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Flammer syndrome

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tension glaucoma with the syndrome has recently been confirmed by a group of Chinese researchers. In a 2016 review on the risk factors for normal tension glaucoma by ophthalmologists from Asia (where this form of glaucoma is much more prevalent than in Europe or North America), Flammer syndrome has been attributed to increase the likelihood of ganglion cell damage in these patients, with disc hemorrhages as a characteristic clinical sign. Migraine attacks, a common feature of Flammer syndrome, have been described as a risk factor for glaucoma progression, in open-angle glaucoma as well as in normal tension glaucoma. Flammer syndrome may also predispose to other eye diseases such as vascular occlusion (especially retinal vein occlusion) in relatively young people or central serous retinopathy. Muscle spasms and tension are common among individuals with Flammer syndrome. Tinnitus and sometimes even acute hearing loss can occur. There is currently insufficient data available on cases where Flammer syndrome is suspected like in the sudden, unexpected deaths of young athletes. People with
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of not being underweight), avoiding periods of fasting, and avoidance of known trigger factors such as cold. Regular physical exercise is beneficial, while extreme sports might be detrimental. Nutrition should contain many antioxidants, such as those found in green tea, black filter coffee, red wine, blueberries, and fruits. Omega-3 fatty acids, especially in the form of fish, improve the regulation of blood flow, as well as ginkgo. If blood pressure is very low, salt intake should be increased. Drugs that can lead to vasoconstriction should be avoided. If blood pressure is too low, sleeping pills should be taken cautiously. Calcium antagonists like magnesium may help against the vascular dysregulation. With lifestyle interventions, attacks—particularly pronounced symptoms such as massive cold extremities, tinnitus, or migraine-like episodes—can be avoided or reduced. Stabilizing weight (i.e. not being underweight) and the avoidance of periods of fasting are important.
207:, resulting in insufficient supply of blood to the corresponding organs or parts of organs. Such spasms can occur at various locations in the human body; in this case medical terminology calls it "vasospastic syndrome". Over the years, it has been established that these spasms are usually part of a general dysregulation of blood vessels. These dysregulations can occur in spasms as well as in excessive or insufficient dilation of arteries, veins, and capillaries. The blood vessels of individuals with vasospastic syndrome respond to stimuli insufficiently. If an identifiable disease does not cause this, it is called a primary vascular dysregulation (PVD); in case of an underlying disease, it is called secondary PVD. PVD is almost always associated with other vascular and non-vascular symptoms and signs. This entire complex (PVD and accompanying symptoms) is today called Flammer syndrome. 71:
and red patches on the face or neck, and migraine-like pain or a feeling of pressure behind the upper eyelid. In addition, there are symptoms not directly resulting from dysregulations of the blood supply, such as a prolonged time needed to fall asleep, a reduced feeling of thirst, and high sensitivity not only to cold but also to odors, vibrations, psychological stress, or certain medications (e.g., calcium antagonists, beta blockers). Pain and muscle spasms are common.
