Reference programme: diagnosis and treatment of headache disorders and facial pain. Danish Headache Society, 3rd edition, 2020

DOI: https://doi.org/10.1186/s10194- 021- 01228- 4

Abstract-Summary Headache and facial pain are among the most common, disabling and costly dis- eases in Europe, which demands for high quality health care on all levels within the health system.

The role of the Danish Headache Society is to educate and advocate for the needs

of patients with headache and facial pain.

The Danish Headache Society has launched a third version of the guideline for the diagnosis, organization and treatment of the most common types of headaches and facial pain in Denmark.

The recommendations for the primary headaches and facial pain are largely in accordance with the European guidelines produced by the European Academy of Neurology.

The guideline should be used a practical tool for use in daily clinical practice for primary care physicians, neurologists with a common interest in headache, as well as other health-care professionals treating headache patients.

The guideline first describes how to examine and diagnose the headache patient

and how headache treatment is organized in Denmark.

Introduction The vast majority of Danes suffering from headache are treated in the primary sec- tor and should for the most part continue to be treated there in the future, but there is an increasing need for clear guidelines for examining and organizing specialist treatment of severe and rare headache conditions.

There are international guidelines and general recommendations for the treat-

ment of migraines and other primary headache diseases.

The Danish Headache Society created a working committee to update the Danish

reference program for headache diseases and facial pain from 2010.

The objective is to create common guidelines for diagnosing, organizing and treating the most common primary headache diseases such as migraines, tension- type headache and cluster headache as well as trigeminal neuralgia in Denmark, as well as describe important warning signs of serious life-threatening and other sec- ondary headache conditions.

Diagnosis and Organization Warning signals, in the medical history or the physical examination, which warrant further examination, are: New onset headache 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)

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Headache with aura developed while using birth control pills New onset of head- ache in a patient with cancer or HIV infection Headache accompanied by fever Headache accompanied by neurological outcomes phrased migraine aura Progressive headache over weeks New onset headache in patients under 10 years of age or over 40 years of age Headache, which is position-dependent Physical and neurological examination is performed to rule out or confirm secondary headaches. CT/MRI scan is most often not indicated in a patient with a long history of head- ache, but should be performed if the history or physical examination is unclear or indicates that the headache is due to secondary condition.

Migraine with and Without Aura Non-pharmacological interventions are an important part of the treatment for some headache patients, although there is generally only sparse evidence for the effect of this type of intervention.

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.

A combination of triptan and NSAIDs may be more effective in some patients

than each drug alone [203].

Approximately 20–50% of patients experience recurrence of migraine within 48

h. An additional dose of triptan is usually effective in these cases.

Botulinum type A toxin (Botox) is in Denmark so far only approved for the pre- ventive treatment of chronic migraine (headache ≥15 days per month, of which at least 8 days with migraine) in patients who have shown insufficient response or intolerance to other migraine preventive drugs.

Tension-Type Headache In patients with frequent episodic and chronic tension-type headaches, the central nervous system has been shown to be hypersensitive to pain stimuli.

There is a well-documented effect of weak analgesics in the individual episodes of tension-type headaches, while the effect is often limited in chronic tension-type headache [204].

Preventive treatment may be indicated in patients with chronic tension-type headache, if there is insufficient effect of non-pharmacological treatment and when medication overuse headache is excluded [205].

Several placebo-controlled studies have shown an effect of the tricyclic antide- pressant amitriptyline [204], which is first choice for preventive treatment of chronic tension-type headache.

In episodic tension-type headache, reported pain from pericranial musculoskel- etal tissues as well as stress are likely to play an important role, while altered central pain modulation is involved in the chronic form.

In patients with chronic tension-type headache, analgesics rarely have an effect, so preventive treatment with amitriptyline, mirtazapine or venlafaxine may be indicated.

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3 Diagnosis

Cluster Headache Cluster headache is divided into two types: An episodic type seen in 80–90% of patients, where the attacks occur in bouts lasting 4–12 weeks separated by attack- free periods of varying length (weeks-years); and a chronic type seen in 10–20% of patients, with bouts lasting longer than 9 months per year.

In chronic cluster headache, in patients with an atypical presentation, onset after 40  years of age or in treatment refractory cluster headache, a cerebral MR scan should be performed to exclude tumours, midline malformations, pathology in the cavernous sinus, pituitary gland and hypothalamus [206].

