Seltene primäre Kopfschmerzen

Primäre Kopfschmerzen werden in häufige und seltene Kopfschmerzsyndrome eingeteilt. Seltene primäre Kopfschmerzen treten in der Regel bei weniger 1% als der Bevölkerung auf. Die Diagnose eines primären Kopfschmerzes kann immer nur gestellt werden, wenn durch Diagnostik eine sekundäre Ursache ausgeschlossen werden kann.

Kernaussagen

• Seltene primäre Kopfschmerzen werden von den häufigen primären Kopfschmerzen wie Migräne, Kopfschmerz vom Spannungstyp und Clusterkopfschmerzen differenziert.

• Bei seltenen primären Kopfschmerzen muss grundsätzlich durch Zusatzdiagnostik eine sekundäre Ursache ausgeschlossen werden.

• Der Hustenkopfschmerz wird durch Husten, Pressen oder ein Valsalva-Manöver ausgelöst.

• Der primäre Anstrengungskopfschmerz wird durch körperliche Aktivität ausgelöst. Er kann erfolgreich mit Indometacin behandelt werden.

• Kopfschmerzen bei sexueller Aktivität können während sexueller Erregung oder während des Orgasmus auftreten.

• Bei den meisten Patienten mit Donnerschlagkopfschmerz handelt es sich wahrscheinlich um eine sekundäre Ursache. Bevor diese Diagnose als primärer Kopfschmerz gestellt wird, ist eine umfangreiche diagnostische Abklärung notwendig.

• Beim kältebedingten Kopfschmerz sollten kalte Speisen und Getränke vermieden werden.

• Der Kopfschmerz durch Einwirkung von Druck oder Zug auf den Kopf tritt als Folge einer anhaltenden Kompression oder Zug auf perikranielle Weichteile auf.

• Beim primär stechenden Kopfschmerz dauern die Kopfschmerzen meist nur einige Sekunden.

• Beim Münzkopfschmerz ist das schmerzhafte Areal in etwa so groß wie eine Münze.

• Der schlafgebundene Kopfschmerz spricht gut auf Koffein an.

• Der neu aufgetretene tägliche Kopfschmerz ist schwierig zu behandeln. Es gibt keine randomisierten placebokontrollierte Studien.

Schlüsselwörter

Kopfschmerzsyndrome - seltene Krankheiten - Schmerzmedizin - Hustenkopfschmerz - Anstrengungskopfschmerz

