Caldera-forming eruptions of island volcanoes generate tsunamis by the interaction of different eruptive phenomena with the sea. Such tsunamis are a major hazard, but forward models of their impacts ...are limited by poor understanding of source mechanisms. The caldera-forming eruption of Santorini in the Late Bronze Age is known to have been tsunamigenic, and caldera collapse has been proposed as a mechanism. Here, we present bathymetric and seismic evidence showing that the caldera was not open to the sea during the main phase of the eruption, but was flooded once the eruption had finished. Inflow of water and associated landsliding cut a deep, 2.0-2.5 km
, submarine channel, thus filling the caldera in less than a couple of days. If, as at most such volcanoes, caldera collapse occurred syn-eruptively, then it cannot have generated tsunamis. Entry of pyroclastic flows into the sea, combined with slumping of submarine pyroclastic accumulations, were the main mechanisms of tsunami production.
Transform margins are first‐order tectonic features that accommodate oceanic spreading. Uncertainties remain about their evolution, genetic relationship to oceanic spreading, and general structural ...character. When the relative motion of the plates changes during the margin evolution, further structural complexity is added. This work investigates the evolution of transform margins and associated rift‐transform intersections, using an analogue modeling approach that simulates changing plate motions. We investigate the effects of different crustal rheologies by using either (a) a two‐layer brittle‐ductile configuration to simulate upper and lower continental crust, or (b) a single layer brittle configuration to simulate oceanic crust. The modeled rifting is initially orthogonal, followed by an imposed plate vector change of 7° that results in oblique rifting and plate overlap (transpression) or underlap (transtension) along each transform margin. This oblique deformation reactivates and overprints earlier orthogonal structures and is representative of natural examples. We find that (a) a transtensional shift in the plate direction produces a large strike‐slip principal displacement zone, accompanied by en‐echelon oblique‐normal faults that accommodate the horizontal displacement until the new plate motion vector is stabilized, while (b) a transpressional shift produces compressional structures such as thrust fronts in a triangular zone in the area of overlap. These observations are in good agreement with natural examples from the Gulf of California (transtensional) and Tanzania Coastal Basin (transpressional) shear margins and illustrate that when these deformation patterns are present, a component of plate vector change should be considered in the evolution of transform margins.
Plain Language Summary
Tectonic plate boundaries on our planet are categorized by their relative motion with respect to each other. The three main categories are those moving away, toward, and parallel to one another. We study the processes occurring when two tectonic plates moving parallel begin to rotate and move away or toward each other. Currently, this is occurring in the Gulf of California, and in the past, it occurred in areas such as the Southern Atlantic, creating the segmented pattern along its midocean ridge. To study these tectonic plate boundaries, we use sandbox modeling. We make miniature models of the Earth's crust with silicone putty and sand and recreate the same movements that tectonic plates go through. This allows us to understand the structures created in such environments better. The pattern and the height or depth of these structures are related to how fast the plates move. This work can help recognize areas where similar deformation has occurred in the past, which is important for hydrocarbon exploration. It can also assist with geothermal energy exploration, as areas where plates move parallel and away from each other present good opportunities for hotter temperatures in the subsurface.
Key Points
We use analogue models to study transtension and transpression on transform margins and rift‐transform intersections due to plate rotation
The extent and topography of the structures created are strongly dependent on the relative velocity between transform margins
Our results show good agreement with natural examples from the Gulf of California and the Tanzania Coastal Basin
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Summary
Purpose: We aimed to assess long‐term seizure outcome and risk factors for seizure recurrence in a cohort of patients who have undergone extratemporal resection for management of refractory ...seizures.
Methods: Eighty‐one patients underwent extratemporal resection at Austin Health, Melbourne, Australia (1991–2004). Seizure recurrence was any postoperative disabling seizure (complex partial seizure CPS ± secondary generalization). Multivariate Cox proportional hazards regression models examined potential preoperative and perioperative risk factors and the risk associated with early postoperative seizures (≤28 days postsurgery). The change between preoperative and postoperative seizure frequency was also measured.
