Download PDF
Klin Monbl Augenheilkd 2016; 233(12): 1331-1336
DOI: 10.1055/s-0042-118881
Übersicht
Georg Thieme Verlag KG Stuttgart · New York

Phänomenologie und klinische Bedeutung des Flammer-Syndroms

Phenomenology and Clinical Relevance of the Flammer Syndrome
K. Konieczka
University of Basel, Department of Ophthalmology, Switzerland
,
J. Flammer
University of Basel, Department of Ophthalmology, Switzerland
› Author Affiliations
Further Information

Publication History

eingereicht 16 August 2016

akzeptiert 08 September 2016

Publication Date:
16 December 2016 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Das Flammer-Syndrom (FS) beschreibt den Phänotyp von Menschen mit einer Prädisposition für eine veränderte Reaktion der Blutgefäße auf Stimuli wie Kälte, emotionellen Stress oder große Höhe. Häufige Symptome sind: kalte Hände und/oder Füße, tiefer Blutdruck, verlängerte Einschlafzeit, reduziertes Durstgefühl, gesteigerte Empfindlichkeit für Gerüche, Schmerzen, Vibrationen und gewisse Medikamente. Menschen mit FS sind im Allgemeinen strebsam und erfolgreich, aber auch perfektionistisch und teils grüblerisch. Häufige Zeichen sind: veränderte Genexpression, verlängerter Flussstillstand in den Nagelfalzkapillaren nach Kälteprovokation, verminderte Autoregulation der okularen Durchblutung, reduzierte Gefäßerweiterung auf Flickerlichtstimulation. Der retinale Venendruck ist im Mittel höher und die Astrozyten sind häufiger aktiviert. FS ist häufiger bei Frauen als bei Männern, häufiger bei dünnen als bei dicken und bei jungen als bei alten Menschen, häufiger bei Akademikern als bei Nichtakademikern. Es ist sehr selten bei Menschen mit Berufen im Freien. Es ist assoziiert mit Normaldruckglaukom, okularen Gefäßverschlüssen, Retinitis pigmentosa, multipler Sklerose, Tinnitus und seltener sogar mit Hörstürzen.

Abstract

The Flammer syndrome (FS) describes the phenotype of people with a predisposition for an altered reaction of the blood vessels to stimuli like coldness, emotional stress or high altitude. Frequent symptoms are: cold hands and/or feet, low blood pressure, prolonged sleep onset time, reduced feeling of thirst, increased sensitivity to odour, pain, vibration and certain drugs. SF subjects are often ambitious and successful but also perfectionistic and sometimes brooding. Frequent signs are: altered gene expression, prolonged blood flow cessation in nailfold capillaroscopy after cold provocation, reduced autoregulation of ocular blood flow, and reduced vasodilation after stimulation with flickering light. Retinal venous pressure is on the average higher and retinal astrocytes are more often activated. FS occurs more often in females than in males, in thin than in obese subjects, in young than in old people, in graduates than in blue collar workers, in subjects with indoor than outdoor jobs. Associated diseases are: normal tension glaucoma, occlusion of ocular vessels, retinitis pigmentosa, multiple sclerosis, tinnitus or even sudden hearing loss.

Schlüsselwörter

primäre vaskuläre Dysregulation - Glaukom - Retinitis pigmentosa - multiple Sklerose - kalte Hände

Key words

primary vascular dysregulation - glaucoma - retinitis pigmentosa - multiple sclerosis - cold hands
 

