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Abstracts

Long-term Developmental Outcome in Infantile Epileptic Spasm Syndrome After High-dose Prednisolone and Vigabatrin Treatment

Abstract number : 2.186
Submission category : 4. Clinical Epilepsy / 4D. Prognosis
Year : 2023
Submission ID : 947
Source : www.aesnet.org
Presentation date : 12/3/2023 12:00:00 AM
Published date :

Authors :
Presenting Author: soyoung jang, MD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea

Hui-Jin Shin, MD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea; Ara Ko, MD/PhD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea; Se Hee Kim, MD/PhD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea; Joon Soo lee, MD/PhD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea; heung Dong Kim, MD/PhD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea; Hoon chul Kang, MD/PhD – Division of Pediatric Neurology, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Epilepsy Research Institute, Seoul, Korea

Rationale: The objective of this study was to assess the efficacy of a treatment protocol using vigabatrin followed by high-dose prednisolone for West syndrome (WS), also known as infantile spasms. WS is known for its detrimental effect on a child's development. The study aimed to evaluate the effectiveness of these therapies in controlling spasms, stabilizing electroencephalography (EEG), and enabling long-term (three years) developmental catch-up.

Methods: Patients diagnosed with WS were initially treated with vigabatrin alone for two weeks. If patients did not respond to vigabatrin, prednisolone was administered in combination with vigabatrin. The drug administration protocol consisted of: vigabatrin 50 mg/kg/day for 1 day, followed by vigabatrin 100 mg/kg/day for three days. If spasms persisted or the burden of amplitudes and epileptiform discharges (BASED) score on EEG was ≥3 on day five, vigabatrin was increased to 150 mg/kg/day. If spasms still persisted or the BASED score was ≥3 on day 14, prednisolone was added at a dose of 40 mg/day. The prednisolone dose was increased to 60 mg/day on day 21 if spasms persisted or the BASED score was ≥3.

Results: Seventy newly diagnosed patients with WS (median seizure onset age: 5.7 months) underwent the vigabatrin and prednisolone therapy protocol. Among these patients, 10 (14.3%) showed resolution of spasms and a BASED score of ≤2 after vigabatrin alone, while 46 (65.7%) achieved resolution of spasms and a BASED score of ≤2 after the combination of vigabatrin and prednisolone. Overall, 56 (80%) patients responded to the protocol without experiencing relapse for at least seven months following WS diagnosis. However, 18 (32%) of the initially responsive patients experienced seizure recurrence during the three year follow-up period. Patients with identified structural or genetic etiologies had a significantly higher recurrence rate compared to those with unknown etiology. Responsive patients exhibited higher mental and psychomotor age quotients at the time of diagnosis, and these remained significantly higher at three years of age. No serious adverse reactions leading to discontinuation or dosage reduction of the drugs were observed.

Conclusions: The study concluded that the vigabatrin and high-dose prednisolone add-on protocol for West syndrome is both safe and effective. Approximately half of the patients achieved controlled spasms and EEG improvement. However, during long-term follow-up, around 30% of the initial responders experienced relapse, particularly among patients with identified structural or genetic causes. Long-term developmental function showed improved cognitive and motor development outcomes in responsive patients.

Funding: None

Clinical Epilepsy