Publication History

Article published online:
05 June 2024

• Literatur

• 1 Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders ICHD-3, 3rd edition. Cephalalgia 2018; 38: 1-211
• 2 Nandyala A, Zhang N. Primary cough headache. Curr Pain Headache Rep 2023; 27: 679-684
• 3 Colombo B, Filippi M. Other primary headaches. Cough headache, nummular headache and primary exercise headache: a secondary point of view. Neurol Sci 2020; 41: 377-379
• 4 Ekbom K. Cough headache. In: Rose FC. Headache handbook of clinical neurology. Amsterdam: Elsevier; 1986: 367-371
• 5 Rasmussen BK, Olesen J. Symptomatic and nonsymptomatic headaches in a general population. Neurology 1992; 42: 1225-1231
• 6 Pascual J, Gonzalez-Mandly A, Martin R. et al. Headaches precipitated by cough, prolonged exercise or sexual activity: a prospective etiological and clinical study. J Headache Pain 2008; 9: 259-266
• 7 Mathew NT. Indomethacin responsive headache syndromes. Headache 1981; 21: 147-150
• 8 Chen PK, Fuh JL, Wang SJ. Cough headache: a study of 83 consecutive patients. Cephalalgia 2009; 29: 1079-1085
• 9 Wang SJ, Fuh JL, Lu SR. Benign cough headache is responsive to acetazolamide. Neurology 2000; 55: 149-150
• 10 Cordenier A, De Hertogh W, De Keyser J. et al. Headache associated with cough: a review. J Headache Pain 2013; 14: 42
• 11 Lipton RB, Lowenkopf T, Bajwa ZH. et al. Cardiac cephalgia: a treatable form of exertional headache. Neurology 1997; 49: 813-816
• 12 Pascual J, Iglesias F, Oterino A. et al. Cough, exertional, and sexual headaches: an analysis of 72 benign and symptomatic cases. Neurology 1996; 46: 1520-1524
• 13 González-Quintanilla V, Madera J, Pascual J. Update on headaches associated with physical exertion. Cephalalgia 2023; 43: 3331024221146989
• 14 Upadhyaya P, Nandyala A, Ailani J. Primary exercise headache. Curr Neurol Neurosci Rep 2020; 20: 9
• 15 Alvarez R, Ramón C, Pascual J. Clues in the differential diagnosis of primary vs secondary cough, exercise, and sexual headaches. Headache 2014; 54: 1560-1562
• 16 Sandoe CH, Kingston W. Exercise headache: a review. Curr Neurol Neurosci Rep 2018; 18: 28
• 17 Lance JW. Headaches related to sexual activity. J Neurol Neurosurg Psychiatry 1976; 39: 1226-1230
• 18 Yeh YC, Fuh JL, Chen SP. et al. Clinical features, imaging findings and outcomes of headache associated with sexual activity. Cephalalgia 2010; 30: 1329-1335
• 19 Lin P-T, Chen S-P, Wang S-J. Update on primary headache associated with sexual activity and primary thunderclap headache. Cephalalgia 2023; 43
• 20 Frese A, Eikermann A, Frese K. et al. Headache associated with sexual activity: Demography, clinical features, and comorbidity. Neurology 2003; 61: 796-800
• 21 Frese A, Rahmann A, Gregor N. et al. Headache associated with sexual activity: prognosis and treatment options. Cephalalgia 2007; 27: 1265-1270
• 22 Rasmussen BK, Olesen J. Symptomatic and nonsymptomatic headaches in a general population. Neurology 1992; 42: 1225-1231
• 23 Lui JL, Shaw NM, Abbasi B. et al. Adverse reactions of PDE5 inhibitors: an analysis of the World Health Organization pharmacovigilance database. Andrology 2023; 11: 1408-1417
• 24 Ściślicki P, Sztuba K, Klimkowicz-Mrowiec A. et al. Headache associated with sexual activity-a narrative review of literature. Medicina (Kaunas) 2021; 57: 735
• 25 Bahra A. Other primary headaches-thunderclap-, cough-, exertional-, and sexual headache. J Neurol 2020; 267: 1554-1566
• 26 Frese A, Gantenbein A, Marziniak M. et al. Triptans in orgasmic headache. Cephalalgia 2006; 26: 1458-1461
• 27 Day JW, Raskin NH. Thunderclap headache: symptom of unruptured cerebral aneurysm. Lancet 1986; 2: 1247-1248
• 28 Velez A, McKinney JS. Reversible cerebral vasoconstriction syndrome: a review of recent research. Curr Neurol Neurosci Rep 2013; 13: 319
