Clusterkopfschmerz – Geballte schwerste Kopfschmerzattacken, mit Bewegungsdrang und dem Wunsch den Kopf aus dem Fenster zu halten

Die Diagnose des Clusterkopfschmerzes ist genauso wie die Behandlung in der Hand des erfahrenen Arztes keine Schwierigkeit.

Es handelt sich um in Haufen auftretende  stärkste immer dieselbe Kopfhälfte betreffende Schmerzen, die oft hinter das betroffene Auge ausstrahlen und  eine Schwellung der betroffenen Nasenhälfte mit leichtem Austritt von Nasenflüssigkeit herbeiführen. Die betroffene Wangenhälfte kann dabei zusätzlichen leicht geschwollen, gerötet oder auch überwärmt sein.

Die Kopfschmerzattacken treten in einer Abfolge zwischen ca. 15 bis 60 Minuten, häufig zur gleichen Tages- oder Nachtzeit auf. Betroffene Patienten verspüren den Wunsch herum zulaufen und kühle Frischluft einzuatmen.

Nach einigen Tagen können die Beschwerden sich bis zur nächsten Attacke spontan zurückbilden

Nikotin- und Alkoholgenuss sowie auch der Genuss scharfer Nahrungsmittel können gelegentlich bei den Betroffenen Attacken auslösen.

Ausführliche Informationen zum Krankheitsbild erfahren sie im folgenden Artikel.

 

„Zitat“

 

Clusterkopfschmerz und trigeminoautonome Kopfschmerzen

Entwicklungsstufe: S1
Stand: September 2012
AWMF-Registernummer: 030/036
Federführend
Prof. Dr. Arne May, Hamburg

a.may@uke.uni-hamburg.de

PDF-Download der Leitlinie
COI-Erklärung

Inhaltsverzeichnis

Was gibt es Neues?

  • Die bilaterale Stimulation des N. occipitalis major ist in etwa 50 % der Fälle erfolgreich bei medikamentenresistenten chronischen Clusterkopfschmerzen; alternativ kann auch die wiederholte Injektion von Kortikoiden und Lokalanästhetika an den N. occipitalis major versucht werden. Langzeiterfahrungen stehen noch aus.
  • In therapierefraktären Fällen ist eine Tiefenhirnstimulation des posterioren, inferioren Hypothalamus in etwa 50 % der Fälle erfolgreich durchgeführt worden.
  • Operative Methoden sollten nur in spezialisierten Zentren mit Kopfschmerz-Schwerpunkt durchgeführt werden.
  • Erstmalig ist in einer placebokontrollierten Studie die Wirksamkeit der Inhalation mit reinem Sauerstoff bewiesen worden.
  • Topiramat hat wahrscheinlich eine gute Wirksamkeit, valide randomisierte Studien hierzu fehlen.

Die wichtigsten Empfehlungen auf einen Blick

  • Die parenteral wirkenden 5-HT1B/D-Agonisten Sumatriptan (6 mg s. c. oder nadelfrei) und Zolmitriptan (5–10 mg nasal) sind die Substanzen mit der besten Wirksamkeit in der akuten Clusterkopfschmerzattacke. Die orale Applikation eines Triptans ist nur bei langen Attacken sinnvoll.
  • Die Inhalation von 100 % Sauerstoff über Gesichtsmaske (7–15 l/min über 15–20 min) ist bei 60–70 % der Clusterpatienten wirksam.
  • Kortikoide sind wirksam, sollten als Mittel der ersten Wahl in der Regel aber nur kurzfristig (< 4 Wochen) ­verwendet werden (z.B. Prednison mindestens 1 mg/kg KG).
  • Verapamil ist die Substanz der ersten Wahl in der prophylaktischen Behandlung des Clusterkopfschmerzes (3–6 × 80 mg/d, selten bis 960 mg/d; cave Bradykardien).
  • Lithium (Plasmaspiegel 0,6–1,2 mmol/l) und Topiramat (100–200 mg/d) sind Mittel der 2. Wahl in der prophylaktischen Behandlung des Clusterkopfschmerzes.
  • Mittel der ersten Wahl in der Behandlung der episodischen und chronischen paroxysmalen Hemikranie ist Indometacin (100–200 mg/d, häufig geringere Dosen ausreichend), Mittel der zweiten Wahl ist Gabapentin.
  • Mittel der Wahl in der Behandlung des SUNCT-Syndroms ist Lamotrigin (mindestens 100–200 mg/d).