166:, a disease with an impaired regulation of blood flow in a large number of patients. If glaucomatous damage occurs despite normal eye pressure or if glaucomatous damage is progressive despite normalized intraocular pressure, frequently Flammer syndrome is the cause. In these eyes, an elevated pressure in the retinal veins has been observed. 152:
The diagnosis of Flammer syndrome is primarily based on the patient's history with its typical features as well as the findings of nail fold capillaroscopy. This test shows an excessive constriction (narrowing) in the smallest blood vessels (capillaries) of the fingers in response to being exposed to
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Flammer syndrome requires no therapy as long as the individual does not experience the symptoms or pathological sequelae occur. The treatment is based on three pillars: lifestyle interventions, diet, and medication. A healthy lifestyle should include regular sleep, weight stabilization (in the sense
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Most of the symptoms of Flammer syndrome result from an impaired regulation of the blood supply. How these symptoms affect the patient depends on to which organs or body parts blood supply is inhibited. Typical symptoms of Flammer syndrome are cold hands or feet, low blood pressure, occasional white
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Medical treatment is necessary when normal tension glaucoma is present. In these cases, in addition to the ophthalmologic treatment of glaucoma, the patient's usually low blood pressure should be addressed. With dietary measures such as salt intake and sometimes with low-dose steroids, a drop in
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Glaucoma patients with Flammer syndrome show some specific clinical signs like increased frequency of optic disc haemorrhages, activated retinal astrocytes, elevated retinal venous pressure, optic nerve compartmentalization, and fluctuating diffuse visual field defects. The association of normal
82:, while intraocular pressure is normal. Many patients with normal tension glaucoma have a basic constitution, which corresponds to the Flammer syndrome. Some of its features were formerly summarized under the term "primary vascular dysregulation syndrome". 161:
Having Flammer syndrome does not necessarily mean that an individual is sick. Most of those affected are and remain healthy. Certain diseases such as arteriosclerosis and its consequences are probably even rarer. Best known is the higher risk of
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Yeghiazaryan K, Flammer J, OrgĂĽl S, Wunderlich K, Golubnitschaja O.: Vasospastic individuals demonstrate significant similarity to glaucoma patients as revealed by gene expression profiling in circulating leukocytes. Mol Vis. 2009;15:
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Konieczka, Katarzyna; Ritch, Robert; Traverso, Carlo Enrico; Kim, Dong Myung; Kook, Michael Scott; Gallino, Augusto; Golubnitschaja, Olga; Erb, Carl; Reitsamer, Herbert A; Kida, Teruyo; Kurysheva, Natalia; Yao, Ke (2014-07-08).
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Vollenweider, S; Wirz-Justice, A; et al. (Feb 2008). "Chronobiological characterization of women with primary vasospastic syndrome: body heat loss capacity in relation to sleep initiation and phase of entrainment".
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blood pressure, especially during sleep, can often be prevented. These nightly "dips" of blood pressure in normal tension glaucoma patients with Flammer syndrome can significantly damage the sensory cells of the retina.
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Kräuchi, K; Gasio, PF; Vollenweider, S; et al. (Dec 2008). "Cold extremities and difficulties initiating sleep: evidence of co-morbidity from a random sample of a Swiss urban population".
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Quill B, Henry E, Simon E, O'Brien C: Evaluation of the Effect of Hypercapnia on Vascular Function in Normal Tension Glaucoma. Biomed Res Int 2015, published online October 18
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cold. Patients with vasospastic symptoms characteristic of Flammer syndrome have shown a unique gene expression when their lymphocytes underwent gene expression profiling.
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is a described clinical entity comprising a complex of clinical features caused mainly by dysregulation of the blood supply. It was previously known as
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seem to have Flammer syndrome quite frequently, and possible associations with the vascular factor endothelin-1 are under investigation.
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Todorova, M.G.; et al. (2015). "Endothelin-1 Plasma Levels in Patients with both Retinitis Pigmentosa and Flammer Syndrome".
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have been known, particularly in the vessels supplying the retina with blood. These vasospasms are temporary narrowings of
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Kim, KE; Park, KH (2016). "Update on the Prevalence, Etiology, Diagnosis, and Monitoring of Normal-Tension Glaucoma".
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People with Flammer syndrome sleep poorly and have a decreased sensation of thirst.
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Nakazawa, T (2016). "Ocular Blood Flow and Influencing Factors for Glaucoma".
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excessive response to mental or physical stimuli such as stress or cold
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Konieczka K, Fränkl S. Primary Vascular Dysregulation and Glaucoma.
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Fang, Lei; Baertschie, Michael; Mozaffarieh, Manneli (2014).
58:. Flammer syndrome is named after the Swiss ophthalmologist 792:"The effect of Flammer syndrome on retinal venous pressure" 555:"The effect of flammer-syndrome on venous retinal pressure" 884:
Konieczka, Katarzyna; Ritch, Robert; et al. (2014).
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Konieczka, Katarzyna; Ritch, Robert; et al. (2014).
