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Cluster headache and trigeminal autonomic headache

What's new?

  • The Hemicrania Continua and the SUNA syndrome (short lasting neuralgiform attacks with autonomic symptoms) now belong to the group of trigeminal autonomic headaches
  • The stimulation of the sphenopalatine ganglion (SPG) in cluster headache patients was able to show in a multicenter, randomized study that in over 70% of the patients either the acute attack was docked or the attack frequency decreased or both took place. Almost all patients experienced side effects from the operation (mild to moderate hypoesthesia of the maxillary nerve for up to 3 months)
  • Bilateral stimulation of the greater occipital nerve is successful in about 50% of cases in drug-resistant chronic cluster headaches; the rate of side effects is relatively high and therefore not approved for this indication. Alternatively, repeated injections of corticosteroids and local anesthetics into the greater occipital nerve can be attempted. Long-term experience is still pending.
  • Surgical methods should only be performed in specialized centers that focus on headaches.
  • The effectiveness of inhalation with pure oxygen for attack docking has been proven in a placebo-controlled study for cluster headaches.

The most important recommendations at a glance

  • The parenterally acting 5-HT1B / D agonists sumatriptan (6 mg s.c.) and zolmitriptan (5–10 mg nasal) are the substances with the best effectiveness in acute cluster headache attacks. Oral administration of a triptan only makes sense in a few patients.
  • The inhalation of 100% oxygen through a face mask with a rebreather bag (non-rebreather mask) of 12 l / min for 15-20 min is effective in 78% of cluster patients. The costs for the treatment of cluster headache attacks with oxygen in pressurized gas cylinders are covered by the statutory health insurance companies.
  • Corticoids are effective, but should generally only be used for a short time (<4 weeks) as the first choice (e.g. prednisone at least 1 mg / kg body weight).
  • Verapamil is the substance of first choice in the prophylactic treatment of cluster headaches (3–6 × 80 mg / d, rarely up to 960 mg / d; beware of bradycardia and AV block.
  • Lithium (plasma level: 0.6–1.2 mmol / l) and topiramate (100–200 mg / d) are second choice agents in the prophylactic treatment of cluster headache.
  • The agent of first choice in the treatment of episodic and chronic paroxysmal hemicrania and the hemicrania continua is indomethacin (100-200 mg / d, often lower doses are sufficient), the agent of the second choice is gabapentin or celecoxib.
  • The agent of choice in the treatment of the SUNCT syndrome is lamotrigine (at least 100–200 mg / d).

Introduction: scope and purpose of the guideline

  • Justification of the need for an LL
    Trigeminal autonomic headaches are a group of diseases which, due to the severity of the pain attacks, can lead to the patient's suicide if not adequately treated. Since the diagnosis is quite simple and the treatment is clinically sufficient in most cases, this guideline is intended to help provide arguments to support the individual indication for an effective therapy.
  • Objectives of the guideline
    This guideline provides information about the clinic, the diagnosis and the therapeutic procedure for patients diagnosed with trigeminal autonomic headache syndrome.
  • Patient target group
    Anyone with trigeminal autonomic headache syndrome.
  • Supply area
    Outpatient and inpatient care in the field of headache diagnosis and therapy.
  • Addressees of the guideline
    Resident neurologists, neurologists in acute clinics and rehabilitation facilities, experts.
  • keywords
    Headache, trigeminoautonomous, cluster headache, SUNCT syndrome, paroxysmal hemicrania. ICD 10: G44.0.

Definition and clinic

In the classification of the International Headache Society (IHS), which was revised in 2013, the so-called trigeminal autonomic headaches (TAK) are summarized (Headache Classification Committee of the International Headache Society 2013, ICHD-3 Beta). All headache syndromes in this group have two things in common: the mostly short-lasting pain attacks and the almost obligatory accompanying autonomic symptoms (Goadsby 1999). The autonomic accompanying symptoms such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, hyperhidrosis, skin reddening and eyelid swelling occur strictly ipsilateral to the pain (Sjaastad 1992) and are absent in only 3% of cases.

According to the ICHD 3 beta, trigeminal autonomic headaches include:

  • the episodic and chronic cluster headache (CK)
  • episodic and chronic paroxysmal hemicrania (CPH)
  • the Hemicrania Continua (HC)
  • the SUNCT syndrome (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing).
  • the SUNA syndrome (Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms)

The SUNCT syndrome is probably a sub-form of the SUNA, but was first described and examined and is therefore still listed as a separate syndrome. The primary sleep-related headache (hypnic headache) is not currently included in this group. The circadian rhythm, the response to e.g. indomethacin or verapamil and the results of the functional imaging speak for integration into the group of TAK. However, the primarily sleep-related headache has hardly any or only very few autonomous accompanying symptoms, which was the main argument for keeping it in group 4 of the classification (Goadsby 1999). The pathophysiological relationships are currently being intensively investigated (May 2005). Chronic and episodic paroxysmal hemicrania respond obligatorily and almost exclusively to indomethacin (Headache Classification Committee of the International Headache Society 2013, ICHD-3 Beta).

Episodic and chronic cluster headache (IHS 3.1)

The cluster headache is clinically defined as an attack-like, strictly unilateral, extremely violent headache with a retroorbital punctum maximum, which affects men and women in a ratio of 3: 1. The occurrence of autonomic symptoms (Horner's syndrome, lacrimation, rhinorrhea) at the same time and ipsilateral to the pain is obligatory. Pathological sweating in the forehead or face area, reddening in the forehead or face area and a feeling of fullness in the ear can also occur. The attacks occur up to 8 times a day, traditionally with an accumulation at night, and last between 15 and 180 minutes. Typically, the patients report pronounced agitation (pacing around) during the attacks, which is now one of the diagnostic criteria. About 50% of all patients also have a (often one-sided and constant) accompanying headache (Marmura et al. 2010). Migraine-like symptoms such as aura, nausea, phonophobia and photophobia can also occur (Schürks et al. 2006). In the predominantly occurring episodic form of cluster headache (80%), the symptomatic episodes (bout), which last a few weeks to months, are interrupted by symptomatic periods of months to years. If the cluster period lasts for over a year without spontaneous remission or if the remission phases are shorter than 1 month, this is referred to as chronic cluster headache. The attacks often occur at the same hour during the day, more frequently 1–2 hours after falling asleep (and / or in the first REM phase) or in the early hours of the morning (> 50%). Another indication of the presence of a biological rhythm disturbance is shown in the increased frequency of cluster episodes in spring and autumn as well as disturbances in the circadian secretion of many hormones.