Einführung

Die trigeminoautonomen Kopfschmerzen sind eine Gruppe von Erkrankungen, die aufgrund der Stärke der Schmerzattacken zum Suizid des Patienten führen können, wenn nicht adäquat behandelt wird. Da die Diagnose recht einfach ist und die Behandlung in den meisten Fällen klinisch ausreichend gelingt, soll diese Leitlinie helfen, die individuelle Indikation für eine wirksame Therapie argumentativ zu unterstützen.

Definition und Klinik

In der 2004 überarbeiteten Klassifikation der IHS (International Headache Society) werden die sog. trigeminoautonomen Kopfschmerzen (TAK), zusammengefasst (Headache Classification Committee of the International Headache Society 2004). Alle Kopfschmerzsyndrome dieser Gruppe haben zwei Dinge gemeinsam: die meist kurz dauernden Schmerzattacken und die nahezu obligat vorhandenen autonomen Begleitsymptome (Goadsby 1999). Die autonomen Begleitsymptome wie Lakrimation, konjunktivale Injektion, Rhinorrhö, nasale Kongestion, Hyperhidrose, Hautrötung und Lidschwellung treten streng ipsilateral zum Schmerz auf (Sjaastad 1992) und fehlen in nur 3 % der Fälle.

Nach dem aktuellen Wissensstand gehören zu den trigeminoautonomen Kopfschmerzen (Headache Classification Committee of the International Headache Society 2004):

  • der episodische und chronische Clusterkopfschmerz (CK),
  • die episodische und chronische paroxysmale Hemikranie (CPH) und
  • das SUNCT-Syndrom (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing).

Momentan nicht zu dieser Gruppe gezählt werden die Hemicrania continua und der primäre schlafgebundene Kopfschmerz (engl. hypnic headache). Bei beiden Syndromen sprechen die zirkadiane Rhythmik, das Ansprechen auf z.B. Indometacin und Ergebnisse der funktionellen Bildgebung für das Eingliedern in die Gruppe der TAK, allerdings haben beide kaum oder nur sehr geringe autonome Begleitsymptome, was das Hauptargument dafür ist, sie in der Gruppe 4 der Klassifikation zu belassen (Goadsby 1999). Die pathophysiologischen Zusammenhänge werden derzeit intensiv untersucht (May 2005). Die chronische paroxysmale Hemikranie und die episodische paroxysmale Hemikranie sprechen obligat und fast ausschließlich auf Indometacin an (Headache Classification Committee of the International Headache Society 2004).

54.1 Episodischer und ­chronischer Clusterkopfschmerz (IHS 3.1)

Epidemiologie

Die 1-Jahres-Prävalenz des Clusterkopfschmerzes liegt zwischen 0,1 % und 0,9 %. Das Verhältnis von betroffenen Männern zu Frauen beträgt 3:1. Vererbungsfaktoren sind bislang nicht bekannt, es wird jedoch eine familiäre Belastung von ca. 2–7 % angenommen.

Der Kopfschmerz beginnt im Mittel mit 28–30 Jahren, kann aber in jedem Lebensalter anfangen. Im Regelfall leiden bis zu 80 % der Patienten nach 15 Jahren noch immer an Clusterepisoden. Allerdings remittiert der Schmerz bei einigen Patienten in höherem Alter. Bei bis zu 12 % geht eine primär-episodische in eine chronische Verlaufsform über, seltener ist dies auch vice versa beschrieben.

Klinik

Der Clusterkopfschmerz ist klinisch definiert als ein attackenartig auftretender, streng einseitiger, extremster Kopfschmerz mit retroorbitalem Punctum maximum. Obligat ist das Auftreten von autonomen Symptomen (Horner-Syndrom, Lakrimation, Rhinorrhö) gleichzeitig und ipsilateral zum Schmerz nach der Klassifikation der Internationalen Kopfschmerzgesellschaft (IHS). Die Attacken treten bis zu 8-mal täglich auf, klassischerweise mit einer nächtlichen Häufung, und dauern zwischen 15 und 180 Minuten. Typischerweise berichten die Patienten eine Bewegungsunruhe („pacing around“) während der Attacken. Etwa 50 % aller Patienten haben darüber hinaus einen (häufig einseitig betonten und stetigen) Begleitkopfschmerz (Marmura et al. 2010). Bei der überwiegend vorkommenden episodischen Form des Clusterkopfschmerzes (80 %) werden die symptomatischen Episoden („bout“), die wenige Wochen bis Monate dauern, von symptomfreien Zeitspannen von Monaten bis Jahren unterbrochen. Dauert die Clusterperiode über ein Jahr ohne spontane Remission an oder sind die Remissionsphasen kürzer als 1 Monat, spricht man vom chronischen Clusterkopfschmerz. Die Attacken treten oft zur gleichen Stunde im Tagesverlauf auf, gehäuft 1–2 Stunden nach dem Einschlafen (und/oder in der ersten REM-Phase) oder in den frühen Morgenstunden (> 50 %). Ein weiterer Hinweis auf das Vorliegen einer biologischen Rhythmusstörung zeigt sich in der gehäuften Frequenz von Clusterepisoden im Frühjahr und Herbst sowie in Störungen der zirkadianen Ausschüttung vieler Hormone.