• 29 Yang CW, Fuh JL. Thunderclap headache: an update. Expert Rev Neurother 2018; 18: 915-924
• 30 Dilli E. Thunderclap headache. Curr Neurol Neurosci Rep 2014; 14: 437
• 31 García-Azorín D, González-García N, Abelaira-Freire J. et al. Management of thunderclap headache in the emergency room: a retrospective cohort study. Cephalalgia 2021; 41: 711-720
• 32 Long D, Koyfman A, Long B. The thunderclap headache: approach and management in the emergency department. J Emerg Med 2019; 56: 633-641
• 33 Schwaiger J, Kiechl S, Seppi K. et al. Prevalence of primary headaches and cranial neuralgias in men and women aged 55–94 years (Bruneck Study). Cephalalgia 2009; 29: 179-187
• 34 Sekhon S, Sharma R, Cascella M. Thunderclap headache. Treasure Island, FL: StatPearls; 2023.
• 35 Chen SP, Fuh JL, Lirng JF. et al. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder?. Neurology 2006; 67: 2164-2169
• 36 Chebini A, Dilli E. Cold stimulus headache. Curr Neurol Neurosci Rep 2019; 19: 46
• 37 Kraya T, Schulz-Ehlbeck M, Burow P. et al. Prevalence and characteristics of headache attributed to ingestion or inhalation of a cold stimulus (HICS): a cross-sectional study. Cephalalgia 2020; 40: 299-306
• 38 Bonemazzi I, Pelizza MF, Berti G. et al. Cold-stimulus headache in children and adolescents. Life (Basel) 2023; 13: 973
• 39 Zierz AM, Mehl T, Kraya T. et al. Ice cream headache in students and family history of headache: a cross-sectional epidemiological study. J Neurol 2016; 263: 1106-1110
• 40 Khoo A, Kiley M, Goadsby PJ. Managing external cold-stimulus headache with preventive naproxen. Cephalalgia Rep 2020; 3: 2515816320915696
• 41 Krymchantowski AV. Headaches due to external compression. Curr Pain Headache Rep 2010; 14: 321-324
• 42 Krymchantowski A, Barbosa JS, Cvaigman M. et al. Helmet-related, external compression headache among police officers in Rio de Janeiro. MedGenMed 2004; 6: 45
• 43 Jafari E, Togha M, Kazemizadeh H. et al. Evaluation of headache associated with personal protective equipment during COVID-19. Brain Behav 2021; 11: e2435
• 44 Oliveira R, Plácido M, Pereira L. et al. Headaches and the use of personal protective equipment in the general population during the COVID-19 pandemic. Cephalalgia 2022; 42: 608-617
• 45 Fuh JL, Kuo KH, Wang SJ. Primary stabbing headache in a headache clinic. Cephalalgia 2007; 27: 1005-1009
• 46 Kim DY, Lee MJ, Choi HA. et al. Clinical patterns of primary stabbing headache: a single clinic-based prospective study. J Headache Pain 2017; 18: 44
• 47 Murray D, Dilli E. Primary stabbing headache. Curr Neurol Neurosci Rep 2019; 19: 47
• 48 Wang SJ, Fuh JL. The "other" headaches: primary cough, exertion, sex, and primary stabbing headaches. Curr Pain Headache Rep 2010; 14: 41-46
• 49 Raskin NH, Schwartz RK. Icepick-like pain. Neurology 1980; 30: 203-205
• 50 Summ O, Andreou AP, Akerman S. et al. Differential actions of indomethacin: clinical relevance in headache. Pain 2021; 162: 591-599
• 51 Pareja JA, Ruiz J, Deisla C. et al. Idiopathic stabbing headache (jabs and jolts syndrome). Cephalalgia 1996; 16: 93-96
• 52 Pareja JA, Caminero AB, Serra J. et al. Nummular headache: a coin-shaped cephalgia. Neurology 2002; 58: 1678-1679
• 53 Clar-de-Alba B, Barriga FJ, Rodríguez-Caravaca G. Clinical and pathophysiological description of nummular headaches: a case series. Rev Neurol 2020; 70: 171-178
• 54 Rammohan K, Mundayadan SM, Mathew R. Nummular headache: clinico-epidemiological features in South Indian population. J Neurosci Rural Pract 2016; 7: 532-536
• 55 Patel UK, Saleem S, Anwar A. et al. Characteristics and treatment effectiveness of the nummular headache: a systematic review and analysis of 110 cases. BMJ Neurol Open 2020; 2: e000049
• 56 Martins IP, Abreu L. Nummular headache: clinical features and treatment response in 24 new cases. Cephalalgia Rep 2018; 1: 2515816318802771
