockers, valproate and amitriptyline may be used [82].
Children and headaches
Diagnosis
1.1 [G43.1b] Migraine without aura | |
A. At least five attacks fulfilling criteria B-D. | |
B. Headache attacks lasting 2–72 h (untreated or unsuccessfully treated) | |
C. Headache at least two of the following four characteristics: | |
1. unilateral location | |
2. pulsating quality | |
3. moderate or severe pain intensity | |
4. aggravation by or causing avoidance of routine physical activity (e.g. walking or climbing stairs) | |
D. During headache at least one of the following: | |
1. nausea and/or vomiting | |
2. photophobia and phonophobia | |
E. Not better accounted for by another ICDH-3 diagnosis | |
1.1 [G43.1b] migraine with aura | |
Children and adults share the same diagnostic criteria | |
1.6.1.1 [G43.1B] Cyclical vomiting syndrome | |
A. At least five attacks of intense nausea and vomiting, fulfilling criteria B and C | |
B. Stereotypical in the individual patient and recurring with predictable periodicity | |
C. All of the following: | |
1. nausea and vomiting occur at least four times per hour | |
2. attacks last for ≥1 h, up to 10 days | |
3. attacks occur ≥1 week apart | |
D. Complete freedom from symptoms between attacks | |
E. Not attributed to another disorder | |
1.6.1.2 [G43.1B] Abdominal migraine | |
A. At least five attacks of abdominal pain, fulfilling criteria B–D | |
B. Attacks last 2–72 h when untreated or unsuccessfully treated | |
C. Pain has at least two of the following three characteristics: | |
1. midline location, periumbilical or poorly localized | |
2. dull or “just sore” quality | |
3. moderate or severe intensity | |
D. At least two of the following four associated symptoms or signs: | |
1. anorexia | |
2. nausea | |
3. vomiting | |
4. pallor | |
E. Complete freedom from symptoms between attacks | |
F. Not attributed to another disorder | |
1.6.2. [G43.1B] Benign paroxysmal vertigo | |
A. At least five attacks fulfilling criteria B and C | |
B. Vertigo occurring without warning, maximal at onset and resolving spontaneously after minutes to hours without loss of consciousness | |
C. At least one of the following five associated symptoms or signs: | |
1. nystagmus | |
2. ataxia | |
3. vomiting | |
4. pallor | |
5. fearfulness | |
D. Normal neurological examination and audiometric and vestibular functions between attacks | |
E. Not attributed to another disorder | |
[G44.2] Tension-type headache | |
Children and adults share the same diagnostic criteria |
Background
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Shorter attack duration,
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More frequent bilateral localization
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Pronounced gastrointestinal symptoms.
Clinical assessment
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Older children and young people can use the headache diary without any problems.
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Most 7–11-year-olds can self-report pain frequency and intensity but may depend on an adult to record their other symptoms.
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In young children, parents can observe and report their children’s symptoms [83] and the intensity of the pain can be determined by children, using a visual analog scale.
Treatment
Non-pharmacological treatment
Non-pharmacological treatment of headache in children | |
• Objective examination and reassurance. | |
• Exclude other underlying disorder e.g. stress, psychogenic factors (problems at home, school or / and among peers), depression, depression, anxiety, refraction anomalies, strabismus, over-strained eyes (computer work / games), oromandibular dysfunction, sinusitis, posture anomaly, passive / active smoking and inappropriate lifestyle. | |
• Exclude medication-overuse headache. | |
• Inform about disease mechanisms so that both child and parents understand it. | |
• Minimize or eliminate triggers, e.g. stress or poor posture during schoolwork. |
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Identification and elimination / reduction of provocative headache trigger factors.
-
Talk to the child about fluid intake, regular meals / diets and sleep patterns.
-
Talk to the child about excessive TV-screen time and lack of physical activity.
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Talk to the child about any stressors / pain management. In case of stress and / or pain, biofeedback / relaxation and cognitive therapy for pain coping is used.
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Identify possible comorbidity (focus points are attention problems, social difficulties, professional difficulties, family challenges / illness) - as unidentified comorbidity can cause stress. In case of symptoms of stress, relevant investigation / help is initiated.
-
Identify any dental or back problems - initiate appropriate help.
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Identify any vision problems - initiate relevant help.
-
Identify medication overuse headache.
-
Thorough information of children / youth and their parents regarding conclusion and plan for treatment. In addition, it is important to minimize their possible concern about serious illness - when there is no evidence of this.
Pharmacological treatment
Pharmacological treatment of headache in children | |
• Treatment of acute tension headache attacks (paracetamol and / or NSAIDs). | |
• Treatment of acute migraine attacks (paracetamol and / or ibuprofen possibly combined with domperidone (at age > 12 years and weight > 35 kg)), alternatively (sumatriptan nasal spray (children> 12 years) or tablet zolmitriptan (children> 12 years) possibly in combination with ibuprofen). | |
• Generally avoid overuse of painkillers. | |
• Preventive treatment is considered for very frequent or severely disabling headaches, where there has been insufficient effect of the non-pharmacological treatment and where the acute seizure treatment is inadequate and medication overuse headache is excluded. | |
• Beta-blockers and flunarizine have some proven prophylactic effect, but in general, there is very little scientific evidence that prophylactic medical treatment has an effect in children with migraines and tension headaches. | |
• If prophylactic treatment is needed, the general rules for adults are followed. |