  • Literatur

  • 1 Konieczka K, Ritch R, Traverso CE et al. Flammer syndrome. EPMA J 2014; 5: 11
    CrossrefPubMedGoogle Scholar
  • 2 Flammer J, Konieczka K, Flammer AJ. The primary vascular dysregulation syndrome: implications for eye diseases. EPMA J 2013; 4: 14
    CrossrefPubMedGoogle Scholar
  • 3 Flammer J, Konieczka K, Bruno RM et al. The eye and the heart. Eur Heart J 2013; 34: 1270-1278
    CrossrefPubMedGoogle Scholar
  • 4 Flammer J, Orgul S, Costa VP et al. The impact of ocular blood flow in glaucoma. Prog Retin Eye Res 2002; 21: 359-393
    CrossrefPubMedGoogle Scholar
  • 5 Konieczka K, Flammer AJ, Todorova M et al. Retinitis pigmentosa and ocular blood flow. EPMA J 2012; 3: 17
    CrossrefPubMedGoogle Scholar
  • 6 Konieczka K, Koch S, Schoetzau A et al. Increased prevalence of Flammer syndrome in patients with retinitis pigmentosa. Klin Monatsbl Augenheilkd 2016; 233: 448-452
    Article in Thieme ConnectPubMedGoogle Scholar
  • 7 Konieczka K, Koch S, Binggeli T et al. Multiple sclerosis and primary vascular dysregulation (Flammer syndrome). EPMA J 2016; 7: 13
    CrossrefPubMedGoogle Scholar
  • 8 Flammer J, Mozaffarieh M. What is the present pathogenetic concept of glaucomatous optic neuropathy?. Surv Ophthalmol 2007; 52 (Suppl. 02) S162-S173
    CrossrefPubMedGoogle Scholar
  • 9 Pillunat LE, Stodtmeister R. Inzidenz des Niederdruckglaukoms bei hämodynamisch relevanter Karotisstenose. Spektrum Augenheilkd 1988; 2: 24-27
    PubMedGoogle Scholar
  • 10 Flammer J. Psychophysical mechanisms and treatment of vasospastic disorders in normal-tension glaucoma. Bull Soc Belge Ophtalmol 1992; 244: 129-134
    PubMedGoogle Scholar
  • 11 Emre M, Orgul S, Gugleta K et al. Ocular blood flow alteration in glaucoma is related to systemic vascular dysregulation. Br J Ophthalmol 2004; 88: 662-666
    CrossrefPubMedGoogle Scholar
  • 12 Konieczka K, Frankl S, Todorova MG et al. Unstable oxygen supply and glaucoma. Klin Monatsbl Augenheilkd 2014; 231: 121-126
    Article in Thieme ConnectPubMedGoogle Scholar
  • 13 Flammer J, Guthauser U, Mahler F. Do ocular vasospasms help cause low-tension glaucoma?. Doc Ophthalmol Proc Ser 1987; 49: 397-399
    PubMedGoogle Scholar
  • 14 Guthauser U, Flammer J, Mahler F. The relationship between digital and ocular vasospasm. Graefes Arch Clin Exp Ophthalmol 1988; 226: 224-226
    CrossrefPubMedGoogle Scholar
  • 15 Gasser P, Flammer J. Blood-cell velocity in the nailfold capillaries of patients with normal-tension and high-tension glaucoma. Am J Ophthalmol 1991; 111: 585-588
    CrossrefPubMedGoogle Scholar
  • 16 Flammer J, Haefliger IO, Orgul S et al. Vascular dysregulation: a principal risk factor for glaucomatous damage?. J Glaucoma 1999; 8: 212-219
    Google Scholar
  • 17 Saner H, Wurbel H, Mahler F et al. Microvasculatory evaluation of vasospastic syndromes. Adv Exp Med Biol 1987; 220: 215-218
    PubMedGoogle Scholar
  • 18 Mahler F, Saner H, Wurbel H et al. Local cooling test for clinical capillaroscopy in Raynaudʼs phenomenon, unstable angina, and vasospastic visual disorders. Vasa 1989; 18: 201-204
    PubMedGoogle Scholar
  • 19 Gherghel D, Orgul S, Gugleta K et al. Retrobulbar blood flow in glaucoma patients with nocturnal over-dipping in systemic blood pressure. Am J Ophthalmol 2001; 132: 641-647
    CrossrefPubMedGoogle Scholar
  • 20 Vollenweider S, Wirz-Justice A, Flammer J et al. Chronobiological characterization of women with primary vasospastic syndrome: body heat loss capacity in relation to sleep initiation and phase of entrainment. Am J Physiol Regul Integr Comp Physiol 2008; 294: R630-R638