Diagnostik

Die Diagnose eines zum Formenkreis der TAKs gehörenden Kopfschmerzes beruht wie auch bei der Migräne oder dem Kopfschmerz vom Spannungstyp auf einer ausführlichen Anamnese und einer klinisch-neurologischen Untersuchung. Elektrophysiologische, laborchemische und Liquoruntersuchungen helfen nicht in der positiven Diagnosestellung. Bei der Erstdiagnose oder bei begleitenden neurologischen Ausfallerscheinungen sollten jedoch ein kranielles Computertomogramm der Schädelbasis (Knochenfenster) und eine zerebrale Kernspintomografie mit Darstellung des kraniozervikalen Übergangs durchgeführt werden (s.u.), da gerade beim Clusterkopfschmerz im höheren Lebensalter nicht selten symptomatische Ursachen vorliegen. In der Literatur werden diesbezüglich vor allem mittelliniennahe intrakranielle Raumforderungen genannt, die frontal wie auch okzipital oder sogar im Kleinhirn liegen können. Zu diesen gehören unter anderem Tumoren, arteriovenöse Malformationen, Karotisdissektionen, aber auch Hirninfarkte oder entzündliche Plaques.

Notwendige Untersuchungen:

  • neurologischer Status mit besonderer Berücksichtigung der Lokalregion und des ophthalmischen Astes des N. trigeminus
  • nur selten Neurografie: Trigeminus-SEP und/oder Blink­reflex (bei klinischem Hinweis auf Schädigung V1, wenn pathologisch, dann weitere Diagnostik)
  • Ausschluss Glaukom
  • insgesamt niedrige Schwelle zur apparativen Diagnostik

Im Einzelfall erforderliche Untersuchungen:

Beim erstmaligen Auftreten, bei auffälliger neurologischer Untersuchung, Auftreten im hohen Alter (Erstmanifestation > 60 Jahre), bei Veränderung der Symptome oder bei Veränderung des Ansprechens auf die Therapie und bei untypischer Symptomatik (Favier et al. 2007):

  • cCT der Schädelbasis (Ausschluss knochendestruierender Prozesse)
  • MRT des Zerebrums mit kraniozervikalem Übergang, ggf. MRT-Angiografie (Ausschluss einer mittelliniennahen zerebralen Pathologie, Ausschluss AVM)
  • Liquoruntersuchung (Ausschluss entzündlicher Erkrankungen)

Stationäre Aufnahme sinnvoll bei:

  • Ersteinstellung auf Sauerstofftherapie (in Fällen, in denen eine ambulante Einstellung z. B. aufgrund örtlicher Begebenheiten nicht möglich ist)
  • Erstdiagnose eines atypischen Falls
  • Versagen der prophylaktischen Therapie

Therapie

Medikamentös

Prinzipiell wird zwischen der Therapie der Einzelattacke und der Prophylaxe unterschieden

Attackenkupierung

  • Inhalation von 100 % Sauerstoff über Gesichtsmaske ­(7–15 l/min über 15–20 min)
  • 6 mg Sumatriptan s. c.
  • 5–10 mg Zolmitriptan-Nasenspray.
  • bei langen Attacken 20 mg Sumatriptan nasal
  • intranasale Applikation von Lidocain 4 %.