• 57 Schwartz DP, Robbins MS, Grosberg BM. Nummular headache update. Curr Pain Headache Rep 2013; 17: 340
• 58 García-Iglesias C, González-Celestino A, Sierra Mencía Á. et al. Long-term outcomes of nummular headache: a series of 168 patients and 1198 patient-years of follow-up. Cephalalgia 2023; 43: 3331024231201576
• 59 Pellesi L, Cevoli S, Favoni V. et al. Nummular headache: a gender-oriented perspective on a case series from the RegistRare Network. Neurol Sci 2020; 41: 583-589
• 60 Wilhour D, Ceriani CEJ, Nahas SJ. Nummular headache. Curr Neurol Neurosci Rep 2019; 19: 26
• 61 Trigo J, García-Azorín D, Martinez Pias E. et al. Clinical characteristics of nummular headache and differentiation between spontaneous and posttraumatic variant: an observational study. J Headache Pain 2019; 20: 34
• 62 García-Iglesias C, Puledda F, Echavarría-Íñiguez A. et al. Treatment of primary nummular headache: a series of 183 patients from the NUMITOR study. J Clin Med 2022; 12: 122
• 63 García-Azorín D, Trigo-López J, Sierra Á. et al. Observational, open-label, non-randomized study on the efficacy of onabotulinumtoxin A in the treatment of nummular headache: the pre-numabot study. Cephalalgia 2019; 39: 1818-1826
• 64 Jiang L, Li M, Liu Q. et al. Nummular headache: 2 cases with good beta blocker responses and a narrative review. Headache 2019; 59: 593-602
• 65 Cuadrado ML, López-Ruiz P, Guerrero Á L. Nummular headache: an update and future prospects. Expert Rev Neurother 2018; 18: 9-19
• 66 Silva-Neto RP, Santos P, Peres MFP. Hypnic headache: a review of 348 cases published from 1988 to 2018. J Neurol Sci 2019; 401: 103-109
• 67 Evers S GP. Hypnic headache: clinical features, pathophysiology, and treatment. Neurology 2003; 60: 905-909
• 68 Ferretti A, Velardi M, Fanfoni C. et al. Pediatric hypnic headache: a systematic review. Front Neurol 2023; 14: 1254567
• 69 Melchior AG, Ayyoub A, Christensen RH. et al. Epidemiology and clinical features of hypnic headache: a systematic review and meta-analysis. Cephalalgia 2023; 43
• 70 Eliasson JH, Scher AI, Buse DC. et al. The prevalence of hypnic headache in Iceland. Cephalalgia 2020; 40: 863-865
• 71 Holle D, Wessendorf TE, Zaremba S. et al. Serial polysomnography in hypnic headache. Cephalalgia 2011; 31: 286-290
• 72 Burish MJ, Chen Z, Yoo S-H. Emerging relevance of circadian rhythms in headaches and neuropathic pain. Acta Physiologica 2019; 225: e13161
• 73 Holle D, Naegel S, Krebs S. et al. Hypothalamic gray matter volume loss in hypnic headache. Ann Neurol 2011; 69: 533-539
• 74 Naegel S, Huhn JI, Gaul C. et al. No Pattern alteration in single nocturnal melatonin secretion in patients with hypnic headache: a case-control study. Headache 2017; 57: 648-653
• 75 Diener HC, Obermann M, Holle D. Hypnic headache: clinical course and treatment. Curr Treat Options Neurol 2012; 14: 15-26
• 76 Lipton RB, Diener HC, Robbins MS. et al. Caffeine in the management of patients with headache. J Headache Pain 2017; 18: 107
• 77 Dissanayake KP, Wanniarachchi DP, Ranawaka UK. Case report of hypnic headache: a rare headache disorder with nocturnal symptoms. BMC Res Notes 2017; 10: 318-318
• 78 Lindner D, Scheffler A, Nsaka M. et al. Hypnic headache - what do we know in 2022?. Cephalalgia 2023; 43: 3331024221148659
• 79 Zduńska A, Cegielska J, Zduński S. et al. Caffeine for headaches: helpful or harmful? A brief review of the literature. Nutrients 2023; 15: 3170
• 80 Holle D, Naegel S, Krebs S. et al. Clinical characteristics and therapeutic options in hypnic headache. Cephalalgia 2010; 30: 1435-1442
• 81 Liang JF, Wang SJ. Hypnic headache: a review of clinical features, therapeutic options and outcomes. Cephalalgia 2014; 34: 795-805
• 82 Kesserwani H. Hypnic headache responds to topiramate: a case report and a review of mechanisms of action of therapeutic agents. Cureus 2021; 13: e13790
