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Journal of Pediatric Epilepsy 2023; 12(04): 140-143
DOI: 10.1055/s-0043-1770052
Case Report

Neonatal Status Epilepticus Secondary to Nonketotic Hyperglycinemia: Efficacy of Low-Dose Dextromethorphan

Sara Vila-Bedmar 
1   Department of Pediatric Neurology, Hospital Universitario 12 de Octubre, Madrid, Spain
,
Maite La-Vega Talbott
2   Department of Pediatric Neurology-Epilepsy Unit, Hospital Mount Sinai, New York, United States
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Abstract

Nonketotic hyperglycinemia is a severe form of early onset epileptic encephalopathy caused by disturbances in the glycine cleavage system, leading to neurological damage attributed to overstimulation of the N-methyl-D-aspartate receptor. Although there are no interventions known to be effective in altering the natural history of nonketotic hyperglycinemia, it is very important that the clinician recognizes this disease and initiates early evaluation and treatment to attain the best possible outcome. Here we present a newborn diagnosed with a severe form of nonketotic hyperglycinemia with frequent myoclonic seizures, which were resistant to phenobarbital, levetiracetam, ketogenic diet, sodium benzoate, and perampanel. Dextromethorphan reduced epileptic myoclonic jerks and improved the background activity on the electroencephalogram.

Keywords

neonatal status epilepticus - EEG - nonketotic hyperglycinemia - dextromethorphan

Note

Permission for publication was obtained from our institution’s Institutional Review Board.




Publication History

Received: 01 March 2023

Accepted: 09 May 2023

Article published online:
19 June 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
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