Abstracts

Rationale: Introduction:
Lennox- Gastaut Syndrome (LGS) is a childhood-onset epileptic encephalopathy that is often highly refractory to multiple treatments. There is mixed efficacy regarding the optimal combination of pharmacologic management options. While benzodiazepines (BZDs) are widely used across epilepsy types, in LGS, they carry the risk of worsening tonic seizures. Although there is better tolerability of clobazam (CLB) compared to short acting BZDs, its effect in LGS specifically is poorly understood. Current literature is equivocal regarding the efficacy of lacosamide (LAC).


Methods: Case:

We have a 16 yo M with LGS who presented with acute repetitive generalized tonic seizures secondary to suboptimal medication adherence on dual regimen of levetiracetam (LEV) and lamotrigine (LTG). Notable history includes MRI notable for scattered T2 hyperintensities in the periventricular and pericallosal regions. Additionally, he was notably previously treated with valproate (VPA) but had discontinued due to adverse metabolic side effects. He had poor response to initial abortive BZDs, fosphenytoin and VPA loads and was ultimately preemptively intubated and placed on a midazolam drip with escalating doses (to maximum of 20 mg/hr). Although clinical seizures stopped, he electrographically remained on the ictal-interictal continuum. Short acting BZDs resulted in electrographic worsening of seizures. LTG was discontinued and CLB was added without significant improvement. His initial VPA loads resulted with transient quiescence of his ictal pattern and was added back to his regimen. He continued to have frequent generalized tonic seizures lasting 60-90 seconds occuring 20 times over 30-60 minutes thus loaded with LAC and started standing dose. LAC stopped all seizures upon initiation; however, complicated initially by excessive sedation that took days to normalize.


Results: Conclusion:

This case study of a teenager with LGS presenting with intractable acute repetitive seizures who notably had great efficacy with the load and addition of LAC to his regimen. Following his acute presentation, he had complete cessation of his seizures once initiated on LAC. Once seizures recurred, he had a modest decrease in the frequency and duration of his generalized tonic seizures. Upon discharge, he was stable on his current regimen of LEV, LAC, and VPA. Genetic testing is pending to garner greater understanding in this patient. Although there is mixed data regarding the efficacy of LAC in treatment of LGS, this case report supports favorable outcome with adjuvant treatment of LAC in refractory cases. However, he remains at elevated risk for SUDEP and is undergoing surgical evaluation for Vagal Nerve Stimulator placement.


Conclusions:

References:

Panda et al., Efficacy and Tolerability of Lacosamide in Lennox-Gastaut Syndrome: A Systematic Review and Meta-analysis. J Neurosci Rural Pract. 2022 Jan 7;13(1):32-42.



van Rijckevorsel K. Treatment of Lennox-Gastaut syndrome: overview and recent findings. Neuropsychiatr Dis Treat. 2008 Dec;4(6):1001-19.


Funding: There was no funding for this study.