Research Article
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Retrospective analysis of patients with West Syndrome in a newly established pediatric neurology clinic

Year 2020, Volume: 6 Issue: 2, 161 - 166, 30.08.2020
https://doi.org/10.30569/adiyamansaglik.710597

Abstract

Aim: West syndrome (WS), is characterized by spasm seizures accompanied by a pattern of hypsarrhythmia on electroencephalography in the interictal period, leading to severe mental retardation.
Materials and Methods: The study included 12 patients admitted to the Pediatric Neurology Clinic of Adıyaman University Hospital between 01 April 2018 and 30 June 2019 and diagnosed with West Syndrome as a result of electroencephalographic study and clinical findings.
Results: Of the 12 patients included in the study, 7 were female (58.4%) and 5 (41.6%) were male. The common finding on physical examinations of all patients was hypotonicity and coarse/dysmorphic facial appearance.
Conclusion: During the nine follow-up, patients with West syndrome could not be kept under control despite multiple antiepileptic drugs, and varying degrees of psychomotor retardation were observed in these cases.

References

  • 1.Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev. 2014;36:739–51.
  • 2.Wilmshurst JM, Ibekwe RC, O’ Callaghan FJK. Epileptic spasms 175 years on: trying to teach and old dog new tricks. Seizure. 2017;44:81–6.
  • 3.Panayiotopoulos CP. A Clinical Guide to Epileptic Syndromes and their Treatment. 2nd edition. London: Springer; 2011.
  • 4.Heyne HO,Singh T,Stamberger H, et al. De novo variants in neurodevelopmental disorders with epilepsy. Nat. Genet. 2018;50:1048-1053.
  • 5.Osborne JP, Lux AL, Edwards SW, et al. The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infan- tile Spams Study (UKISS) on contemporary causes and their classification. Epilepsia. 2010;51:2168–74.
  • 6.Kossof EH. Infantile spasms. Neurologist. 2010;2:69–75.
  • 7.Gibbs FA, Gibbs EL. Atlas of Electroencephalography, 2nd ed. Addison-Wesley Press. Cambridge, Massachusetts Inc; 1952:222-224.
  • 8.Alonzo RD,Rigante D, Mencaroni E, Esposito S. West Syndrome: A Review and Guide for Paediatricians. Clin Drug Investig. 2018;38:113–124.
  • 9.Pellock JM, Hrachovy R, Shinnar S, et al. Infantile spasms: a U.S. consensus report. Epilepsia. 2010;51(10):2175–89.
  • 10.Shu X-M, Li J, Zhang G-P, Mao Q. A comparative study of conventional dose and low dose adrenocorticotrophic hormone therapy for West syndrome. J Contemp Pediatr. 2009;11(6):445–8.
  • 11.Kondo Y, Okumura A, Watanabe K, et al. Comparison of two low dose ACTH therapies for West syndrome: their efficacy and side effect. Brain Dev. 2005;27(5):326–30.
  • 12.Fejerman N, Cersósimo R, Caraballo R, et al. Vigabatrin as a first-choice drug in the treatment of West syndrome. J Child Neurol. 2000;15(3):161–5.
  • 13.Hrachovy RA, Frost JD. Severe encephalopathic epilepsy in infants: infantile spasms (West syndrome). In. Pellock JM, Boorgeois BF, Dodson WE, Nordi DR Jr, Sankar R. Pediatric Epilepsy: Diagnosis and Therapy. Demos Medical Publishing. New York, NY: Inc; 2008:249.
  • 14.Sillanpaa M, Shinnar S. SUDEP and other causes of mortality in childhood onset epilepsy. Epilepsy Behav. 2013;28:249–255.

