Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience

Authors

  • Naim Zeka Department for Neurology, Paediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo
  • Abdurrahim Gerguri Department for Neurology, Paediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo
  • Ramush Bejiqi Department for Cardiology, Paediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo
  • Ragip Retkoceri Department for Cardiology, Paediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo
  • Armend Vuciterna Department for Neurology, Paediatric Clinic, University Clinical Centre of Kosovo, Prishtina, Kosovo

DOI:

https://doi.org/10.3889/oamjms.2017.192

Keywords:

West Syndrome, Hypsarythmia, Epileptic encephalopathy, Infantile spasm

Abstract

BACKGROUND: West Syndrome (WS) represents as a specific epileptic encephalopathy characterised with a unique type of attacks, called infantile spasms, severe forms of abnormalities in electroencephalographic (EEG) records as a hypsarythmias and delays in the psychomotoric development. The characteristics of the disease, mostly affecting male gender, are infantile spasms and typical findings in EEG as a hypsarythmia. Infantile spasms are a consequence of many factors in the undeveloped brain.

AIM:  We aimed: (1) to see the incidence of the illness and the spreading out because of gender in rapport   with other syndromes in the epileptic encephalopathies group; (2) to show principles of the treatment for the illness; and (3) to present the effects of the disease in the psycho-motoric development of affected children.

METHODS: The study was designed as a cross-sectional study of the patients with epileptic encephalopathies, treated in Paediatric Clinic in Prishtina, from 1st of January 2013 until the 31st of December 2015. 

RESULTS: From the cohort group of 97 children diagnosed with epileptic encephalopathies, in 14 of them clinical and EEG signs of WS were noted. The earliest age of disease manifestation was 74 days (± 63.8 days). On the group of children with WS, 13 of them with Natrium Valpropat were treated, with the doses of 301.9 mg (± 64.1). From the cohort group, in 89 children (91.8%) psychomotoric retardation was documented, within the higher reoccurrence in the undifferentiated epileptic encephalopathies (96%) and the WS (78.6%).

CONCLUSION: WS is a frequent disease of the encephalopathies with the epileptogenic framework. The resistance in anticonvulsive therapy is huge, and psychomotoric retardation follows a big percentage of children with this syndrome.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Plum Analytics Artifact Widget Block

References

Chugani HT. Pathophysiology of infantile spasms. Adv Exp Med Biol. 2002;497:111–121.https://doi.org/10.1007/978-1-4615-1335-3_13 PMid:11993727

Primec ZR, Kopac S, Neubauer D. Epidemiologic features of infantile spasms in Slovenia. Epilepsia. 2002;43(2):183-7. https://doi.org/10.1046/j.1528-1157.2002.26201.x PMid:11903466

Brna PM, Gordon KE, Dooley JM, Wood EP. The epidemiology of infantile spasms. Can J Neurol Sci. 2001;28(4):309-12. https://doi.org/10.1017/S0317167100001517 PMid:11766774

Chiemchanya S, Visudtibhan A, Visudhiphan P. West syndrome in Thailand: a hospital-based survey. Brain Dev. 2001;23(7):605-8. https://doi.org/10.1016/S0387-7604(01)00294-7

Wong V.West syndrome – The University of Hong Kong experience (1970-2000). Brain Dev. 2001; 23(7):609-15. https://doi.org/10.1016/S0387-7604(01)00296-0

Thambyayah M. Early epileptic encephalopathies including West syndrome: a 3-year retrospective study from Klang Hospital, Malaysia. Brain Dev. 2001;23(7):603-4. https://doi.org/10.1016/S0387-7604(01)00293-5

Hrachovy RA, Frost JD. Severe Encephalopathic Epilepsy in Infants: Infantile Spasms (West Syndrome). In: Pellock JM, ourgeois BFD, Dodson WE. Undefined. Pediatric Epilepsy. Third Edition. New York, NY: Demos Medical Publishing, 2008:16.

Lux AL, Osborne JP. A proposal for case definitions and outcome measures in studies of infantile spasms and west syndrome: consensus statement of the west Delphi group. Epilepsia. 2004;45:1416–28. https://doi.org/10.1111/j.0013-9580.2004.02404.x PMid:15509243

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(9):739-51. https://doi.org/10.1016/j.braindev.2013.10.008 PMid:24268986

Published

2017-11-28

How to Cite

1.
Zeka N, Gerguri A, Bejiqi R, Retkoceri R, Vuciterna A. Compare Of the West Syndrome with Other Syndromes in the Epileptic Encephalopathy - Kosovo Experience. Open Access Maced J Med Sci [Internet]. 2017 Nov. 28 [cited 2024 Mar. 28];5(7):925-8. Available from: https://oamjms.eu/index.php/mjms/article/view/oamjms.2017.192

Issue

Section

B - Clinical Sciences

Most read articles by the same author(s)