VESTA SYNDROME: ETIOLOGY AND EARLY DIAGNOSTIC SIGNS. LITERATURE REVIEW
Introduction: Hypsarrhythmia is manifested by chaotic asynchronous spikes with a high amplitude, mainly with slow-wave activity. This electroencephalographic pattern was first noted among a large number of patients with infantile spasms. The triad of symptoms: infantile spasms, delayed psychomotor development and hypsarrhythmia on electroencephalography was called West syndrome, which has a gradual development both in healthy children and against the background of psychomotor delay. West's syndrome is a severe variant of epileptic diseases in children due to complex therapeutic tactics and impaired mental development. Determination of etiological causes and early diagnostic signs contributes to a more accurate prognosis and choice of therapy.
Objective: analyze and systematize publications devoted to the study of the etiology and early diagnostic signs of West syndrome.
Results: In the scientific literature, along with the diagnosis of West syndrome, terms such as infantile spasms or epileptic spasms are used. In recent years, significant advances have been made in all significant and less common aspects of the disorder, including its nomenclature, etiology, associated genetic factors, clinical diversity and complex phenotypes, as well as treatment and prognosis. West syndrome is characterized by a combination of cluster spasms, hypsarrhythmia on an electroencephalogram, and psychomotor regression. Treatment should be started as soon as possible after diagnosis, especially during infancy, to maximize seizure control and improve long-term outcomes.
Conclusions: Predictors of an unfavorable outcome of West syndrome can be considered: the structural and genetic form of the disease, neonatal seizures, the presence of epileptiform activity on electroencephalography, impaired neuropsychic development and the presence of focal pathology before spasms, the presence of seizures other than spasms, persistence of epileptiform activity in dynamics, ineffectiveness of the first therapy lines.
Bibigul A. Abdygalyk1, https://orcid.org/0000-0001-8171-6112
Nazira A. Zharkinbekova2, https://orcid.org/0000-0002-5069-1562
Marzhan M. Lepesova3, Latina A. Tekebaeva1, Elena D. Belousova4
1 Kazakhstan Medical University “KSPH”, Almaty c., Republic of Kazakhstan;
2 South Kazakhstan Medical Academy, Shymkent c., Republic of Kazakhstan;
3 Kazakh medical university of continuing education, Almaty c., Republic of Kazakhstan;
4 The Scientific Research Institute of Pediatrics of the Russian National Research Medical University named N.I. Pirogov, the Department of Psychoneurology and Epileptology, Moscow с., Russian Federation.
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Abdygalyk B.A., Zharkinbekova N.A., Lepesova M.M., Tekebaeva L.A., Belousova E.D. Vesta syndrome: etiology and early diagnostic signs. Literature review // Nauka i Zdravookhranenie [Science & Healthcare]. 2022, (Vol.24) 1, pp. 157-166. doi 10.34689/SH.2022.24.1.018Related publications:
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