Yeni kurulan bir çocuk nöroloji kliniğinde West Sendrom’lu olguların retrospektif olarak değerlendirilmesi

Year 2020, Volume: 6 Issue: 2, 161 - 166, 30.08.2020
https://doi.org/10.30569/adiyamansaglik.710597

Abstract

Amaç: West sendromu (WS), spazm şeklindeki nöbetlere elektroensefalografide interiktal dönemde hipsaritmi paterninin eşlik etmesi ve ağır mental geriliğe yol açması ile tanımlanmaktadır.
Gereç ve Yöntem: 01 Nisan 2018-30 Haziran 2019 tarihleri arasında Adıyaman Üniversitesi Eğitim ve Araştırma Hastanesi, Çocuk Nöroloji Polikliniğine başvuran ve elektroensefalografik inceleme ve klinik bulgular ile West Sendromu tanısı konulan 12 hasta çalışmaya dâhil edildi.
Bulgular: Çalışmaya alınan 12 hastanın 7’si kız (%58,4), 5’i (%41,6) erkek idi. Bütün hastaların fizik muayanesindeki ortak bulgu; hipotonisite ve kaba/dismorfik yüz görünümü idi.
Sonuç: Dokuz ay süreli izlemde West Sendrom’lu hastalarda çoklu antiepileptik ilaç kullanılmasına rağmen nöbetleri kontrol altına alınamadı ve bu olgularda değişen derecelerde psikomotor gerilik izlendi.

References

  • 1.Pavone P, Striano P, Falsaperla R, Pavone L, Ruggieri M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev. 2014;36:739–51.
  • 2.Wilmshurst JM, Ibekwe RC, O’ Callaghan FJK. Epileptic spasms 175 years on: trying to teach and old dog new tricks. Seizure. 2017;44:81–6.
  • 3.Panayiotopoulos CP. A Clinical Guide to Epileptic Syndromes and their Treatment. 2nd edition. London: Springer; 2011.
  • 4.Heyne HO,Singh T,Stamberger H, et al. De novo variants in neurodevelopmental disorders with epilepsy. Nat. Genet. 2018;50:1048-1053.
  • 5.Osborne JP, Lux AL, Edwards SW, et al. The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infan- tile Spams Study (UKISS) on contemporary causes and their classification. Epilepsia. 2010;51:2168–74.
  • 6.Kossof EH. Infantile spasms. Neurologist. 2010;2:69–75.
  • 7.Gibbs FA, Gibbs EL. Atlas of Electroencephalography, 2nd ed. Addison-Wesley Press. Cambridge, Massachusetts Inc; 1952:222-224.
  • 8.Alonzo RD,Rigante D, Mencaroni E, Esposito S. West Syndrome: A Review and Guide for Paediatricians. Clin Drug Investig. 2018;38:113–124.
  • 9.Pellock JM, Hrachovy R, Shinnar S, et al. Infantile spasms: a U.S. consensus report. Epilepsia. 2010;51(10):2175–89.
  • 10.Shu X-M, Li J, Zhang G-P, Mao Q. A comparative study of conventional dose and low dose adrenocorticotrophic hormone therapy for West syndrome. J Contemp Pediatr. 2009;11(6):445–8.
  • 11.Kondo Y, Okumura A, Watanabe K, et al. Comparison of two low dose ACTH therapies for West syndrome: their efficacy and side effect. Brain Dev. 2005;27(5):326–30.
  • 12.Fejerman N, Cersósimo R, Caraballo R, et al. Vigabatrin as a first-choice drug in the treatment of West syndrome. J Child Neurol. 2000;15(3):161–5.
  • 13.Hrachovy RA, Frost JD. Severe encephalopathic epilepsy in infants: infantile spasms (West syndrome). In. Pellock JM, Boorgeois BF, Dodson WE, Nordi DR Jr, Sankar R. Pediatric Epilepsy: Diagnosis and Therapy. Demos Medical Publishing. New York, NY: Inc; 2008:249.
  • 14.Sillanpaa M, Shinnar S. SUDEP and other causes of mortality in childhood onset epilepsy. Epilepsy Behav. 2013;28:249–255.
There are 14 citations in total.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Research Article
Authors

Hilal Aydın 0000-0002-2448-1270

İbrahim Bucak 0000-0002-3074-6327

Publication Date August 30, 2020
Submission Date March 28, 2020
Acceptance Date May 1, 2020
Published in Issue Year 2020 Volume: 6 Issue: 2

Cite

AMA Aydın H, Bucak İ. Yeni kurulan bir çocuk nöroloji kliniğinde West Sendrom’lu olguların retrospektif olarak değerlendirilmesi. ADYÜ Sağlık Bilimleri Derg. August 2020;6(2):161-166. doi:10.30569/adiyamansaglik.710597