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reversible patchy white or red discoloration of the skin
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The disease is more prevalent in the following groups:
604:Wojcik-Gryciuka, A; Skup, M; Waleszczyk, M (2016). 427:Flammer, J.; Konieczka, K.; Flammer, A. J. (2013). 29: 24: 982:Flammer, J; Konieczka, K; et al. (May 2013). 790:Fang, L; Baertschi, M; Mozaffarieh, M (Oct 2014). 935:Konieczka, K.; Fränkl, S.; et al. (2014). " 655:"Ocular Blood Flow and Normal Tension Glaucoma" 606:"Glaucoma – state of the art and perspectives" 140:in academics compared with blue collar-workers 1031:Flammer, Josef; Konieczka, Katarzyna (2017). 8: 941:Klinische Monatsblätter fĂĽr Augenheilkunde 21: 1052: 1007: 911: 901: 817: 807: 680: 670: 653:Fan, N; Wang, P; Tang, L; Liu, X (2015). 629: 580: 570: 522:Zeitschrift fĂĽr praktische Augenheilkunde 454: 444: 403: 393: 308: 259: 241: 215: 104:increased sensitivity to pain and odors 610:Restorative Neurology and Neuroscience 335:Am J Physiol Regul Integr Comp Physiol 7: 1081:Scientific essay on Flammer syndrome 937:Unstable oxygen supply and glaucoma. 134:in people with lower blood pressure 101:prolongation of time to fall asleep 85:Signs of Flammer syndrome include: 137:in Asians compared with Caucasians 16:Syndrome of vascular dysregulation 14: 1076:On Flammer syndrome and glaucoma 492:10.1111/j.1365-2869.2008.00678.x 289:British Journal of Ophthalmology 1: 761:10.1097/apo.0000000000000183 718:10.1097/apo.0000000000000177 107:sensitivity to certain drugs 1071:Website on Flammer syndrome 843:Klin. Monatsbl. Augenheilkd 98:decreased feeling of thirst 89:cold hands or feet, or both 1122: 347:10.1152/ajpregu.00609.2007 1045:10.1007/s13167-017-0090-x 480:Journal of Sleep Research 1000:10.1093/eurheartj/eht023 809:10.1186/1471-2415-14-121 572:10.1186/1471-2415-14-121 285:"Vasospasm and glaucoma" 131:in women compared to men 984:"The Eye and the Heart" 749:Asia Pac. J. Ophthalmol 706:Asia Pac. J. Ophthalmol 164:normal tension glaucoma 56:normal tension glaucoma 953:10.1055/s-0033-1360242 903:10.1186/1878-5085-5-11 855:10.1055/s-0035-1545674 446:10.1186/1878-5085-4-14 395:10.1186/1878-5085-5-11 283:O'Brien, Colm (1998). 243:10.1186/1878-5085-5-11 48:vascular dysregulation 157:Clinical significance 95:lower body mass index 301:10.1136/bjo.82.8.855 172:retinitis pigmentosa 92:arterial hypotension 74:In many people with 672:10.1155/2015/308505 195:For over 100 years 886:"Flammer syndrome" 622:10.3233/rnn-150599 378:"Flammer syndrome" 226:"Flammer syndrome" 66:Signs and symptoms 52:low blood pressure 1096:Vascular diseases 559:BMC Ophthalmology 41: 40: 19:Medical condition 1113: 1059: 1058: 1056: 1028: 1022: 1021: 1011: 979: 973: 972: 932: 926: 925: 915: 905: 881: 875: 874: 838: 832: 831: 821: 811: 787: 781: 780: 744: 738: 737: 701: 695: 694: 684: 674: 650: 644: 643: 633: 601: 595: 594: 584: 574: 550: 544: 541: 535: 531: 525: 524:2013; 34:207-215 518: 512: 511: 475: 469: 468: 458: 448: 433:The EPMA Journal 424: 418: 417: 407: 397: 373: 367: 366: 329: 323: 322: 312: 280: 274: 273: 263: 245: 230:The EPMA Journal 220: 44:Flammer syndrome 25:Flammer syndrome 22: 1121: 1120: 1116: 1115: 1114: 1112: 1111: 1110: 1086: 1085: 1067: 1062: 1030: 1029: 1025: 981: 980: 976: 934: 933: 929: 883: 882: 878: 840: 839: 835: 789: 788: 784: 746: 745: 741: 703: 702: 698: 659:Biomed Res. 