Neuropediatrics 2019; 50(S 02): S1-S55
DOI: 10.1055/s-0039-1698210
Poster Presentations
Poster Area GNP Epilepsy 1
Georg Thieme Verlag KG Stuttgart · New York

Second Treatment in Infants with West Syndrome: The Effectiveness of the Ketogenic Diet versus ACTH and Other Treatment Options

Anastasia Dressler
1   Kinder- und Jugendheilkunde, Kinder- und Jugendheilkunde, Wien, Austria
,
Chiara Häfele
2   Universitätsklinik für Kinder- und Jugendheilkunde, Medizinische Universität Wien, Neonatologie und Neuropädiatrie, Wien, Austria
,
Petra Trimmel-Schwahofer
2   Universitätsklinik für Kinder- und Jugendheilkunde, Medizinische Universität Wien, Neonatologie und Neuropädiatrie, Wien, Austria
,
Franz Benninger
3   Medizinische Universität Wien, Kinder- und Jugendpsychiatrie, Vienna, Austria
,
Barbara Porsche
2   Universitätsklinik für Kinder- und Jugendheilkunde, Medizinische Universität Wien, Neonatologie und Neuropädiatrie, Wien, Austria
,
Christoph Male
1   Kinder- und Jugendheilkunde, Kinder- und Jugendheilkunde, Wien, Austria
› Author Affiliations
Further Information

Publication History

Publication Date:
11 September 2019 (online)

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Aim: To assess in infants with West Syndrome after failure of first treatment with either the ketogenic diet (KD) or standard adrenocorticotropic hormone (ACTH), the effectiveness of successive treatments with ACTH, KD, or antiepileptic drugs (AEDs).

Method: This is a per protocol follow-up study of infants with WS after failure to the first treatment with KD or ACTH in a parallel-cohort randomized controlled trial. Infants were switched to the respective other treatment (ACTH or KD), or if impossible to AEDs for second treatment, and if failing to respond, to AEDs as third treatment. Study outcome was the frequency of sustained seizure freedom until last follow-up, assessed in response to second treatment, and cumulative responses after first and second treatment.

Results: 101 infants were included into the study. Overall, 34% of infants showed sustained seizure freedom in response to first treatment, 31% to the second and 15% to the third treatment. Cumulative response after third treatment was 61%. KD as second treatment achieved sustained seizure freedom in 50%, ACTH in 19%, and AEDs in 9%. Children eligible for epilepsy surgery as second treatment achieved sustained seizure freedom in 80%. After a third treatment with AEDs, cumulative response after a KD-ACTH sequence was 78%, and 72% after an ACTH-KD sequence. Subgroup analysis with Vigabatrin showed second KD treatment to be more effective than ACTH, but no difference for third treatment with AEDs.

Interpretation: The KD is at least as effective as ACTH as second treatment and significantly more effective than AED in achieving sustained seizure freedom.