Die topische Anwendung von Lokalanästhetika wie auch die von Sauerstoff (Cohen et al. 2009) hilft nur einem Teil der Patienten und auch nicht immer. Trotzdem sollte jeder Patient mit Clusterkopfschmerzen einmal im Leben diese Therapien ausprobiert haben, da bei Wirksamkeit systemische Nebenwirkungen vermieden werden (May 2005). Dies ist umso wichtiger, als die Attackenfrequenz ausnahmsweise 8 und mehr Attacken pro 24 Stunden umfassen kann. Im Übrigen ist Sumatriptan Mittel der Wahl, da es parenteral verabreicht werden kann und eine oral zugeführte Medikation wegen der kurzen Attackendauer von 15–180 Minuten zu spät wirkt. Von Sumatriptan s.c. sind wahrscheinlich auch niedrigere Dosierungen als 6 mg wirksam. Sumatriptan s.c. gibt es inzwischen auch als nadelfreie Applikation.

Prophylaxe

  • Verapamil 3–4 × 80 mg, zunächst steigern bis 480 mg/d, ggf. weiter steigern (vorher und im Verlauf EKG nötig)
  • Kortikoide (Prednisolon) mindestens 1 mg/kg KG initial für 2–5 Tage, dann individuell abdosieren
  • Lithium 600–1500 mg/d (Serumspiegel 0,6–0,8 mmol/l)
  • Topiramat (100–200 mg/d), in Einzelfällen sind höhere Dosierungen nötig
  • Methysergid bis zu 12 mg/d (Medikation bis maximal 6 Monate Dauer)

Verapamil ist in der Dosierung von 3–4 × 80 mg täglich das Mittel der ersten Wahl bei episodischem und chronischem Clusterkopfschmerz (May et al. 2004). Es gibt keine valide Studie dazu, wie eindosiert werden sollte, jedoch hat sich bewährt, mit 3 × 80 mg/d zu beginnen und 1 × pro Woche um 80 mg zu steigern. In Abhängigkeit vom Therapieerfolg muss manchmal von erfahrenen Spezialisten unter kardialer Kontrolle auch höher (bis 960 mg) dosiert werden. EKG-Kontrollen sind zu Beginn und auch bei längerer subjektiv nebenwirkungsfreier Anwendung nötig (Lanteri-Minet et al. 2009). Kortikosteroide werden häufig additiv eingesetzt, z. B. im Sinne einer überbrückenden Therapie bei langsamem Wirkungseintritt von Verapamil. Vereinzelt kann eine Clusterepisode unter Kortikosteroiden abklingen. Ergotamin oder lang wirksame Triptane wie Naratriptan und Frovatriptan können in der Kurzprophylaxe (d. h., bis eine andere prophylaktische Therapie greift) abends eingesetzt werden, vor allem bei Patienten, die unter nächtlichen Attacken leiden (Siow et al. 2004). Studien hierzu sind begrenzt und schwierig durchzuführen (Pageler er al. 2011). Für Warfarin (subtherapeutische Antikoagulation) ist eine positive randomisiert-kontrollierte Pilotstudie publiziert (Hakim 2011), aus der Expertenrunde bestehen keine klinischen Erfahrungen. Einzelberichte bzw. offene Studien beschreiben einen positiven Effekt von Topiramat und Melatonin. Im Gegensatz zu anderen trigeminoautonomen Kopfschmerzen wirkt Indometacin bei Clusterkopfschmerzen nicht.

Insbesondere die Therapie des chronischen Clusterkopfschmerzes ist schwierig und benötigt häufig auch Kombinationen der oben genannten Medikamente (May et al. 2006). In diesem Fall ist meist eine Überweisung zu einer spezialisierten Kopfschmerzambulanz nötig. Bei abschätzbar bekannter Länge der aktiven Periode wird eine wirksame Prophylaxe erst dann langsam reduziert und sukzessive abgesetzt. Bei chronischen Clusterkopfschmerzen sollte etwa alle 3–6 Monate ein Versuch der Reduzierung der Medikation versucht werden.

Es gibt Hinweise, dass die i. v. Therapie mit Dihydroergotamin (9 mg in 3 Tagen z.B. über Perfusor) einen positiven Effekt sowohl beim episodischen als auch beim chronischen Clusterkopfschmerz haben kann (Magnoux u. Zlotnik 2004). Dihydroergotamin i.v., Pizotifen und Methysergid sind nur über die internationale Apotheke erhältlich.