Key Findings: Median follow‐up was 10.3 years (range 1–17.7). The probabilities of freedom from disabling seizures (on or off antiepileptic medication) were 40.7% (95% confidence interval CI 30–51) at 1 month, 23.5% (95% CI 15–33) at 1 year, and 14.7% (95% CI 8–23) at 5 years postoperative. Reduction of disabling seizures to at least 20% of preoperative frequency was attained by 57% of patients at 5 postoperative years. Of the preoperative/perioperative factors, focal cortical dysplasia (FCD) type 1 (hazard ratio HR 1.90, 95% CI 1.08–3.34, p = 0.025) and incomplete resection (HR 1.71, 95% CI 1.06–2.76, p = 0.028) were independent recurrence risks. After surgery, an early postoperative seizure was the only factor associated with higher risk (HR 4.28 2.42–7.57, p = 0.00).
Significance: Distinction between subtypes of focal cortical dysplasia, which can be made using magnetic resonance imaging (MRI) criteria, may be useful for preoperative prognostication. Early seizures after surgery are not benign and may be markers of factors that contribute to seizure recurrence. Most patients achieve substantial reduction in seizure frequency. Further study of the significance of this reduction in terms of surgical “success” or otherwise is required.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Action myoclonus-renal failure syndrome (AMRF) is an autosomal-recessive disorder with the remarkable combination of focal glomerulosclerosis, frequently with glomerular collapse, and progressive ...myoclonus epilepsy associated with storage material in the brain. Here, we employed a novel combination of molecular strategies to find the responsible gene and show its effects in an animal model. Utilizing only three unrelated affected individuals and their relatives, we used homozygosity mapping with single-nucleotide polymorphism chips to localize AMRF. We then used microarray-expression analysis to prioritize candidates prior to sequencing. The disorder was mapped to 4q13-21, and microarray-expression analysis identified
SCARB2/Limp2, which encodes a lysosomal-membrane protein, as the likely candidate. Mutations in
SCARB2/Limp2 were found in all three families used for mapping and subsequently confirmed in two other unrelated AMRF families. The mutations were associated with lack of SCARB2 protein. Reanalysis of an existing
Limp2 knockout mouse showed intracellular inclusions in cerebral and cerebellar cortex, and the kidneys showed subtle glomerular changes. This study highlights that recessive genes can be identified with a very small number of subjects. The ancestral lysosomal-membrane protein SCARB2/LIMP-2 is responsible for AMRF. The heterogeneous pathology in the kidney and brain suggests that SCARB2/Limp2 has pleiotropic effects that may be relevant to understanding the pathogenesis of other forms of glomerulosclerosis or collapse and myoclonic epilepsies.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Basement structure is known to exert strong magmatic and morphological control on continental volcanoes, but relatively little is known about the structural control of submarine volcanoes. Here we ...investigate the morphology of the Tasmantid Seamounts, a >2,400 km long chain of age‐progressive intraplate volcanoes, ranging from 56 to 7 Ma. The seamounts are emplaced over the extinct Tasman Sea spreading center, which was active between 84 and 52 Ma. While thick sediment (∼1 km) obscures much of the basement, detailed morphological and geophysical analyses of the seamounts reveal a strong correlation between tectonic setting, seamount orientation, and volcanic structure, despite the ≥20 Ma interval between spreading cessation and seamount emplacement. Seamounts emplaced on fracture zones or spreading segment‐transform fault inside corners are typically large and elongate. Where original morphology is preserved, they often appear rugged and predominantly fissure‐fed. By contrast, comparatively smooth, conical seamounts with isolated dike‐fed flank cones are often found midsegment and at outside corners. Volcanic fabrics also align closely with the expected principal stress directions for strong mechanical coupling across transform faults. This behavior suggests the lithosphere is dissected by numerous deep faults, channeling magma along preexisting structural trends. Generally, low effective elastic thicknesses (<10 km) and lack of correlation with plate age at emplacement suggest that structural inheritance is also a major control on lithospheric strength near the extinct spreading center. Our study clearly demonstrates that, like in the continents, structural inheritance in oceanic lithosphere can exert significant control on the morphology of submarine volcanoes.
