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Dramatic Response to Neurostimulation in Children With Medically Intractable Epilepsy Related to Pseudoisodicentric
Trevor Lockard1, Sookyong Koh1, Drew M Thodeson1
1Division of Neurology Omaha, Department of Pediatrics, NE Children's Nebraska, University of Nebraska Medical Center, Omaha, Nebraska.
Background:
15q11-q13 duplications (dup15q syndrome) in children with neurodevelopmental disorder may present with variable epilepsy phenotypes. The more common pseudoisodicentric or isodicentric chromosome 15 duplication often presents with medically refractory epilepsy. This case series illustrates 3 cases with dramatic response to neurostimulation in dup15q syndrome with medically refractory epilepsy.
Methods:
We present 3 clinical cases analyzed by querying the medical record. Demography, medical history, and treatment efficacy were systematically reported and analyzed.
Results:
All 3 cases showed dramatic response to neurostimulation where medication management failed. Treatment responses ranged from greater than 90% reduction in seizure frequency to seizure freedom. Moreover, all patients showed clinically significant developmental gains.
Conclusions:
Neurostimulation produced dramatic seizure reduction in our cohort of dup15q syndrome. Early neurostimulation should be considered in medically refractory epilepsy in dup15q syndrome. Further clinical trials investigating the efficacy of neurostimulation will be helpful in this rare neurodevelopmental condition.
