Contralateral hippocampal sclerosis following functional hemispherectomy in children: A report of three cases.

Seizure

Epilepsy Program, Division of Neurology, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. Electronic address:

Published: October 2023

AI Article Synopsis

  • Hippocampal Sclerosis (HS) can occur alongside other brain lesions in children, leading to persistent seizures even after functional hemispherectomy (FH), a surgical procedure to remove one hemisphere of the brain.
  • A retrospective study identified three children who developed contralateral HS after undergoing FH for unilateral cortical malformations, all of whom continued to experience seizures post-surgery.
  • The research suggests that the underlying mechanisms for contralateral HS in these cases are unclear, but genetic mutations may influence seizure outcomes after epilepsy surgery.

Article Abstract

Purpose: Hippocampal Sclerosis (HS) may co-exist with temporal or extratemporal lesions (dual pathology) in children and is usually ipsilateral to the radiological lesion. Here were report three cases with extensive hemispheric cortical malformation and drug resistant epilepsy who had persistent seizures after functional hemispherectomy (FH) and developed contralateral HS after the surgery.

Methods: This retrospective study enrolled children who underwent FH and developed contralateral HS after surgery. Their clinical, EEG, radiological and pathological data were reviewed and summarized.

Results: Ninety-five children underwent FH during the study period; Three cases (3.2%) were eligible. They all had unilateral extensive hemispheric cortical malformation who underwent FH between 3 and 5 months of age with no clinical, EEG or radiological suggestion for involvement of contralateral hemisphere prior to FH. All three patients had persisting seizures after FH. Contralateral HS was detected between 2.2 to 3.7 years after FH in all three cases. Two of the patients showed pathogenic variants in GATOR1 pathway genes.

Conclusions: The genesis of contralateral HS in the reported patients remains unexplained. The presence and distribution of "second-hit" somatic mutations may play an important role in governing the seizure outcomes of epilepsy surgery in patients with apparently unilateral malformations of cortical development.

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Source
http://dx.doi.org/10.1016/j.seizure.2023.08.014DOI Listing

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