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Recurrent paroxysmal headache

MedGen UID:
927617
Concept ID:
C4293708
Sign or Symptom
HPO: HP:0002331

Definition

Repeated episodes of headache with rapid onset, reaching a peak within minutes and of short duration (less than one hour) with pain that is throbbing, pulsating, or bursting in quality. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Recurrent paroxysmal headache

Conditions with this feature

Paragangliomas 3
MedGen UID:
340200
Concept ID:
C1854336
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 4
MedGen UID:
349380
Concept ID:
C1861848
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 1
MedGen UID:
488134
Concept ID:
C3494181
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.

Professional guidelines

PubMed

Clifton-Bligh RJ
Rev Endocr Metab Disord 2023 Feb;24(1):49-56. Epub 2023 Jan 13 doi: 10.1007/s11154-022-09773-2. PMID: 36637675Free PMC Article
Shen Y, Qi X
Neurol Sci 2022 Mar;43(3):1659-1666. Epub 2022 Jan 11 doi: 10.1007/s10072-022-05872-9. PMID: 35015204
van de Berg R, Widdershoven J, Bisdorff A, Evers S, Wiener-Vacher S, Cushing SL, Mack KJ, Kim JS, Jahn K, Strupp M, Lempert T
J Vestib Res 2021;31(1):1-9. doi: 10.3233/VES-200003. PMID: 33386837Free PMC Article

Recent clinical studies

Etiology

Kuo MJM, Nazari MA, Jha A, Pacak K
Front Endocrinol (Lausanne) 2022;13:936178. Epub 2022 Jul 12 doi: 10.3389/fendo.2022.936178. PMID: 35903274Free PMC Article
Leone M, Ferraro S, Proietti Cecchini A
Handb Clin Neurol 2021;182:401-414. doi: 10.1016/B978-0-12-819973-2.00027-7. PMID: 34266608
Russo A, Bruno A, Trojsi F, Tessitore A, Tedeschi G
Curr Pain Headache Rep 2016 Feb;20(2):9. doi: 10.1007/s11916-016-0539-y. PMID: 26757711
Costa A, Antonaci F, Ramusino MC, Nappi G
Curr Neuropharmacol 2015;13(3):304-23. doi: 10.2174/1570159x13666150309233556. PMID: 26411963Free PMC Article
Cass SP, Furman JM, Ankerstjerne K, Balaban C, Yetiser S, Aydogan B
Ann Otol Rhinol Laryngol 1997 Mar;106(3):182-9. doi: 10.1177/000348949710600302. PMID: 9078929

Diagnosis

Ananthavarathan P, Kamourieh S
Handb Clin Neurol 2023;198:221-227. doi: 10.1016/B978-0-12-823356-6.00005-6. PMID: 38043964
Silva VPR, Castro LHM, Calderaro M
Arq Neuropsiquiatr 2022 May;80(5 Suppl 1):232-237. doi: 10.1590/0004-282X-ANP-2022-S111. PMID: 35976301Free PMC Article
van de Berg R, Widdershoven J, Bisdorff A, Evers S, Wiener-Vacher S, Cushing SL, Mack KJ, Kim JS, Jahn K, Strupp M, Lempert T
J Vestib Res 2021;31(1):1-9. doi: 10.3233/VES-200003. PMID: 33386837Free PMC Article
Bisdorff A
Curr Opin Neurol 2014 Feb;27(1):105-10. doi: 10.1097/WCO.0000000000000061. PMID: 24316729
Strupp M, Versino M, Brandt T
Handb Clin Neurol 2010;97:755-71. doi: 10.1016/S0072-9752(10)97062-0. PMID: 20816468

Therapy

Russo A, Bruno A, Trojsi F, Tessitore A, Tedeschi G
Curr Pain Headache Rep 2016 Feb;20(2):9. doi: 10.1007/s11916-016-0539-y. PMID: 26757711
Cologno D, Mazzeo A, Lecce B, Mundi C, Petretta V, Casucci G, d'Onofrio F
Neurol Sci 2012 May;33 Suppl 1:S193-8. doi: 10.1007/s10072-012-1066-1. PMID: 22644202
Strupp M, Versino M, Brandt T
Handb Clin Neurol 2010;97:755-71. doi: 10.1016/S0072-9752(10)97062-0. PMID: 20816468
Clemett D, Markham A
Drugs 2000 Feb;59(2):271-7; discussion 278-9. doi: 10.2165/00003495-200059020-00016. PMID: 10730550
Diener HC, Kaube H, Limmroth V
J Neurol 1999 Jul;246(7):515-9. doi: 10.1007/s004150050396. PMID: 10463349

Prognosis

Costa A, Antonaci F, Ramusino MC, Nappi G
Curr Neuropharmacol 2015;13(3):304-23. doi: 10.2174/1570159x13666150309233556. PMID: 26411963Free PMC Article
Leroux E, Ducros A
Orphanet J Rare Dis 2008 Jul 23;3:20. doi: 10.1186/1750-1172-3-20. PMID: 18651939Free PMC Article
Plouin PF, Gimenez-Roqueplo AP
Orphanet J Rare Dis 2006 Dec 8;1:49. doi: 10.1186/1750-1172-1-49. PMID: 17156452Free PMC Article
Neuhauser H, Lempert T
Cephalalgia 2004 Feb;24(2):83-91. doi: 10.1111/j.1468-2982.2004.00662.x. PMID: 14728703
Vickers ER, Cousins MJ
Aust Endod J 2000 Apr;26(1):19-26. doi: 10.1111/j.1747-4477.2000.tb00146.x. PMID: 11359293

Clinical prediction guides

Kuo MJM, Nazari MA, Jha A, Pacak K
Front Endocrinol (Lausanne) 2022;13:936178. Epub 2022 Jul 12 doi: 10.3389/fendo.2022.936178. PMID: 35903274Free PMC Article
Cao Y, Wu J, Yue Y, Zhang C, Liu S, Zhong P, Wang S, Huang X, Deng W, Pan J, Zheng L, Liu Q, Shang L, Zhang B, Yang J, Chen G, Chen S, Cao L, Luan X
Acta Neurol Belg 2022 Jun;122(3):647-658. Epub 2021 Feb 24 doi: 10.1007/s13760-021-01622-4. PMID: 33625684
Costa A, Antonaci F, Ramusino MC, Nappi G
Curr Neuropharmacol 2015;13(3):304-23. doi: 10.2174/1570159x13666150309233556. PMID: 26411963Free PMC Article
Yadav YR, Yadav N, Parihar V, Kher Y, Ratre S
Turk Neurosurg 2015;25(3):362-71. doi: 10.5137/1019-5149.JTN.11086-14.1. PMID: 26037175
Flicinski J, Wigowska-Sowinska J, Winczewska-Wiktor A, Steinborn B
Neurol Neurochir Pol 2015;49(1):74-7. Epub 2015 Jan 14 doi: 10.1016/j.pjnns.2015.01.004. PMID: 25666779

Recent systematic reviews

Morra ME, Elgebaly A, Elmaraezy A, Khalil AM, Altibi AM, Vu TL, Mostafa MR, Huy NT, Hirayama K
J Headache Pain 2016 Dec;17(1):63. Epub 2016 Jul 5 doi: 10.1186/s10194-016-0651-8. PMID: 27377706Free PMC Article

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