Operative Verfahren

Erst nach Versagen aller medikamentösen Maßnahmen und sicherem Ausschluss eines symptomatischen Clusterkopfschmerzes sind in absoluten Ausnahmefällen operative Verfahren zu erwägen. Der Grund liegt darin, dass sie offenbar nicht immer wirken und nicht immer eine anhaltende Besserung der Symptomatik bewirken, jedoch die Gefahr einer zusätzlich und dann iatrogen hervorgerufenen Neuralgie des N. trigeminus oder einer Anaesthesia dolorosa bergen. In wenigen Einzelfallstudien wurde ein Effekt beschrieben durch die Applikation von Glycerol oder Lokalanästhetika in die Cisterna trigeminalis bzw. das Ganglion Gasseri, durch Hochfrequenz-Rhizotomien des Ganglion Gasseri, vaskuläre Dekompressionen, Radiation der Eintrittszone des N. trigeminus (Gamma Knife) oder Resektionen des N. petrosus superficialis major oder des Ganglion sphenopalatinum. Es gibt jedoch auch diverse Fallstudien mit negativem oder sogar verschlechtertem Ausgang. In einigen (wenigen) Fällen ist die unspezifische Blockade des N. occipitalis major erfolgreich (Ambrosini et al. 2005) und daher auf jeden Fall vor einer operativen Therapie zu versuchen. Von neurodestruierenden Verfahren wird abgeraten.

Basierend auf PET und morphometrischen Arbeiten wurde in therapierefraktären Fällen eine Tiefenhirnstimulation des posterioren, inferioren Hypothalamus diskutiert. Positive Erfahrungen liegen auch im Langzeitverlauf, in einer Studie auch doppelblind, vor (Leone et al. 2003, Franzini et al. 2003, Leone et al. 2009, Fontaine et al. 2010a, b). Sekundäre Verschlechterungen nach initialer Besserung sind beschrieben worden. Dasselbe gilt für die bilaterale Stimulation des N. occipitalis major (Burns et al. 2007, Magis et al. 2007). Zusammenfassend haben beide Verfahren eine etwa 50 %ige Chance, eine signifikante Besserung der Klinik zu erreichen. Da die Tiefenhirnstimulation ein höheres Risikoprofil hat, wird im Fall der medikamentösen Therapieresistenz die okzipitale Stimulation der Tiefenhirnstimulation vorgezogen.

Off-Label-Problematik

In zunehmendem Maße haben niedergelassene Ärzte, aber auch Spezialambulanzen Regressforderungen bei gesetzlich versicherten Patienten wegen zulassungsüberschreitender Verschreibung zu befürchten. Dies gilt insbesondere für die Behandlung seltener primärer Kopfschmerzerkrankungen. Für den Clusterkopfschmerz sind nur Sumatriptan in der parenteralen (s.c. Spritze) Applikationsform, Zolmitriptan-Nasenspray und Lithium (hier nur Quilonum) zugelassen. Alle anderen Medikamente der ersten Wahl (Verapamil, Topiramat und Methysergid) haben keine Zulassung für diese Indikation und bei ihrer Verschreibung bei gesetzlich versicherten Patienten handelt es sich um eine Off-Label-Anwendung. Die Erstattungsfähigkeit von Verapamil für die Behandlung des Clusterkopfschmerzes wird derzeit vom BfArM vorbereitet. Dihydroergotamin i.v., Pizotifen und Methysergid sind in Deutschland nicht mehr zugelassen und nur noch als Importarzneimittel zu beziehen. Zuvor sollte die Kostenübernahme mit dem zuständigen Kostenträger geklärt werden.

Für die anderen trigeminoautonomen Kopfschmerzformen ist in Deutschland kein Medikament zugelassen. Unterwww.dmkg.de werden neben den evidenzbasierten Leitlinien auch valide Publikationslisten für die einzelnen Indikationen publiziert, um gerade niedergelassenen Kollegen Argumentationshilfen im Falle eines Regresses an die Hand geben zu können.

54.2 Episodische und ­chronische ­paroxysmale Hemikranie (CPH; IHS 3.2)

Epidemiologie

Die erste Beschreibung dieses Syndroms findet sich 1976. Die Prävalenz ist sehr niedrig. Man schätzt den relativen Anteil der Patienten mit einer CPH an allen trigeminoautonomen Kopfschmerzpatienten mit etwa 3–6 %. Ähnlich dem Clusterkopfschmerz beginnt die Erkrankung zwischen dem 20. und 40. Lebensjahr. Auffallend ist jedoch die umgekehrte Geschlechterverteilung: Frauen überwiegen gegenüber Männern im Verhältnis von 3:1.

