The Timing, Nature, and Range of Neurobehavioral Comorbidities in Juvenile Myoclonic Epilepsy

Almane, Dace; Jones, Jana E.; McMillan, Taylor; Stafstrom, Carl E.; Hsu, David; Seidenberg, Michael; Hermann, Bruce P.; Oyegbile, Temitayo O.

doi:10.1016/j.pediatrneurol.2019.03.011

Cited by 28 publications

(31 citation statements)

References 55 publications

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“…Antenatal and birth history, and cognition are typically normal, although impairments in specific cognitive domains (e.g., executive functions, attention, decision‐making) can be seen 59,96–100 . Progressive decline in cognition after seizure onset should suggest a progressive myoclonus epilepsy.…”

Section: Juvenile Myoclonic Epilepsymentioning

confidence: 99%

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

et al. 2022

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In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the "genetic generalized epilepsies" (GGEs), which contained the "idiopathic generalized epilepsies" (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE's Task Force on Nosology and Definitions (2017-2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonictonic-clonic seizures, with 2.5-5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.

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Section: Juvenile Myoclonic Epilepsymentioning

confidence: 99%

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

et al. 2022

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“…dysfunction, especially impulsiveness, is a very wellknown feature accompanying JME. Other psychosocial complications, such as ADHD or impaired facial emotional recognition, may also accompany the seizures, raising the question whether the spectrum for IGE goes beyond just a seizure disorder (Gomez-Ibañez et al, 2014;Almane et al, 2019;Syvertsen et al, 2019). A comprehensive neuropsychological evaluation of these patients may enhance our knowledge about GGE/IGE and enable each individual to reach their full potential.…”

Section: Case Notesmentioning

confidence: 99%

How to diagnose and classify idiopathic (genetic) generalized epilepsies

Elmalı¹,

Auvin²,

Bast³

et al. 2020

Epileptic Disorders

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Idiopathic or genetic generalized epilepsies (IGE) constitute an electroclinically well‐defined group that accounts for almost one third of all people with epilepsy. They consist of four well‐established syndromes and some other rarer phenotypes. The main four IGEs are juvenile myoclonic epilepsy, childhood absence epilepsy, juvenile absence epilepsy and IGE with generalized tonic‐clonic seizures alone. There are three main seizure types in IGE, namely generalized tonic‐clonic seizures, typical absences and myoclonic seizures, occurring either alone or in any combination. Diagnosing IGEs requires a multidimensional approach. The diagnostic process begins with a thorough medical history with a specific focus on seizure types, age at onset, timing and triggers. Comorbidities and family history should be questioned comprehensively. The EEG can provide valuable information for the diagnosis, including specific IGE syndromes, and therefore contribute to their optimal pharmacological treatment and management.

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“…However, some recent studies using quantitative EEG (qEEG) analysis reported abnormal changes in background activity in JME patients [ 5 – 7 ]. In addition, neuropsychological studies showed cognitive dysfunction [ 8 – 10 ], and several studies using multimodal methods such as magnetoencephalography (MEG), functional magnetic resonance imaging (fMRI), and evoked potentials have supported these abnormalities in JME [ 11 – 15 ].…”

Section: Introductionmentioning

confidence: 99%

Comparative analysis of background EEG activity in juvenile myoclonic epilepsy during valproic acid treatment: a standardized, low-resolution, brain electromagnetic tomography (sLORETA) study

et al. 2022

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Background By definition, the background EEG is normal in juvenile myoclonic epilepsy (JME) patients and not accompanied by other developmental and cognitive problems. However, some recent studies using quantitative EEG (qEEG) reported abnormal changes in the background activity. QEEG investigation in patients undergoing anticonvulsant treatment might be a useful approach to explore the electrophysiology and anticonvulsant effects in JME. Methods We investigated background EEG activity changes in patients undergoing valproic acid (VPA) treatment using qEEG analysis in a distributed source model. In 17 children with JME, non-parametric statistical analysis using standardized low-resolution brain electromagnetic tomography was performed to compare the current density distribution of four frequency bands (delta, theta, alpha, and beta) between untreated and treated conditions. Results VPA reduced background EEG activity in the low-frequency (delta-theta) bands across the frontal, parieto-occipital, and limbic lobes (threshold log-F-ratio = ±1.414, p < 0.05; threshold log-F-ratio= ±1.465, p < 0.01). In the delta band, comparative analysis revealed significant current density differences in the occipital, parietal, and limbic lobes. In the theta band, the analysis revealed significant differences in the frontal, occipital, and limbic lobes. The maximal difference was found in the delta band in the cuneus of the left occipital lobe (log-F-ratio = −1.840) and the theta band in the medial frontal gyrus of the left frontal lobe (log-F-ratio = −1.610). Conclusions This study demonstrated the anticonvulsant effects on the neural networks involved in JME. In addition, these findings suggested the focal features and the possibility of functional deficits in patients with JME.

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The Timing, Nature, and Range of Neurobehavioral Comorbidities in Juvenile Myoclonic Epilepsy

Cited by 28 publications

References 55 publications

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

How to diagnose and classify idiopathic (genetic) generalized epilepsies

Comparative analysis of background EEG activity in juvenile myoclonic epilepsy during valproic acid treatment: a standardized, low-resolution, brain electromagnetic tomography (sLORETA) study

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