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Recurrent paroxysmal headache

MedGen UID:
340201
Concept ID:
C1854337
Finding
Synonyms: Headache (with pheochromocytoma)
 
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  
  • CROGVRecurrent 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 symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; 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 hypersecrete catecholamines. Symptoms of PGL/PCC result either from 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 malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.
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 symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; 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 hypersecrete catecholamines. Symptoms of PGL/PCC result either from 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 malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.
Paragangliomas 1
MedGen UID:
358258
Concept ID:
C1868633
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues symmetrically distributed along the paravertebral axis from the base of the skull to the pelvis) and by pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas hypersecrete catecholamines; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base, neck, and upper medistinum; 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 hypersecrete catecholamines. Symptoms of PGL/PCC result either from 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 malignant transformation is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas or skull base and neck paragangliomas.

Recent clinical studies

Etiology

Marcelli V, Russo A, Cristiano E, Tessitore A
Cephalalgia 2015 May;35(6):538-44. Epub 2014 Sep 1 doi: 10.1177/0333102414547781. PMID: 25179293
Ogun OA, Janky KL, Cohn ES, Büki B, Lundberg YW
PLoS One 2014;9(9):e105546. Epub 2014 Sep 4 doi: 10.1371/journal.pone.0105546. PMID: 25187992Free PMC Article
Faralli M, Cipriani L, Del Zompo MR, Panichi R, Calzolaro L, Ricci G
B-ENT 2014;10(2):133-9. PMID: 25090812
Pollak L, Pollak E
Ann Otol Rhinol Laryngol 2014 Dec;123(12):875-80. Epub 2014 Jul 11 doi: 10.1177/0003489414539921. PMID: 25015924
Galli F, D'Antuono G, Tarantino S, Viviano F, Borrelli O, Chirumbolo A, Cucchiara S, Guidetti V
Cephalalgia 2007 Mar;27(3):211-9. doi: 10.1111/j.1468-2982.2006.01271.x. PMID: 17381555

Diagnosis

Lugovskaya N, Vinson DR
Am J Case Rep 2016 Jan 13;17:23-6. PMID: 26757615Free PMC Article
Tatuene JK, Excoffier S, Vallee JP, Kleinschmidt A
BMC Res Notes 2015 Jun 17;8:246. doi: 10.1186/s13104-015-1223-8. PMID: 26082134Free PMC Article
Pollak L, Pollak E
Ann Otol Rhinol Laryngol 2014 Dec;123(12):875-80. Epub 2014 Jul 11 doi: 10.1177/0003489414539921. PMID: 25015924
Wang Y, Tian MM, Wang XH, Zhu XQ, Liu Y, Lu YN, Pan QQ
J Headache Pain 2014 Jun 26;15:45. doi: 10.1186/1129-2377-15-45. PMID: 24966056Free PMC Article
Maniyar FH, Starr P, Goadsby PJ
Cephalalgia 2012 Jun;32(8):641-4. Epub 2012 Apr 23 doi: 10.1177/0333102412442412. PMID: 22529193

Therapy

Lugovskaya N, Vinson DR
Am J Case Rep 2016 Jan 13;17:23-6. PMID: 26757615Free PMC Article
Maniyar FH, Starr P, Goadsby PJ
Cephalalgia 2012 Jun;32(8):641-4. Epub 2012 Apr 23 doi: 10.1177/0333102412442412. PMID: 22529193
Tarantino S, Vollono C, Capuano A, Vigevano F, Valeriani M
J Headache Pain 2011 Apr;12(2):263-7. Epub 2011 Feb 22 doi: 10.1007/s10194-011-0315-7. PMID: 21340658Free PMC Article
Galli F, D'Antuono G, Tarantino S, Viviano F, Borrelli O, Chirumbolo A, Cucchiara S, Guidetti V
Cephalalgia 2007 Mar;27(3):211-9. doi: 10.1111/j.1468-2982.2006.01271.x. PMID: 17381555
Dodick DW
Cephalalgia 2000 Jun;20(5):509-14. doi: 10.1046/j.1468-2982.2000.00073.x. PMID: 11037747

Prognosis

Marcelli V, Russo A, Cristiano E, Tessitore A
Cephalalgia 2015 May;35(6):538-44. Epub 2014 Sep 1 doi: 10.1177/0333102414547781. PMID: 25179293
Pollak L, Pollak E
Ann Otol Rhinol Laryngol 2014 Dec;123(12):875-80. Epub 2014 Jul 11 doi: 10.1177/0003489414539921. PMID: 25015924
Brantberg K, Baloh RW
Acta Otolaryngol 2011 Jul;131(7):722-7. Epub 2011 Apr 6 doi: 10.3109/00016489.2011.556661. PMID: 21469911
Leroux E, Ducros A
Orphanet J Rare Dis 2008 Jul 23;3:20. doi: 10.1186/1750-1172-3-20. PMID: 18651939Free PMC Article
Lewis DW, Ashwal S, Dahl G, Dorbad D, Hirtz D, Prensky A, Jarjour I; Quality Standards Subcommittee of the American Academy of Neurology.; Practice Committee of the Child Neurology Society.
Neurology 2002 Aug 27;59(4):490-8. PMID: 12196640

Clinical prediction guides

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
Marcelli V, Russo A, Cristiano E, Tessitore A
Cephalalgia 2015 May;35(6):538-44. Epub 2014 Sep 1 doi: 10.1177/0333102414547781. PMID: 25179293
Faralli M, Cipriani L, Del Zompo MR, Panichi R, Calzolaro L, Ricci G
B-ENT 2014;10(2):133-9. PMID: 25090812
Lewis DW, Ashwal S, Dahl G, Dorbad D, Hirtz D, Prensky A, Jarjour I; Quality Standards Subcommittee of the American Academy of Neurology.; Practice Committee of the Child Neurology Society.
Neurology 2002 Aug 27;59(4):490-8. PMID: 12196640
Vaidyanathan S, Soni BM, Sett P, Watt JW, Oo T, Bingley J
Spinal Cord 1998 Nov;36(11):761-70. PMID: 9848483

Recent systematic reviews

Lewis DW, Ashwal S, Dahl G, Dorbad D, Hirtz D, Prensky A, Jarjour I; Quality Standards Subcommittee of the American Academy of Neurology.; Practice Committee of the Child Neurology Society.
Neurology 2002 Aug 27;59(4):490-8. PMID: 12196640

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