Abstract
Besides gelastic seizures, hypothalamic hamartoma (HH) is also noted for its susceptibility to remote secondary epileptogenesis. Although clinical observations have demonstrated its existence, and a three-stage theory has been proposed, how to determine whether a remote symptom is spontaneous or dependent on epileptic activities of HH is difficult in some cases. Herein, we report a case of new non-gelastic seizures in a 9-year-old female associated with a postoperatively remaining HH. Electrophysiological examinations and stereo-electroencephalography (SEEG) demonstrated seizure onsets with slow-wave and fast activities on the outside of the HH. By using computational methodologies to calculate the network dynamic effective connectivities, the importance of HH in the epileptic network was revealed. After SEEG-guided thermal coagulation of the remaining HH, the patient finally was seizure-free at the 2-year follow-up. This case showed the ability of computational methods to reveal information underlying complex SEEG signals, and further demonstrated the dependent-stage secondary epileptogenesis, which has been rarely reported.
Highlights
As a rare congenital malformation disease, hypothalamic hamartoma (HH) has four major impacts on patients, especially in the pediatric populations: precocious puberty (PP) [1], seizures that are mainly gelastic seizures (GS) [2], cognitive and behavioral impairments [3], and developmental delays [4]
Previous clinical observations have demonstrated the existence of the independent stage, but methods to distinguish this stage once the secondary epileptogenesis emerges have rarely been described [7]
Despite the difficulty of diagnosing the stage of secondary epileptogenesis with information from magnetic resonance imaging (MRI) scans, scalp video electroencephalograms EEGs, and SEEG, use of a novel computational method based on SEEG data suggested the possibility of a dependent stage, and the patient’s seizure control succeeded with SEEG-guided thermal coagulation
Summary
As a rare congenital malformation disease, hypothalamic hamartoma (HH) has four major impacts on patients, especially in the pediatric populations: precocious puberty (PP) [1], seizures that are mainly gelastic seizures (GS) [2], cognitive and behavioral impairments [3], and developmental delays [4]. GS is a hallmark, mostly drug resistant and is verified by stereo-electroencephalography (SEEG) as originating from HH [5, 6]. Due to the observation of multiple other seizure types associated with extra-lesion areas, a hypothesis of secondary epileptogenesis, that persistent seizure activities from HH could induce seizure activities in various neocortical areas, has been suggested [5, 7]. According to Morell’s postulation, secondary epileptogenesis develops in three stages (i.e., dependent, intermediate, and independent) [8, 9]
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