Effect of age of onset and treatment on control of epileptic spasms: Findings from the Sri Lanka infantile spasms registry

2021 ◽  
Vol 429 ◽  
pp. 118900
Author(s):  
Jithangi Wanigasinghe ◽  
Gemunu Hewawitharana ◽  
Saraji Wijesekera ◽  
Pyara Ratnayake ◽  
Chathurika Weeraratne ◽  
...  
2020 ◽  
Author(s):  
Jedidiah I Morton ◽  
Danny Liew ◽  
Stephen P McDonald ◽  
Jonathan E Shaw ◽  
Dianna J Magliano

<b>Objective</b>: The long-term risk of end-stage kidney disease (ESKD) in type 2 diabetes is poorly described, as is the effect that younger age of diabetes onset has on this risk. Therefore, we aimed to estimate the effect of age of onset on the cumulative incidence of ESKD from onset of type 2 diabetes. <p><b>Research Design and Methods: </b>This study included 1,113,201 people with type 2 diabetes registered on the Australian National Diabetes Services Scheme (NDSS) followed from 2002 until 2013. The NDSS was linked to the Australia and New Zealand Dialysis and Transplant Registry and the Australian National Death Index. </p> <p><b>Results: </b>Between 2002 and 2013,<b> </b>there were 7,592 incident cases of ESKD during 7,839,075 person-years of follow up. In the first 10-15 years following onset of diabetes, the incidence of ESKD was highest in those with an older age of onset of diabetes, whereas over longer durations of diabetes the incidence of ESKD became higher in those with younger-onset diabetes. After 40 years of diabetes, the cumulative incidence of ESKD was 11.8% and 9.3% in those diagnosed with diabetes aged 10-29 and 30-39 years, respectively. When death from ESKD without renal replacement therapy was included, incidence of ESKD remained higher in older onset diabetes for the initial 20 years, with no clear effect of age thereafter.</p> <p><b>Conclusions: </b>The long-term risk of ESKD in type 2 diabetes is high, which disproportionately affects those with younger-onset of diabetes as they are more likely to survive to longer diabetes durations.</p>


2019 ◽  
Vol 18 (01) ◽  
pp. 039-044
Author(s):  
Behshad Charkhand ◽  
Natarie Liu ◽  
Karlene T. Barrett ◽  
Walla Al-Hertani ◽  
Morris H. Scantlebury

AbstractThe infantile spasms (IS) syndrome is a developmental epileptic encephalopathy disorder characterized by epileptic spasms occurring in infancy, hypsarrhythmia on the electroencephalography (EEG) and developmental arrest or regression. The etiologies include structural, metabolic, and genetic causes. We report an unusual case of IS due to a de novo variant in the MECP2 gene. The patient also had variants of uncertain significance in the SCN9A and SCN5A genes inherited from the father and mother, respectively. This report highlights the need for broad genetic testing in MECP2-related disorders with atypical presentations to better understand the disease etiology.


1989 ◽  
Vol 2 (4) ◽  
pp. 182-187 ◽  
Author(s):  
Nathan Herrmann ◽  
Susan Lieff ◽  
Michel Silberfeld

2020 ◽  
Author(s):  
Hiroki Nariai ◽  
Shaun A. Hussain ◽  
Danilo Bernardo ◽  
Hirotaka Motoi ◽  
Masaki Sonoda ◽  
...  

ABSTRACTObjectiveTo investigate the diagnostic utility of high frequency oscillations (HFOs) via scalp electroencephalogram (EEG) in infantile spasms.MethodsWe retrospectively analyzed interictal slow-wave sleep EEGs sampled at 2,000 Hz recorded from 30 consecutive patients who were suspected of having infantile spasms. We measured the rate of HFOs (80-500 Hz) and the strength of the cross-frequency coupling between HFOs and slow-wave activity (SWA) at 3-4 Hz and 0.5-1 Hz as quantified with modulation indices (MIs).ResultsTwenty-three patients (77%) exhibited active spasms during the overnight EEG recording. Although the HFOs were detected in all children, increased HFO rate and MIs correlated with the presence of active spasms (p < 0.001 by HFO rate; p < 0.01 by MIs at 3-4 Hz; p = 0.02 by MIs at 0.5-1 Hz). The presence of active spasms was predicted by the logistic regression models incorporating HFO-related metrics (AUC: 0.80-0.98) better than that incorporating hypsarrhythmia (AUC: 0.61). The predictive performance of the best model remained favorable (87.5% accuracy) after a cross-validation procedure.ConclusionsIncreased rate of HFOs and coupling between HFOs and SWA are associated with active epileptic spasms.SignificanceScalp-recorded HFOs may serve as an objective EEG biomarker for active epileptic spasms.HighlightsObjective analyses of scalp high frequency oscillations and its coupling with slow-wave activity in infantile spasms were feasible.Increased rate of high frequency oscillations and its coupling with slow-wave activity correlated with active epileptic spasms.The scalp high frequency oscillations were also detected in neurologically normal children (although at the low rate).


Seizure ◽  
2019 ◽  
Vol 71 ◽  
pp. 174-178 ◽  
Author(s):  
Zhaoshi Yi ◽  
Huaping Wu ◽  
Xiongying Yu ◽  
Jian Zha ◽  
Hui Chen ◽  
...  

2007 ◽  
Vol 254 (1) ◽  
pp. 38-45 ◽  
Author(s):  
D. Aarsland ◽  
J.T. Kvaløy ◽  
K. Andersen ◽  
J.P. Larsen ◽  
M.X. Tang ◽  
...  
Keyword(s):  

2018 ◽  
Vol 19 (6) ◽  
pp. 879-886 ◽  
Author(s):  
Sanminder Singh ◽  
Robert E. Kalb ◽  
Elke M. G. J. de Jong ◽  
Neil H. Shear ◽  
Mark Lebwohl ◽  
...  

2017 ◽  
Vol 32 (10) ◽  
pp. 861-866 ◽  
Author(s):  
Adam Wallace ◽  
Victoria Allen ◽  
Kristen Park ◽  
Kelly Knupp

The association of infantile spasms and periventricular leukomalacia and/or intraventricular hemorrhage is well documented. Data regarding early treatment-based and long-term outcomes are limited. A retrospective chart review identified children with infantile spasms born prematurely (<37 weeks) with diagnoses of periventricular leukomalacia and/or intraventricular hemorrhage. Thirteen children were included. Median gestational age was 30 weeks and age of onset of infantile spasms was 8 months. Nine children had intraventricular hemorrhage, 10 had periventricular leukomalacia, and 6 children had both. Twelve of 13 children had resolution of spasms. In responders, the successful medication was adrenocorticotropic hormone (ACTH) in 7, topiramate in 3, and vigabatrin in 2. Follow-up after a median of 7.1 years found that all patients had developmental delay but only 1 had refractory epilepsy. Standard therapies (ACTH and vigabatrin) appeared to be more effective than other treatments. Developmental delay is common in children with periventricular leukomalacia / intraventricular hemorrhage and infantile spasms, but refractory epilepsy might be less frequent.


2007 ◽  
Vol 90 (11) ◽  
pp. 1343-1355 ◽  
Author(s):  
A Verrotti ◽  
D Trotta ◽  
A Blasetti ◽  
L Lobefalo ◽  
P Gallenga ◽  
...  
Keyword(s):  

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