Klinik

Das plötzliche Auftreten von einseitigen attackenartigen Schmerzepisoden (paroxysmal), der Schmerzcharakter (messerstichartig-schneidend oder pulsierend), die Intensität (vernichtend) und die Lokalisation (frontoorbital oder hemikraniell) sind bei der paroxysmalen Hemikranie dem Clusterkopfschmerz sehr ähnlich. Ebenso lassen sich einzelne Schmerzepisoden nicht selten durch Triggerfaktoren wie Alkohol auslösen und werden von Lakrimation oder Injektion der Konjunktiva begleitet. Wie beim Clusterkopfschmerz wurden auch symptomatische Fälle beschrieben.

Wichtige Unterschiede zum Clusterkopfschmerz sind dagegen die kürzere Dauer einzelner Attacken (2–45 Minuten) und die höhere Häufigkeit (5–40, durchschnittlich 10 Attacken täglich). Darüber hinaus sind die autonomen Begleitsymptome oftmals weniger stark ausgeprägt. Ebenso berichten einige Patienten über die Auslösbarkeit der Schmerzepisoden durch Kopfwendung oder Druck auf die Segmente C 2/C 3.

Die für Patienten mit Clusterkopfschmerz so typische Unterteilung in aktive und inaktive Phasen findet sich auch bei der paroxysmalen Hemikranie (Headache Classification Committee of the International Headache Society 2004). Ein letztes, aber entscheidendes Unterscheidungsmerkmal zum Clusterkopfschmerz ist die Behandelbarkeit mit Indometacin. Das sichere Ansprechen der Patienten auf diese Substanz ist diagnostisch wegweisend für die paroxysmale Hemikranie oder die Hemicrania continua (Sjaastad et al. 1995). Bereits nach einer Woche (oft innerhalb von 3 Tagen) ist unter der Medikation mit einem deutlichen Rückgang der Beschwerden zu rechnen.

Therapie

  • Indometacin: 3 × 50 mg/d, ggf. erhöhen auf 300 mg, immer unter Magenschutz (z.B. Protonenpumpenhemmer)

Indometacin wird auf 3 Tagesdosen verteilt nach den Mahlzeiten eingenommen. Selten benötigen einige Patienten höhere Dosierungen bis zu 300 mg/d (wegen der kurzen Halbwertszeit von 4 Stunden häufig und kleinere Dosen einsetzen). Alle Patienten sollten insbesondere aufgrund der häufig erforderlichen Dauertherapie einen Magenschutz mit Protonenpumpenhemmern kombinieren. Für gewöhnlich wird nach Sistieren der Schmerzen die Dosis reduziert, bis es zu einem Wiederauftreten der Schmerzen kommt, so kann eine sog. Erhaltungsdosis gefunden werden.

Alternativ können andere NSAIDs, z.B. Naproxen oder Diclofenac, versucht werden. In der Literatur existieren darüber hinaus Berichte über die vereinzelte Wirkung von Gabapentin, Verapamil und Acetazolamid.

54.3 Short-lasting uniform neuralgiform headache with conjunctival injection and tearing (SUNCT; IHS 3.3)

Epidemiologie

Daten zur Prävalenz und zur geschlechtlichen Verteilung für dieses Syndrom zu erheben, gestaltet sich bei der niedrigen Fallzahl von Patienten als äußerst schwierig. Es handelt sich um eine extrem seltene Kopfschmerzerkrankung. Das Verhältnis Frauen zu Männern wird mit 1:4 geschätzt.

Klinik

Die Bezeichnung dieses Kopfschmerzsyndroms beschreibt bereits die wesentlichen klinischen Charakteristika. Patienten mit der Diagnose eines SUNCT klagen über extrem kurz dauernde (15 Sekunden bis 2 Minuten) einschießende Attacken neuralgiformen Schmerzcharakters heftigster und nicht selten vernichtender Intensität. Die Attacken treten durchschnittlich bis zu 60-mal täglich auf (gelegentlich sogar bis zu 200-mal täglich) und sind streng einseitig periorbital. Wie alle TAKs geht das SUNCT mit autonomen Begleitsymptomen einher, jedoch beschränken sie sich im Allgemeinen auf die konjunktivale Injektion und die obligatorisch ausgeprägte Lakrimation. Auch beim SUNCT-Syndrom gibt es eine episodische und eine chronische Verlaufsform. Symptomatische Formen sind auszuschließen.