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Heart J 987: 977: 944: 940: 936: 930: 893: 890:EPMA Journal 889: 879: 846: 842: 836: 799: 795: 785: 755:(1): 38–44. 752: 748: 742: 712:(1): 23–31. 709: 705: 699: 662: 658: 648: 613: 609: 599: 562: 558: 548: 539: 529: 521: 516: 486:(4): 420–6. 483: 479: 473: 436: 432: 422: 385: 382:EPMA Journal 381: 371: 338: 334: 327: 292: 288: 278: 233: 229: 218: 194: 185: 181: 168: 160: 151: 143: 126: 84: 73: 69: 47: 43: 42: 1090:Categories 802:(1): 121. 665:: 308505. 439:(1): S14. 211:References 205:arterioles 197:vasospasms 178:Management 1106:Syndromes 1039:(8): 88. 969:206356894 896:(1): 11. 508:205352251 388:(1): 11. 252:1878-5077 236:(1): 11. 148:Diagnosis 36:Angiology 31:Specialty 1018:23401492 961:24532398 922:25075228 871:23558601 863:25902111 828:25312339 777:31765476 769:26886118 734:30907471 726:26886116 691:26558263 640:26684267 591:25312339 534:2339-48. 500:19021849 465:23742177 414:25075228 355:18046019 270:25075228 201:arteries 116:tinnitus 111:migraine 76:glaucoma 1054:5486542 1009:3640200 913:4113774 819:4216361 682:4628977 631:4927811 582:4216361 565:: 121. 456:3693953 405:4113774 363:4814596 319:9828764 310:1722712 261:4113774 191:History 1051:  1016:  1006:  967:  959:  920:  910:  869:  861:  826:  816:  775:  767:  732:  724:  689:  679:  638:  628:  589:  579:  506:  498:  463:  453:  412:  402:  361:  353:  317:  307:  268:  258:  250:  80:retina 965:S2CID 867:S2CID 773:S2CID 730:S2CID 504:S2CID 359:S2CID 1014:PMID 957:PMID 918:PMID 859:PMID 824:PMID 765:PMID 722:PMID 687:PMID 663:2015 636:PMID 587:PMID 496:PMID 461:PMID 410:PMID 351:PMID 315:PMID 266:PMID 248:ISSN 1049:PMC 1041:doi 1004:PMC 996:doi 949:doi 945:231 939:". 908:PMC 898:doi 851:doi 847:232 814:PMC 804:doi 757:doi 714:doi 677:PMC 667:doi 626:PMC 618:doi 577:PMC 567:doi 488:doi 451:PMC 441:doi 400:PMC 390:doi 343:doi 339:294 305:PMC 297:doi 256:PMC 238:doi 203:or 1092:: 1047:. 1035:. 1012:. 1002:. 992:34 990:. 986:. 963:. 955:. 943:. 916:. 906:. 892:. 888:. 865:. 857:. 845:. 822:. 812:. 800:14 798:. 794:. 771:. 763:. 751:. 728:. 720:. 708:. 685:. 675:. 661:. 657:. 634:. 624:. 614:34 612:. 608:. 585:. 575:. 563:14 561:. 557:. 502:. 494:. 484:17 482:. 459:. 449:. 435:. 431:. 408:. 398:. 384:. 380:. 357:. 349:. 337:. 313:. 303:. 293:82 291:. 287:. 264:. 254:. 246:. 232:. 228:. 62:. 1057:. 1043:: 1020:. 998:: 971:. 951:: 924:. 900:: 894:5 873:. 853:: 830:. 806:: 779:. 759:: 753:5 736:. 716:: 710:5 693:. 669:: 642:. 620:: 593:. 569:: 510:. 490:: 467:. 443:: 437:4 416:. 392:: 386:5 365:. 345:: 321:. 299:: 272:. 240:: 234:5

Index

Specialty
Angiology
low blood pressure
normal tension glaucoma
Josef Flammer
glaucoma
retina
migraine
tinnitus
normal tension glaucoma
retinitis pigmentosa
vasospasms
arteries
arterioles
"Flammer syndrome"
doi
10.1186/1878-5085-5-11
ISSN
1878-5077
PMC
4113774
PMID
25075228
"Vasospasm and glaucoma"
doi
10.1136/bjo.82.8.855
PMC
1722712
PMID
9828764

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