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Epilepsia and paroxyzmal conditions

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WEST SYNDROME REVISITED

https://doi.org/10.17749/2077-8333.2017.9.2.074-090

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Abstract

West syndrome is an epileptic syndrome, characterized by a triad of symptoms: infantile spasms, hypsarrhythmia on interictal EEG, and a regression or delay of psycho-motoric development. According to the current concept, two of these three criteria suffice for the diagnosis. The incidence of this syndrome is 1 per 2000 newborns. This article reviews recent publications on the etiology, pathogenesis, clinical course, diagnosis, and treatment options in West syndrome. The text is supplemented with illustrations of ictal EEG at infantile spasms and variants of hypsarrhythmia. Today, in addition to classical hypsarrhythmia, five versions of modified hypsarrhythmia are proposed: synchronized version, asymmetric version, version with stable focus of spikes or acute waves, version with rhythmic flattening episodes, version with high-amplitude and asynchronous slow activity. The hormonal therapy is recommended to begin the treatment; if not effective, vigabatrin is to be used as the second choice medication.

About the Authors

T. G. Okhapkina
Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation
Russian Federation

Junior Researcher, the Department of Psychoneurology and Epileptology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997



Z. K. Gorchanova
Russian Children’s Clinical Hospital, Health Ministry of Russian Federation
Russian Federation

neurologist, the Department of Psychoneurology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997



I. V. Shulyakova
Russian Children’s Clinical Hospital, Health Ministry of Russian Federation
Russian Federation

neurologist, the Department of Psychoneurology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997



E. S. Ilyina
Russian Children’s Clinical Hospital, Health Ministry of Russian Federation
Russian Federation

Head, the Department of Psychoneurology, Russian Children’s Clinical Hospital, Health Ministry of
Russian Federation

Address: Leninskii pr-t, 117, Moscow, Russia, 119571



E. S. Michurina
Russian Children’s Clinical Hospital, Health Ministry of Russian Federation
Russian Federation

neurologist, the Department of Psychoneurology, Russian Children’s Clinical Hospital, Health Ministry of Russian Federation

Address: Leninskii pr-t, 117, Moscow, Russia, 119571



E. D. Belousova
Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation
Russian Federation

Head, the Department of Psychoneurology and Epileptology, Russian National Research Medical University named after N.I. Pirogov, Health Ministry of Russian Federation

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997



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For citation:


Okhapkina T.G., Gorchanova Z.K., Shulyakova I.V., Ilyina E.S., Michurina E.S., Belousova E.D. WEST SYNDROME REVISITED. Epilepsia and paroxyzmal conditions. 2017;9(2):74-90. (In Russ.) https://doi.org/10.17749/2077-8333.2017.9.2.074-090

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ISSN 2077-8333 (Print)
ISSN 2311-4088 (Online)