Unterschiede zum Clusterkopfschmerz sind die wesentlich höhere Attackenfrequenz, die kürzere Dauer einzelner Schmerzattacken und der neuralgiforme Charakter der Schmerzen. Bei der klassischen Trigeminusneuralgie, die differenzialdiagnostisch ausgeschlossen werden muss, ist die Attackenfrequenz der ebenfalls elektrisierend einschießenden Schmerzepisoden in der Regel noch höher (bis zu mehreren hundert Mal täglich) und es fehlen die autonomen Begleitsymptome. Bei der Trigeminusneuralgie kommt es häufiger zur Triggerung der Attacken durch Kauen, Sprechen oder Kälte. Im Gegensatz zum SUNCT-Syndrom betrifft die Trigeminusneuralgie bevorzugt den zweiten und dritten trigeminalen Ast allein oder in Kombination. Da symptomatische Fälle nicht selten sind, ist eine einmalige MRT-Diagnostik sinnvoll.

Therapie

Derzeit ist eine wirksame Therapie nicht bekannt. Die bei der CPH oder Hemicrania continua erfolgreich angewandte Substanz Indometacin ist nicht wirksam. Studien zur Behandelbarkeit existieren nicht, lediglich einzelne Fallberichte in der Literatur berichten vereinzelte Erfolge durch die Gabe von Lamotrigin, Gabapentin, Carbamazepin/Oxacarbazepin und Topiramat, zum Teil in Kombination (Pareja et al. 2002). In letzter Zeit häufen sich Einzelfallbeschreibungen zur Wirksamkeit von Lamotrigin, sodass ein Therapieversuch mit dieser Substanz vielversprechend erscheint (D’Andrea et al. 2001, Matharu et al. 2003). In Fallserien war auch die intravenöse Gabe von Lidocain erfolgreich in der Behandlung des SUNCT, diese sollte allerdings nur unter Observationsbedingungen durchgeführt werden.

Selbsthilfegruppen

http://ck-wissen.de/
www.clusterkopfschmerzen.net/
www.clusterkopf.de

Redaktionskomitee

Für die DGN:
Prof. Dr. Andreas Straube, Neurologie, Universitätsklinik München

Für die Schweiz:
Dr. Andreas Gantenbein, Neurologie, Universitätsspital Zürich

Für Österreich:
Univ.-Prof. Christian Wöber, Universitätsklinik für ­Neurologie, Wien

Für die DMKG:
Prof Dr. Arne May, Institut für Systemische Neurowissenschaften, Hamburg (UKE), Universitätsklinikum Hamburg-Eppendorf
Prof. Dr. Stefan Evers, Neurologie, Universitätsklinik Münster

Für den BDN:
Dr. V. Malzacher, Neurologische Praxis, Reutlingen

Federführend: Prof. Dr. Arne May, Institut für Systemische Neurowissenschaften, Hamburg (UKE), Martinistraße 52, 20246 Hamburg, E-Mail: a.may@uke.uni-hamburg.de

Entwicklungsstufe der Leitlinie: S1

Zusammensetzung der Leitliniengruppe

Die Auswahl des federführenden Autors sowie der weiteren Mitglieder der Leitliniengruppe erfolgte durch die Leitlinienkommission der DGN.

Recherche und Auswahl der wissenschaftlichen Belege

Die Literaturauswahl erfolgte mittels PubMed mit den Suchbegriffen: cluster headache, SUNCT syndrome, paroxaysmal hemicrania, CH, SUNCT, CPH, EPH, diagnostic procedure, treatment, prevention, symptoms. Die entsprechenden Publikationen wurden nach Evidenzgraden eingeteilt und berücksichtigt.

Verfahren zur Konsensfindung

Zur Konsensusfindung wurde ein modifiziertes Delphi-Verfahren angewendet. Diese Leitlinie entstand ohne Einflussnahme oder Unterstützung durch die Industrie.

