Please use this identifier to cite or link to this item: http://ahro.austin.org.au/austinjspui/handle/1/12246
Title: Lennox-Gastaut syndrome and phenotype: secondary network epilepsies.
Authors: Archer, John S;Warren, Aaron E L;Stagnitti, Monique R;Masterton, Richard A J;Abbott, David F;Jackson, Graeme D
Affiliation: Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia; The Florey Institute of Neuroscience and Mental Health, Melbourne, Victoria, Australia; Austin Health, Melbourne, Victoria, Australia.
Issue Date: 5-Jun-2014
Citation: Epilepsia 2014; 55(8): 1245-54
Abstract: Lennox-Gastaut syndrome (LGS) is a severe epilepsy phenotype with characteristic electroclinical features despite diverse etiologies. We previously found common cerebral networks involved during slow spike-and-wave (SSW) and generalized paroxysmal fast activity (PFA), characteristic interictal discharges. Some patients have a Lennox-Gastaut-like phenotype and cortical lesions. We wished to explore the interaction between cerebral networks and lesions in this group.3 Tesla electroencephalography-functional magnetic resonance imaging (EEG-fMRI) on six subjects with Lennox-Gastaut phenotype and a structural lesion. Timings of SSW and PFA events were used in an event-related fMRI analysis, and to estimate the time course of the hemodynamic response from key regions.(1) PFA-robust fMRI signal increases were observed in frontal and parietal association cortical areas, thalamus, and pons, with simultaneous increases in both "attention" and resting-state (default mode) networks, a highly unusual pattern. (2) SSW showed mixed increased and decreased fMRI activity, with preevent increases in association cortex and thalamus, and then prominent postevent reduction. There was decreased fMRI activity in primary cortical areas. (3) Lesion-variable fMRI increases were observed during PFA and SSW discharges. Three subjects who proceeded to lesionectomy are >1 year seizure-free.We conceptualize Lennox-Gastaut phenotype as a being a network epilepsy, where key cerebral networks become autonomously unstable. Epileptiform activity in Lennox-Gastaut phenotype, and by implication in LGS, appears to be amplified and expressed through association cortical areas, possibly because the attention and default-mode networks are widely interconnected, fundamental brain networks. Seizure freedom in the subjects who proceeded to lesionectomy suggests that cortical lesions are able to establish and maintain this abnormal unstable network behavior. LGS may be considered a secondary network epilepsy because the unifying epileptic manifestations of the disorder, including PFA and SSW, reflect network dysfunction, rather than the specific initiating process.
Internal ID Number: 24902608
URI: http://ahro.austin.org.au/austinjspui/handle/1/12246
DOI: 10.1111/epi.12682
URL: http://www.ncbi.nlm.nih.gov/pubmed/24902608
Type: Journal Article
Subjects: EEG-fMRI
Lennox-Gastaut syndrome
Lesion
Secondary generalized epilepsy
Surgery
Adult
Cerebral Cortex.physiopathology
Epilepsy.diagnosis.physiopathology
Female
Humans
Intellectual Disability.diagnosis.physiopathology
Lennox Gastaut Syndrome
Male
Nerve Net.physiopathology
Phenotype
Spasms, Infantile.diagnosis.physiopathology
Appears in Collections:Journal articles

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