Abstract
Purpose
We aimed to evaluate choice and efficacy of intravenous antiepileptic drugs (AEDs)
for status epilepticus (SE) in Dravet syndrome and to find predictable clinical features
demonstrating the effectiveness of benzodiazepine (BZD) for SE.
Methods
We retrospectively investigated the medical records in patients with Dravet syndrome
and evaluated the effectiveness rate of intravenous AEDs and the rate of adverse effects.
To find the clinical features of BZD-effective SE, we divided the SE episodes into
the following two groups: BZD effective group and BZD non-effective group. The choice
of treatment was dependent on physicians’ discretion according to the protocol for
SE in our institution.
Results
Sixty-eight SE episodes in 10 patients were assessed. The median age at SE was 31 months.
Of 68 episodes, 42 episodes (61.8%) were in the BZD effective group and 26 (38.2%)
in the BZD non-effective group. There were no significant differences in clinical
features. In the BZD non-effective group, the effective rates of continuous midazolam,
phenobarbital, phenytoin/fosphenytoin were 9/9 episodes (100%), 14/17 (82.4%), and
2/5 (40.0%), respectively. Adverse effects were identified in 19/68 episodes (27.9%),
including 11/42 episodes in the BZD effective group and 8/26 in the BZD non-effective
group, which was no statistical difference between the two groups. Respiratory suppression
was found in all 19 episodes and the incidence of endotracheal intubation in the BZD
non-effective group (15.4%) was higher than that in the BZD effective group (2.4%)
(p = 0.046).
Conclusion
BZD may be used as first choice, and phenobarbital prior to continuous midazolam as
second choice for SE with Dravet syndrome. There might be no predictable clinical
features showing that BZD will be effective.
Keywords
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Article info
Publication history
Published online: February 01, 2022
Accepted:
January 16,
2022
Received in revised form:
December 28,
2021
Received:
June 19,
2021
Identification
Copyright
© 2022 The Japanese Society of Child Neurology Published by Elsevier B.V. All rights reserved.