Literatur

  • Ambrosini A, Vandenheede M, Rossi P et al. Suboccipital injection with a mixture of rapid- and long-acting steroids in cluster headache: a double-blind placebo-controlled study. Pain 2005; 118: 92–96
  • Burns B, Watkins L, Goadsby PJ. Treatment of medically intractable cluster headache by occipital nerve stimulation: long-term follow-up of eight patients. Lancet 2007; 369: 1099–1106
  • Cohen AS, Burns B, Goadsby PJ. High-flow oxygen for treatment of cluster headache: a randomized trial. J Am Mes Ass 2009; 302: 2451–2457
  • D’Andrea G, Granella F, Ghiotto N et al. Lamotrigine in the treatment of SUNCT syndrome. Neurology 2001; 57: 1723–1725
  • Favier I, van Vliet JA, Roon KI et al. Trigeminal autonomic cephalgias due to structural lesions: a review of 31 cases. Arch Neurol 2007; 64: 25–31
  • Fontaine D, Lanteri-Minet M, Ouchchane L et al. Anatomical location of effective deep brain stimulation electrodes in chronic cluster headache. Brain 2010a; 133: 1214–1223
  • Fontaine D, Lazorthes Y, Mertens P et al. Safety and efficacy of deep brain stimulation in refractory cluster headache: a randomized placebo-controlled double-blind trial followed by a 1-year open extension. J Headache Pain 2010b; 11: 23–31
  • Franzini A, Ferroli P, Leone M et al. Stimulation of the posterior hypothalamus for treatment of chronic intractable cluster headaches: first reported series. Neurosurgery 2003; 52: 1095–1101
  • Goadsby PJ. Short-lasting primary headaches: focus on trigeminal automatic cephalgias and indomethacin-sensitive headaches. Curr Opin Neurol 1999; 12: 273–277
  • Hakim SM. Warfarin for refractory chronic cluster headache: a randomized pilot study. Headache 2011; 51: 713–725
  • Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 2nd ed. Cephalalgia 2004; 24: 1–160
  • Lanteri-Minet M, Silhol F, Piano V et al. Cardiac safety in cluster headache patients using the very high dose of verapamil (≥ 720 mg/day). J Headache Pain 2011; 12: 173–176
  • Leone M, Franzini A, Broggi G et al. Hypothalamic deep brain stimulation for intractable chronic cluster headache: a 3-year follow-up. Neurol Sci 2003; 24 (Suppl. 2): S143–S145
  • Leone M, Franzini A, Cecchini AP et al. Cluster headache: pharmacological treatment and neurostimulation. Nat Clin Pract Neurol. 2009; 5: 153–162
  • Magis D, Allena M, Bolla M et al. Occipital nerve stimulation for drug-resistant chronic cluster headache: a prospective pilot study. Lancet Neurology 2007; 6: 314–321
  • Magnoux E, Zlotnik G. Outpatient intravenous dihydroergotamine for refractory cluster headache. Headache 2004; 44: 249–255
  • Marmura MJ, Pello SJ, Young WB. Interictal pain in cluster headache. Cephalalgia 2010; 30: 1531–1534
  • Matharu MS, Cohen AS, Boes CJ et al. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review. Curr Pain Headache Rep 2003; 7: 308–318
  • May A. Cluster headache: pathogenesis, diagnosis, and management. Lancet 2005; 366: 843–855
  • May A, Evers S, Straube A et al. Therapie und Prophylaxe von Cluster-Kopfschmerzen und anderen trigemino-autonomen Kopfschmerzen. Überarbeitete Empfehlungen der Deutschen Migräne- und Kopfschmerzgesellschaft. Nervenheilkunde 2004; 23: 478–490
  • May A, Leone M, Afra J et al. EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias. Eur J Neurol 2006; 13: 1066–1077
  • Pageler L, Katsarava Z, Lampl C et al. Frovatriptan for prophylactic treatment of cluster headache: lessons for future trial design. Headache 2011; 51: 129–134
  • Pareja JA, Caminero AB, Sjaastad O. SUNCT syndrome: diagnosis and treatment. CNS Drugs 2002; 16: 373–383
  • Siow HC, Pozo-Rosich P, Silberstein SD. Frovatriptan for the treatment of cluster headaches. Cephalalgia 2004; 24: 1045–1048
  • Sjaastad O, ed. Cluster Headache Syndrome, vol. 23. London: W. B. Saunders; 1992
  • Sjaastad O, Stovne LJ, Stolt Nielsen A et al. CPH and hemicrania continua: requirements of high indomethacin dosages – an ominous sign? Headache 1995; 35: 363–367
  • Zebenholzer K, Wöber C, Vigl M et al. Eletriptan for the short-term prophylaxis of cluster headache. Headache 2004; 44: 361–364

Aus: Hans-Christoph Diener, Christian Weimar (Hrsg.): Leitlinien für Diagnostik und Therapie in der Neurologie, Herausgegeben von der Kommission „Leitlinien“ der Deutschen Gesellschaft für Neurologie, Thieme Verlag, Stuttgart, September 2012

 

5.530 Gedanken zu „Clusterkopfschmerz – Geballte schwerste Kopfschmerzattacken, mit Bewegungsdrang und dem Wunsch den Kopf aus dem Fenster zu halten

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