Clinical features and postoperative seizure outcome in patients with drug-resistant gelastic seizures without hypothalamic hamartoma

Gutierrez, Camilo; Asadi‐Pooya, Ali A.; Skidmore, Christopher; Tobochnik, Steven; LoPinto-Khoury, Carla; Sperling, Michael R.

doi:10.1016/j.yebeh.2016.09.038

Cited by 13 publications

(14 citation statements)

References 9 publications

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“…These include inflammatory, infectious, and dysplastic aetiologies, as well as mesial sclerosis and tumours of all cerebral lobes, the pituitary gland, mammillary bodies and the third ventricle (Tassinari et al, 1991; Munari et al, 1995; Kuzniecky et al, 1997). Although mesial temporal lobe sclerosis was not suggested to be an aetiology of GS based on earlier studies, (Tassinari et al, 1991), later series have shown a predominance of mesial sclerosis, ranging from 12.5 to 21% (Kovac et al, 2015; Gutierrez et al, 2016). In our series, the percentage was smaller (7.4%), suggesting that this preponderance in previous studies is most likely related to the reference bias associated with VEEG.…”

Section: Discussionmentioning

confidence: 99%

Gelastic seizures: a retrospective study in five tertiary hospital centres

Amorim

Pereira

Sequeira

et al. 2020

Epileptic Disorders

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Aim: This study aimed to characterize, clinically and neurophysiologically, a series of patients with gelastic seizures (GS), including both adults and children. Methods: We retrospectively collected patients with GS from epilepsy clinics of five tertiary hospital centres within a single country. Patients were selected through relatives’/caregivers’ descriptions, home video and/or video‐EEG monitoring. GS were identified through ictal semiology. Results: Thirty‐five patients were enrolled; 62.9% had initial GS in infancy, 14.3% in adolescence and 22.8% at adult age. Twenty‐six had abnormal MRI: eight presented with hypothalamic hamartoma (HH) and 16 non‐HH lesions that included different structural aetiologies and genetic, metabolic and immune aetiologies. All patients with HH had their first GS in infancy or adolescence. For the remaining aetiologies, GS started in infancy in 59.3%, in adolescence in 11.1% and at adult age in 29.6%. Video‐EEG data was available for analysis in 11 patients, including seven patients with a non‐HH MRI lesion. The ictal onset topography on scalp video‐EEG was usually concordant with the MRI lesion (in 6/7 patients) and the most frequent ictal onset was fronto‐temporal. In two patients, both video‐EEG and MRI suggested a parietal and occipital epileptogenic zone. Conclusion: Aetiologies and patterns of affected topography unrelated to HH are common in patients with GS, and all age groups may manifest with this type of ictal semiology. This ictal manifestation has no lateralizing value and, despite a clear preponderance for hypothalamic, frontal and temporal lobe origins, other brain areas, namely the parietal and occipital lobes, should be considered.

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Section: Discussionmentioning

confidence: 99%

Gelastic seizures: a retrospective study in five tertiary hospital centres

Amorim

Pereira

Sequeira

et al. 2020

Epileptic Disorders

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“…Routine (interictal) recordings range from normal EEG results to multiple interictal epileptiform features demonstrating generalized, multifocal, and/or focal discharges and suggesting that EEG is not a specific test for the diagnosis of HH‐epilepsy disorders (Scholly et al, ). Ictal recordings may be variable too, though GS show no obvious ictal changes in the majority of events (Gutierrez et al, ), non‐GS may show epileptic activity mainly in frontal and temporal lobes (García‐Morales et al, ; Scholly et al, ).…”

Section: Discussionmentioning

confidence: 99%

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

et al. 2019

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IntroductionClinical manifestations of the hypothalamic hamartoma‐epilepsy syndrome (HH‐ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.MethodsRetrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and epilepsy who attended our tertiary Epilepsy Unit between 2003 and 2018. We report the clinical and electroencephalographic features of a series of adult patients with HH and related epilepsy seen in our center together with the treatments and seizure outcome.ResultsWe describe a series of eight patients. Five males (62.5%), median age at evaluation was 28.5 years (IQR: 15.5). Clinical manifestations included focal with preserved and impaired awareness emotional seizures (gelastic seizures [GS]) in six patients (75%), focal tonic, atonic with impaired awareness and focal to bilateral tonic–clonic seizures. Mild GS were the only symptom in one patient. Three patients (37.5%) had endocrinological disturbances such as obesity and hypothyroidism. Fifty percent of the patients showed psychiatric comorbidity such as anxiety disorder and aggressiveness, and two patients had psychomotor delay. Seven patients (87.7%) had drug‐resistant seizures and three of them were treated with radiosurgery. Out of the treated group, only one (33.3%) became seizure‐free 2 years after surgery but developed psychiatric problems. The other two patients had an Engel IV outcome and received a vagal nerve stimulation (VNS) implant. VNS did not lead to changes either in intensity nor in seizure frequency.ConclusionsHypothalamic hamartoma‐epilepsy syndrome clinical manifestations in adult patients are as variable as at pediatric age. Outcome of therapeutic options such as radiosurgery or VNS may be poorer at this stage.

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“…It is now recognized that they are not pathognomonic of HH and can be found in temporal or extratemporal, lesional or non-lesional epilepsy, mostly in adults [2], [3], [4]. However there is a growing body of evidence of such cases in children [5], [6].…”

Section: Discussionmentioning

confidence: 99%

Successful surgical management of New Onset Refractory Status Epilepticus (NORSE) presenting with gelastic seizures in a 3 year old girl

Marashly

Lew

Koop

2017

Epilepsy & Behavior Case Reports

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Gelastic seizures (GS) are typically associated with hypothalamic hamartomas and present during childhood. However it is now known that GS can be found in focal epilepsies arising from other regions in the brain, including mesial and neocortical frontal, temporal and parietal regions.GS have rarely been described as the presenting manifestation of New Onset Refractory Status Epilepticus (NORSE). In this article we describe a previously healthy 3-year-old who presented with an explosive onset of GS that were refractory to multiple anti-seizure medications. These seizures arose from the right frontal region. An extensive metabolic and immunological evaluation was negative. Her brain magnetic resonance imaging (MRI) was negative, however the Positron Emission Tomography (PET) scan showed a hypermetabolic region in the right frontal inferior gyrus.She underwent a depth electrode evaluation that revealed a widespread irritative zone involving the PET “lesion” as well as mesial and neocortical regions in the right frontal lobe. The seizure onset zone was widespread and non-localizable. However the GS were associated with a clear ictal epileptiform discharge on invasive EEG arising from the depth of the superior frontal gyrus, which was not overlapping with the PET hypermetabolic region. She underwent a right frontal lobectomy sparing the primary motor region in the pre-central gyrus. She has remained seizure free for 15 months since. The pathological analysis showed focal cortical dysplasia type II in the region of the PET scan hypermetabolism. This case expands the clinical spectrum of GS to include cases of NORSE. Additionally the case highlights the role of resective surgery in GS presenting as NORSE and the potentially excellent outcome that can be achieved by early intervention.

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Clinical features and postoperative seizure outcome in patients with drug-resistant gelastic seizures without hypothalamic hamartoma

Cited by 13 publications

References 9 publications

Gelastic seizures: a retrospective study in five tertiary hospital centres

Gelastic seizures: a retrospective study in five tertiary hospital centres

Hypothalamic hamartomas in adulthood: Clinical spectrum and treatment outcome—A unicenter experience

Successful surgical management of New Onset Refractory Status Epilepticus (NORSE) presenting with gelastic seizures in a 3 year old girl

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