Non-pharmacological treatment has not been shown to have an effect in cluster

headache [206].

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.

Medication Overuse Headache Medication overuse headache (MOH) is a chronic headache occurring at least 15 days a month in patients with pre-existing headache.

MOH is treated by withdrawal therapy (stop of the overuse of short-term medica- tion) [207], either by a complete stop of all short-term medication for a 2 months period, or by a reduced intake of short-term medication to maximum 2 days a week in average.

Danish guidelines have recommended to postpone start of preventive headache medication to the end of 2  months withdrawal therapy for two reasons: (1) The headache pattern becomes clearer during withdrawal, and a correct diagnosis would help find the best treatment option for preventive medication; (2) It seemed that some patients did not need preventives after withdrawal.

MOH should be prevented via information to patient with pre-existing headache

and a restrictive approach to prescription of short-term medication.

Secondary Types of Headache Most common in obese people Papilledema is the most prominent feature The head- ache 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 neuro- radiological 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 Investigation and treatment: see national neurological treatment guide.

Link:

http://neuro.dk/wordpress/nnbv/primaer- hjernetumor- lavgradsgliom/ Typical headache accompanied by fever and neck stiffness May present with cog- nitive impairment, photophobia or petechiae May present with seizures Investigation and treatment: see national neurological treatment guide.

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Trigeminal Neuralgia In approximately 15% of TN patients, there is an underlying symptomatic cause of pain (not including a neurovascular contact).

It is not possible to accurately identify all patients with symptomatic TN based

on pain characteristics, clinical examination or treatment response.

As TN usually has an unpredictable pattern of pain frequency and intensity, dose(s) of medical treatment should be titrated and tapered according to pain level.

At complete pain freedom lasting more than 1 month, it is advised to taper off medication by reducing e.g. carbamazepine by 100 mg or gabapentin by 300 mg every 7th–14th day (or comparable doses of other TN drugs) [208].

Titrate 100  mg every 3rd day until pain freedom or unacceptable side effects Typical maintenance dose is 100–600 mg BID Daily doses of 1800 mg or more may be necessary 2.

Approximately 30% of all TN patients do not have sufficient effect from medical

treatment or have unacceptable medical side effects.

Hormones and Migraines Women with migraines with aura should be informed that they have a slightly increased risk of an ischemic stroke in the brain, but that the risk is very small if there are no other risk factors and if they refrain from smoking and taking oestrogen- containing birth control pills.

If contraception is needed in women with migraine with aura, birth control pills with the lowest possible oestrogen content are preferred, and the patient must be informed of the increased risk of ischemic stroke.

In case of need for contraception, where a worsening of migraine without aura is experienced at the same time, the following can be tried: 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.

Children and Headaches Generally inadequate evidence in relation to both acute and preventive treatment of headache diseases in children and adolescents and there is a great need for further randomized placebo-controlled trials (RCTs).

Triptan-related side effects in children/adolescents are comparable to side effects

observed in adults.

Treatment with beta-blockers (propranolol or metoprolol) and flunarizine has a

comparable preventive effect in children and adolescents [209, 210].

With the recent development of calcitonin gene-related peptide (CGRP) antago- nist treatment, which appears to have an effect and good safety profile in adults, it can be hoped that these new types of preventive treatment also play a role in the preventive treatment of migraines in children.

Prevention with amitriptyline may have an effect on chronic tension-type head-

aches in children, but there are no placebo-controlled studies.

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3 Diagnosis

Acknowledgement A machine generated summary based on the work of Schytz, Henrik W.; Amin, Faisal M.; Jensen, Rigmor H.; Carlsen, Louise; Maarbjerg, Stine; Lund, Nunu; Aegidius, Karen; Thomsen, Lise L.; Bach, Flemming W.; Beier, Dagmar; Johansen, Hanne; Hansen, Jakob M.; Kasch, Helge; Munksgaard, Signe B.; Poulsen, Lars; Sørensen, Per Schmidt; Schmidt-Hansen, Peter T.; Cvetkovic, Vlasta V.; Ashina, Messoud; Bendtsen, Lars. 2021 in The Journal of Headache and Pain.

Migraine as a Stroke Mimic and as a Stroke Chameleon