The Use of Ketogenic Diet During Pregnancy to Treat NORSE
Abstract number :
3.339
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2024
Submission ID :
634
Source :
www.aesnet.org
Presentation date :
12/9/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Sarika Sewak, RDN – University of California Los Angeles
Beck Reyes, CPNP, CNRN – University of California Los Angeles
joyce matsumoto, MD – University of California Los Angeles
Amy Schnabel, RDN – University of California Los Angeles
Jennifer White, RDN – University of California Los Angeles
Rationale: Effective treatments for new onset refractory status epilepticus (NORSE) are limited, but include the ketogenic diet (KD). However, implementation in the setting of pregnancy is challenging and poses ethical questions which must be considered.
Methods: We present a 29 y.o.pregnant female (gestational age 20 weeks) super-refractory NORSE of possible autoimmune etiology. Seizure focus was localized in in the left frontotemporal region.,MRI was initially unremarkable, with later appearance of diffusion restriction scattered throughout the left hemisphere. After failure of multiple antiseizure medications including lamotrigine, levetiracetam, lacosamide, perampanel and zonisamide, as well as burst suppression with propofol, midazolam and ketamine infusions, corticosteroids, intravenous gammaglobulins and plasmapheresis, KD therapy was initiated. In pregnancy, there are no known contraindications to restrict carbohydrates and provide high fat however there is a minimum protein requirement of 71gm/day (0.8gm/kg) in order to promote fetal growth, limiting the achievable KD ratio to 2.75:1.
Results: Due to concurrent pentobarbital infusion, at a 2.75 ratio therapeutic ketosis could not be achieved with beta-hydroxybutyrate (BHB) level only up to 0.4 mmol/L. Discussion was initiated by high-risk obstetrics with the father of baby (FOB), regarding the effects of multiple potential teratogenic medications and further protein restriction to maximize KD therapy. Termination of pregnancy or early delivery was discussed with FOB, however he deferred a decision. At 23 weeks, spontaneous delivery occurred with fetal demise. Subsequently postpartum, KD was reinitiated with maximum ratio 4.25:1, achieving BHB ranging 1.2-3.4 mmol/L.
Conclusions: KD therapy is one of a paucity of potentially effective treatments for NORSE, but in the setting of pregnancy therapeutic ketosis may not be achievable due to minimum protein requirements. Because other NORSE treatments also have teratogenic potential, a multidisciplinary discussion of the risks and benefits of all treatments, including possible short term protein restriction to maximize KD therapy, should be initiated early with family.
Funding: None
Clinical Epilepsy