Plain Language Summary
The shapes of volcanoes in continental regions can be strongly influenced by faults. These lines of weakness provide a natural pathway for rising magma so that large volumes are erupted along these trends, and volcanoes are often elongated parallel to underlying faults. However, little is known about the effect of faults on submarine volcanoes. Here we use high‐resolution ocean depth and gravity data to investigate how faulting influences the structure of the Tasmantid Seamounts, a 2,500 km long chain of underwater volcanoes off the east coast of Australia. We find that seamount shapes are strongly influenced by faults that formed when Australia and New Zealand rifted apart, tens of millions of years before the volcanoes formed. The mechanical strength of the crust and mantle beneath the seamounts is also far weaker than expected due to the faulting. Finally, much of the magma making up the volcanoes never reached the surface and instead solidified below the seafloor. This suggests that the hot region in the mantle causing the volcanoes to form was not very strong compared to places like Hawaii or Iceland. This study shows that, like in the continents, preexisting faults can have a profound effects on the structure of submarine volcanoes.
Key Points
Tasmantid Seamount morphology governed by structural inheritance from Tasman spreading center, despite eruption >20 Ma after spreading ceased
This morphological dependence, low mass‐wasting rates, and evidence of dense intrusive cores suggest a relatively weak Tasmantid plume
Te measurements suggest lithosphere is heavily faulted near ridge‐transform intersections, deforming predominantly by brittle yielding
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
There is little information available relevant to long-term seizure outcome after anterior temporal lobectomy, particularly at extended postoperative periods. The aim of this study was an in-depth ...examination of patterns of longitudinal outcome and potential risk factors for seizure recurrence after lobectomy, utilizing a large patient sample with long follow-up. Included were 325 patients who underwent anterior temporal lobectomy between 1978 and 1998 (mean follow-up 9.6 ± 4.2 years). Retrospective data were analysed using survival analysis and multivariate regression with Cox proportional hazard models. The probability of complete seizure freedom at 2 years post-surgery was 55.3% 95% confidence interval (CI) 50–61; at 5 years, 47.7% (95% CI 42–53); and at 10 postoperative years it was 41% (95% CI 36–48). Patients with discrete abnormalities preoperatively (i.e. lesions and hippocampal sclerosis) had a significantly higher probability of seizure freedom than patients without obvious abnormality. The latter group had a pattern of recurrence similar to that in patients with lesions outside the area of excision. After adjustment for preoperative pathology, only the presence of preoperative secondarily generalized seizures had a significant association with recurrence occasional preoperative generalized seizures, hazard ratio (HR) 1.6, 95% CI 1.1–2.3; frequent seizures, HR 2.0, 95% CI 1.4–2.9 compared with absence of preoperative generalized seizures. Duration of preoperative epilepsy, age of seizure onset and age at surgery did not have an effect on outcome. Patients with two seizure-free postoperative years had a 74% (95% CI 66–81) probability of seizure freedom by 10 postoperative years. This late seizure recurrence was not associated with any identified risk factors. Specifically, patients with hippocampal sclerosis were not at higher risk. Surprisingly, complete discontinuation of anti-epileptic drugs (AEDs) after two postoperative years was not associated with an increased risk of recurrence (HR 1.03, 95% CI 0.5–2.1). This may be because selection of patients for AED discontinuation is biased towards those individuals perceived as ‘low risk’. The results of this study indicate that the lack of an obvious abnormality or the presence of diffuse pathology, and preoperative secondarily generalized seizures are risk factors for recurrence after surgery. Late recurrence after initial seizure freedom is not a rare event; risk factors specific to this phenomenon are as yet unidentified.
Resection of dysembryoplastic neuroepithelial tumor (DNET) is thought to result in favorable seizure outcome, but long-term follow-up data are scarce. The authors present a review of 18 patients who ...underwent surgical removal of a DNET: 12 via temporal lobectomy and six via lesionectomy.
The mean long-term follow up was 10.8 years (median 10.4 years, range 7.8 to 14.8 years), and results obtained during this time period were compared with previously reported short-term (mean 2.7 years) seizure outcome data. In the current study, 66.7% patients had an Engel Class I outcome and 55.6% had an Engel Class IA outcome compared with 77.8% and 55.6%, respectively. Temporal lobectomy (Engel Class I, 83.3%; Engel Class IA, 66.7%) led to a better seizure outcome than lesionectomy (Engel Classes I and IA, 33.3%). Two patients (11.1%) required repeated operation and both had an incomplete lesionectomy initially.
Results indicated that complete resection of a DNET leads to a favorable seizure outcome, with epilepsy cure in those who had experienced early postoperative seizure relief. Long-term seizure outcome after surgery is predictable based on the result of short-term follow up.
