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A Rare Case of Idiopathic Harlequin Syndrome

Year 2019, Issue: 4, 779 - 781, 01.12.2019

Abstract

Harlequin Syndrome is a rare malady of the sympathetic nervous system which is accompanied by unilateral anhydrosis and dull skin of the face, contralateral hyperhidrosis and flushing. It is a benign autonomic dysfunction which becomes more pronounced with environmental and/or emotional stress and generally requires treatment. Although traumatic and degenerative processes lead to the syndrome, mostly there are no etiologic factors. A 42 years-old-woman presented with hyperhidrosis and flushing of unilateral of the face without ocular findings ptosis, myosis, etc. , in the time of stress and/or thermo-rising. There was no history of trauma or surgery. Magnetic resonance imaging of the brain, cervical spine or thoracal spine were normal. A complaint becomes to the level of interfering with social intercourse/life so the patient was referred to sympathetic ganglion blockage for symptomatic treatment. We present the case to aim or The aim of presenting this case is to call attention to a rarely encountered case of idiopathic harlequin syndrome

References

  • Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988;51:635–42. [CrossRef]
  • Kaur S, Aggarwal P, Jindal N, Dayal S, Jairath V, Jain VK, Virdi S. Harlequin syndrome: a mask of rare dysautonomic syndromes. Dermatol Online J 2015;21. pii: 13030/qt3q39d7mz.
  • Boling B, Key CC, Wainscott J, Rebel AE. Harlequin Syndrome as a Complication of Epidural Anesthesia. Crit Care Nurse 2014;34:57–61. [CrossRef]
  • Willaert WIM, Scheltinga MRM, Steenhuisen SF, Hiel JA. Harlequin Syndrome: two new cases and a management proposal. Acta Neurol Belg 2009;109:214–20.
  • Bremner F, Smith S. Pupillographic Findings in 39 Consecutive Cases of Harlequin Syndrome. J Neuro-Ophthalmol 2008;28:171–7. [CrossRef]
  • Drummond PD, Lance JW. Site of autonomic deficit in Harlequin syndrome: local autonomic failure affecting the arm and the face. Ann Neurol 1993;34:814–9. [CrossRef]
  • Jain P, Arya R, Jaryal A, Gulati S. Idiopathic harlequin syndrome: a pediatric case. J Child Neurol 2013;28:527–30. [CrossRef]
  • Wasner G, Maag R, Ludwig J, Binder A, Schattschneider J, Stingele R, Baron R.. Harlequin syndrome —one face of many etiologies. Nat Clin Pract Neurol 2005;1:54–9. [CrossRef]
  • Reddy H, Fatah S, Gulve A, Carmichael AJ. Novel management of harlequin syndrome with stellate ganglion block. Br J Dermatol 2013;169:954–6. [CrossRef] 1
  • Manhães RK, Spitz M, Vasconcellos LF. Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord 2016;23:112–3. [CrossRef]

İdyopatik Harlequin Sendromlu Nadir Bir Olgu

Year 2019, Issue: 4, 779 - 781, 01.12.2019

Abstract

Harlequin sendromu, yüzde unilateral anhidrozis ve mat cilt rengine kontralateral hiperhidrozis ve kızarıklığın eşlik ettiği nadir bir sempatik sinir sistemi hastalığıdır. Çevresel ve/veya emosyonel stres ile belirginleşen hastalık, çoğunlukla tedavi gerektirmez ve benign bir otonom disfonksiyondur. Travmatik ya da dejeneratif süreçler tabloya yol açsa da çoğunlukla etyolojik neden bulunamaz. 42 yaşındaki kadın olgumuz, ısı artışı ve stres anında, göz bulgularının ptozis, anormal pupil vb. eşlik etmediği, yüz sağ yarımında aşırı terleme, kızarma ile başvurmuşdu. Travma veya cerrahi girişim öyküsü olmayan hastanın beyin, servikal ve torakal görüntülemelerinde etyolojik bir neden saptanmadı. Şikayetleri sosyal ilişkilerini etkileyecek düzeye ulaşan hasta semptomatik tedavi amacıyla sempatik ganglion blokajına yönlendirildi. Olgumuz nadir olarak karşılaşılan idyopatik harlequin sendromuna dikkat çekmek amacıyla sunulmuştur

References

  • Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988;51:635–42. [CrossRef]
  • Kaur S, Aggarwal P, Jindal N, Dayal S, Jairath V, Jain VK, Virdi S. Harlequin syndrome: a mask of rare dysautonomic syndromes. Dermatol Online J 2015;21. pii: 13030/qt3q39d7mz.
  • Boling B, Key CC, Wainscott J, Rebel AE. Harlequin Syndrome as a Complication of Epidural Anesthesia. Crit Care Nurse 2014;34:57–61. [CrossRef]
  • Willaert WIM, Scheltinga MRM, Steenhuisen SF, Hiel JA. Harlequin Syndrome: two new cases and a management proposal. Acta Neurol Belg 2009;109:214–20.
  • Bremner F, Smith S. Pupillographic Findings in 39 Consecutive Cases of Harlequin Syndrome. J Neuro-Ophthalmol 2008;28:171–7. [CrossRef]
  • Drummond PD, Lance JW. Site of autonomic deficit in Harlequin syndrome: local autonomic failure affecting the arm and the face. Ann Neurol 1993;34:814–9. [CrossRef]
  • Jain P, Arya R, Jaryal A, Gulati S. Idiopathic harlequin syndrome: a pediatric case. J Child Neurol 2013;28:527–30. [CrossRef]
  • Wasner G, Maag R, Ludwig J, Binder A, Schattschneider J, Stingele R, Baron R.. Harlequin syndrome —one face of many etiologies. Nat Clin Pract Neurol 2005;1:54–9. [CrossRef]
  • Reddy H, Fatah S, Gulve A, Carmichael AJ. Novel management of harlequin syndrome with stellate ganglion block. Br J Dermatol 2013;169:954–6. [CrossRef] 1
  • Manhães RK, Spitz M, Vasconcellos LF. Botulinum toxin for treatment of Harlequin syndrome. Parkinsonism Relat Disord 2016;23:112–3. [CrossRef]
There are 10 citations in total.

Details

Primary Language Turkish
Journal Section Case Report
Authors

Saadet Sayan

Publication Date December 1, 2019
Published in Issue Year 2019Issue: 4

Cite

EndNote Sayan S (December 1, 2019) İdyopatik Harlequin Sendromlu Nadir Bir Olgu. Acıbadem Üniversitesi Sağlık Bilimleri Dergisi 4 779–781.