    PubMedGoogle Scholar
  • 21 Pache M, Krauchi K, Cajochen C et al. Cold feet and prolonged sleep-onset latency in vasospastic syndrome. Lancet 2001; 358: 125-126
    CrossrefPubMedGoogle Scholar
  • 22 Mozaffarieh M, Hauenstein D, Schoetzau A et al. Smell perception in normal tension glaucoma patients. Mol Vis 2010; 16: 506-510
    PubMedGoogle Scholar
  • 23 Wunderlich K, Zimmerman C, Gutmann H et al. Vasospastic persons exhibit differential expression of ABC-transport proteins. Mol Vis 2003; 9: 756-761
    PubMedGoogle Scholar
  • 24 Teuchner B, Orgul S, Ulmer H et al. Reduced thirst in patients with a vasospastic syndrome. Acta Ophthalmol Scand 2004; 82: 738-740
    CrossrefPubMedGoogle Scholar
  • 25 Flammer J, Pache M, Resink T. Vasospasm, its role in the pathogenesis of diseases with particular reference to the eye. Prog Retin Eye Res 2001; 20: 319-349
    CrossrefPubMedGoogle Scholar
  • 26 Yeghiazaryan K, Flammer J, Orgul S et al. Vasospastic individuals demonstrate significant similarity to glaucoma patients as revealed by gene expression profiling in circulating leukocytes. Mol Vis 2009; 15: 2339-2348
    PubMedGoogle Scholar
  • 27 Gherghel D, Orgul S, Dubler B et al. Is vascular regulation in the central retinal artery altered in persons with vasospasm?. Arch Ophthalmol 1999; 117: 1359-1362
    CrossrefPubMedGoogle Scholar
  • 28 Oettli A, Gugleta K, Kochkorov A et al. Rigidity of retinal vessel in untreated eyes of normal tension primary open-angle glaucoma patients. J Glaucoma 2011; 20: 303-306
    CrossrefPubMedGoogle Scholar
  • 29 Gugleta K, Zawinka C, Rickenbacher I et al. Analysis of retinal vasodilation after flicker light stimulation in relation to vasospastic propensity. Invest Ophthalmol Vis Sci 2006; 47: 4034-4041
    CrossrefPubMedGoogle Scholar
  • 30 Grieshaber MC, Terhorst T, Flammer J. The pathogenesis of optic disc splinter haemorrhages: a new hypothesis. Acta Ophthalmol Scand 2006; 84: 62-68
    PubMedGoogle Scholar
  • 31 Flammer J, Mozaffarieh M, Bebie H. Basic Sciences in Ophthalmology. Berlin, Heidelberg: Springer; 2013
    CrossrefGoogle Scholar
  • 32 Fang L, Baertschi M, Mozaffarieh M. The effect of flammer-syndrome on retinal venous pressure. BMC Ophthalmol 2014; 14: 121
    Google Scholar
  • 33 Vahedian Z, Amini H, Tehrani MH et al. Retinal venous pressure in chronic smokers. EPMA J 2015; 6: 8
    CrossrefPubMedGoogle Scholar
  • 34 Flammer J, Konieczka K. Retinal venous pressure: the role of endothelin. EPMA J 2015; 6: 21
    CrossrefPubMedGoogle Scholar
  • 35 Baertschi M, Dayhaw-Barker P, Flammer J. The effect of hypoxia on intra-ocular, mean arterial, retinal venous and ocular perfusion pressures. Clin Hemorheol Microcirc 2016; 63: 293-303
    Google Scholar
  • 36 Mozaffarieh M, Bartschi M, Henrich PB et al. Retinal venous pressure in the non-affected eye of patients with retinal vein occlusions. Graefes Arch Clin Exp Ophthalmol 2014; 252: 1569-1571
    Google Scholar
  • 37 Fang L, Turtschi S, Mozaffarieh M. The effect of nifedipine on retinal venous pressure of glaucoma patients with the Flammer-Syndrome. Graefes Arch Clin Exp Ophthalmol 2015; 253: 935-939
    CrossrefPubMedGoogle Scholar
  • 38 Neumann T, Baertschi M, Vilser W et al. Retinal vessel regulation at high altitudes1. Clin Hemorheol Microcirc 2016; 63: 281-292
    PubMedGoogle Scholar
  • 39 Grieshaber MC, Orgul S, Schoetzau A et al. Relationship between retinal glial cell activation in glaucoma and vascular dysregulation. J Glaucoma 2007; 16: 215-219
    Google Scholar
  • 40 Mozaffarieh M, Fontana Gasio P, Schotzau A et al. Thermal discomfort with cold extremities in relation to age, gender, and body mass index in a random sample of a Swiss urban population. Popul Health Metr 2010; 8: 17
    CrossrefPubMedGoogle Scholar
  • 41 Kavroulaki D, Gugleta K, Kochkorov A et al. Relation of body mass index and blood pressure to subjective and objective acral temperature. Klin Monatsbl Augenheilkd 2009; 226: 328-331
    Article in Thieme ConnectPubMedGoogle Scholar
  • 42 Konieczka K, Gugleta K. Glaukom. 4. Aufl. Bern: Hans Huber; 2015
    Google Scholar
  • 43 Konieczka K, Todorova MG, Bojinova RI et al. Unexpected effect of calcium channel blockers on the optic nerve compartment syndrome. Klin Monatsbl Augenheilkd 2016; 233: 387-390
    Article in Thieme ConnectPubMedGoogle Scholar
  • 44 Fraenkl SA, Mozaffarieh M, Flammer J. Retinal vein occlusions: The potential impact of a dysregulation of the retinal veins. EPMA J 2010; 1: 253-261
    CrossrefPubMedGoogle Scholar
  • 45 Messerli J, Flammer J. [Central vein thrombosis in younger patients]. Klin Monatsbl Augenheilkd 1996; 208: 303-305
    Article in Thieme ConnectPubMedGoogle Scholar
  • 46 Konieczka K, Todorova MG, Chackathayil TN et al. Cilioretinal artery occlusion in a young patient with flammer syndrome and increased retinal venous pressure. Klin Monatsbl Augenheilkd 2015; 232: 576-578
    Article in Thieme ConnectPubMedGoogle Scholar
  • 47 Bojinova RI, Konieczka K, Todorova MG. Unilateral loss of vision after spinal surgery in a patient with Flammer syndrome. Klin Monatsbl Augenheilkd 2016; 233: 429-431
    Article in Thieme ConnectPubMedGoogle Scholar
  • 48 Bojinova RI, Konieczka K, Meyer P et al. The trilateral link between anaesthesia, perioperative visual loss and Flammer syndrome. BMC Anesthesiol 2016; 16: 10
    PubMedGoogle Scholar
  • 49 Prunte C, Flammer J. Choroidal capillary and venous congestion in central serous chorioretinopathy. Am J Ophthalmol 1996; 121: 26-34
    CrossrefPubMedGoogle Scholar
  • 50 Pirodda A, Brandolini C, Cassandro E et al. Primary vascular dysregulation syndrome: possible implications for inner ear acute diseases?. Med Hypotheses 2015; 85: 586-587
    CrossrefPubMedGoogle Scholar
  • 51 Golubnitschaja O, Debald M, Yeghiazaryan K et al. Breast cancer epidemic in the early twenty-first century: evaluation of risk factors, cumulative questionnaires and recommendations for preventive measures. Tumour Biol 2016; DOI: 10.1007/s13277-016-5168-x.
    PubMedGoogle Scholar
  • 52 Konieczka K, Fränkl S. Primäre vaskuläre Dysregulation und Glaukom (Primary vascular dysregulation and glaucoma). Z prakt Augenheilkd 2013; 34: 207-215
    PubMedGoogle Scholar
  • 53 Mozaffarieh M, Konieczka K, Flammer J. Calcium channel blockers: their use in normal tension glaucoma. Expert Rev Ophthalmol 2010; 5: 617-625
    CrossrefPubMedGoogle Scholar
  • 54 Shin S, Kim KJ, Cho IJ et al. Effect of Triflusal on primary vascular dysregulation compared with Aspirin: a double-blind, randomized, crossover trial. Yonsei Med J 2015; 56: 1227-1234
    CrossrefPubMedGoogle Scholar
  • 55 Kaiser HJ, Stumpfig D, Flammer J. Short-term effect of dipyridamole on blood flow velocities in the extraocular vessels. Int Ophthalmol 1995; 19: 355-358
    PubMedGoogle Scholar
  • 56 Cybulska-Heinrich AK, Mozaffarieh M, Flammer J. Ginkgo biloba: an adjuvant therapy for progressive normal and high tension glaucoma. Mol Vis 2012; 18: 390-402
    PubMedGoogle Scholar