• 83 Palacios-Ceña D, Talavera B, Gómez-Mayordomo V. et al. The day my life changed: a qualitative study of the experiences of patients with new daily persistent headache. Headache 2020; 60: 124-140
• 84 Li D, Rozen TD. The clinical characteristics of new daily persistent headache. Cephalalgia 2002; 22: 66-69
• 85 Uniyal R, Paliwal VK, Tripathi A. Psychiatric comorbidity in new daily persistent headache: a cross-sectional study. Eur J Pain 2017; 21: 1031-1038
• 86 Castillo J, Munoz P, Guitera V. et al. Epidemiology of chronic daily headache in the general population. Headache 1999; 39: 190-196
• 87 Grande RB, Aaseth K, Lundqvist C. et al. Prevalence of new daily persistent headache in the general population. The Akershus study of chronic headache. Cephalalgia 2009; 29: 1149-1155
• 88 Yamani N, Olesen J. New daily persistent headache: a systematic review on an enigmatic disorder. J Headache Pain 2019; 20: 80
• 89 Papetti L, Sforza G, Tarantino S. et al. Features and management of new daily persistent headache in developmental-age patients. Diagnostics (Basel) 2021; 11: 385
• 90 Reidy BL, Riddle EJ, Powers SW. et al. Clinic-based characterization of continuous headache in children and adolescents: comparing youth with chronic migraine to those with new daily persistent headache. Cephalalgia 2020; 40: 1063-1069
• 91 Cheema S, Mehta D, Ray JC. et al. New daily persistent headache: a systematic review and meta-analysis. Cephalalgia 2023; 43: 3331024231168089
• 92 Meineri P, Torre E, Rota E. et al. New daily persistent headache: clinical and serological characteristics in a retrospective study. Neurol Sci 2004; 25: S281-282
• 93 Riddle EJ, Smith JH. New daily persistent headache: a diagnostic and therapeutic odyssey. Curr Neurol Neurosci Rep 2019; 19: 21
• 94 Liu J, de Luca RD, Mello Neto HO. et al. Post-COVID-19 Syndrome? New daily persistent headache in the aftermath of COVID-19. Arq Neuropsiquiatr 2020; 78: 753-754
• 95 Sampaio Rocha-Filho PA. Headache associated with COVID-19: epidemiology, characteristics, pathophysiology, and management. Headache 2022; 62: 650-656
• 96 Rozen TD. Daily persistent headache after a viral illness during a worldwide pandemic may not be a new occurrence: Lessons from the 1890 Russian/Asiatic flu. Cephalalgia 2020; 40: 1406-1409
• 97 Rozen TD. Triggering events and new daily persistent headache: age and gender differences and insights on pathogenesis – a clinic-based study. Headache 2016; 56: 164-173
• 98 Rozen T, Swidan SZ. Elevation of CSF tumor necrosis factor alpha levels in new daily persistent headache and treatment refractory chronic migraine. Headache 2007; 47: 1050-1055
• 99 Peng KP, Rozen TD. Update in the understanding of new daily persistent headache. Cephalalgia 2023; 43: 3331024221146314
• 100 Pomeroy JL, Marmura MJ, Nahas SJ. et al. Ketamine infusions for treatment refractory headache. Headache 2017; 57: 276-282
• 101 Nierenburg H, Newman LC. Update on new daily persistent headache. Curr Treat Options Neurol 2016; 18: 25
• 102 Rozen TD. New daily persistent headache: an update. Curr Pain Headache Rep 2014; 18: 431
• 103 Lobo R, Wang M, Lobo S. et al. Time to retire 'new daily persistent headache': mode of onset of chronic migraine and tension-type headache. Cephalalgia 2022; 42: 385-395
• 104 Dono F, Consoli S, Evangelista G. et al. New daily persistent headache after SARS-CoV-2 infection: a report of two cases. Neurol Sci 2021; 42: 3965-3968
• 105 Ali A, Kriegler J, Tepper S. et al. New daily persistent headache and onabotulinumtoxin A therapy. Clin Neuropharmacol 2019; 42: 1-3
• 106 Lagrata S, Cheema S, Watkins L. et al. Long-term outcomes of occipital nerve stimulation for new daily persistent headache with migrainous features. Neuromodulation. 2020; 24: 1093-1099