Summary
Focal cortical dysplasia is a common cortical malformation and an important cause of epilepsy. There is evidence for shared molecular mechanisms underlying cortical dysplasia, ganglioglioma, ...hemimegalencephaly, and dysembryoplastic neuroepithelial tumor. However, there are no familial reports of typical cortical dysplasia or co‐occurrence of cortical dysplasia and related lesions within the same pedigree. We report the clinical, imaging, and histologic features of six pedigrees with familial cortical dysplasia and related lesions. Twelve patients from six pedigrees were ascertained from pediatric and adult epilepsy centers, eleven of whom underwent epilepsy surgery. Pedigree data, clinical information, neuroimaging findings, and histopathologic features are presented. The families comprise brothers with focal cortical dysplasia, a male and his sister with focal cortical dysplasia, a female with focal cortical dysplasia and her brother with hemimegalencephaly, a female with focal cortical dysplasia and her female first cousin with ganglioglioma, a female with focal cortical dysplasia and her male cousin with dysembryoplastic neuroepithelial tumor, and a female and her nephew with focal cortical dysplasia. This series shows that focal cortical dysplasia can be familial and provides clinical evidence suggesting that cortical dysplasia, hemimegalencephaly, ganglioglioma, and dysembryoplastic neuroepithelial tumors may share common genetic determinants.
A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
OBJECTIVETo delineate the epileptology, a key part of the SYNGAP1 phenotypic spectrum, in a large patient cohort.
METHODSPatients were recruited via investigatorsʼ practices or social media. We ...included patients with (likely) pathogenic SYNGAP1 variants or chromosome 6p21.32 microdeletions incorporating SYNGAP1. We analyzed patientsʼ phenotypes using a standardized epilepsy questionnaire, medical records, EEG, MRI, and seizure videos.
RESULTSWe included 57 patients (53% male, median age 8 years) with SYNGAP1 mutations (n = 53) or microdeletions (n = 4). Of the 57 patients, 56 had epilepsygeneralized in 55, with focal seizures in 7 and infantile spasms in 1. Median seizure onset age was 2 years. A novel type of drop attack was identified comprising eyelid myoclonia evolving to a myoclonic-atonic (n = 5) or atonic (n = 8) seizure. Seizure types included eyelid myoclonia with absences (65%), myoclonic seizures (34%), atypical (20%) and typical (18%) absences, and atonic seizures (14%), triggered by eating in 25%. Developmental delay preceded seizure onset in 54 of 56 (96%) patients for whom early developmental history was available. Developmental plateauing or regression occurred with seizures in 56 in the context of a developmental and epileptic encephalopathy (DEE). Fifty-five of 57 patients had intellectual disability, which was moderate to severe in 50. Other common features included behavioral problems (73%); high pain threshold (72%); eating problems, including oral aversion (68%); hypotonia (67%); sleeping problems (62%); autism spectrum disorder (54%); and ataxia or gait abnormalities (51%).
CONCLUSIONSSYNGAP1 mutations cause a generalized DEE with a distinctive syndrome combining epilepsy with eyelid myoclonia with absences and myoclonic-atonic seizures, as well as a predilection to seizures triggered by eating.
In transform margins, oblique structural inheritance and plate motion vector changes have a direct impact on the margin's morphology and duration of transform activity. We investigate the effect of ...these two factors using numerical modelling. To simulate oblique inheritance in continental lithosphere, we model an initial rift-transform-rift configuration oriented at a range of angles (−45o to +45o) with respect to the extension direction. In a second suite of calculations, we first extend a rift-transform-rift system orthogonally and then vary the extension direction to simulate rotation of the far-field stress directions, and hence the relative plate motion. We found that transpressional deformation on the transform results in increased duration of fault activity and diffuse, longer transform zones at higher angles, while the opposite is true for the transtensional case. These observations are in good agreement with natural examples such as the Ungava Transform Zone, the Gulf of California and the Gulf of Aden, indicating that relative plate rotation plays an important role in the structural evolution of transform margins. Finally, we present a metric that links current transform margin morphology to past evolution.
•Models test oblique inheritance and plate motion changes on transform margins.•Changes in relative plate motion increase duration of transform margin activity.•Increasing transpressive obliquity or rotation increases transform margin length.•We present natural examples that exemplify the modelled results.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP