scholarly journals Acute encephalopathy after febrile status epilepticus: an underdiagnosed, misunderstood complication of Dravet syndrome

2018 ◽  
Vol 60 (6) ◽  
pp. 534-534
Author(s):  
Charlotte Dravet
Keyword(s):  
Status Epilepticus ◽  
Dravet Syndrome ◽  
Acute Encephalopathy ◽  
Febrile Status Epilepticus

scholarly journals Reply to Dravet, C. Different Outcomes of Acute Encephalopathy after Status Epilepticus in Patients with Dravet Syndrome. How to Avoid Them? Comment on “De Liso et al. Fatal Status Epilepticus in Dravet Syndrome. Brain Sci. 2020, 10, 889”

2021 ◽  
Vol 11 (6) ◽  
pp. 811
Author(s):  
Paola De Liso ◽  
Virginia Pironi ◽  
Massimo Mastrangelo ◽  
Domenica Battaglia ◽  
Dana Craiu ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Dravet Syndrome ◽  
Acute Encephalopathy ◽  
To Receive

It has been an honor for us to receive a comment on our article “Fatal Status Epilepticus in Dravet Syndrome” [...]

scholarly journals The clinical outcome and neuroimaging of acute encephalopathy after status epilepticus in Dravet syndrome

2018 ◽  
Vol 60 (6) ◽  
pp. 566-573 ◽  
Author(s):  
Xiaojuan Tian ◽  
Jintang Ye ◽  
Qi Zeng ◽  
Jing Zhang ◽  
Xiaoling Yang ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Clinical Outcome ◽  
Dravet Syndrome ◽  
Acute Encephalopathy

Prediction of acute encephalopathy with biphasic seizures and late reduced diffusion in patients with febrile status epilepticus

2016 ◽  
Vol 38 (2) ◽  
pp. 217-224 ◽  
Author(s):  
Takaoki Yokochi ◽  
Takahito Takeuchi ◽  
Jumpei Mukai ◽  
Yukihiro Akita ◽  
Kojiro Nagai ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Acute Encephalopathy ◽  
Febrile Status Epilepticus

Incidence and risk factors of acute encephalopathy with biphasic seizures in febrile status epilepticus

2021 ◽  
Author(s):  
Fumio Ichinose ◽  
Takuji Nakamura ◽  
Ryutaro Kira ◽  
Kenji Furuno ◽  
Shigeki Ishii ◽  
...  
Keyword(s):  
Risk Factors ◽  
Status Epilepticus ◽  
Acute Encephalopathy ◽  
Febrile Status Epilepticus

scholarly journals Fatal Status Epilepticus in Dravet Syndrome

2020 ◽  
Vol 10 (11) ◽  
pp. 889
Author(s):  
Paola De Liso ◽  
Virginia Pironi ◽  
Massimo Mastrangelo ◽  
Domenica Battaglia ◽  
Dana Craiu ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Characteristic Feature ◽  
Respiratory Infection ◽  
Premature Mortality ◽  
Dravet Syndrome ◽  
Reference Network ◽  
Acute Encephalopathy ◽  
European Reference Network

Dravet Syndrome (DS) is burdened by high epilepsy-related premature mortality due to status epilepticus (SE). We surveyed centres within Europe through the Dravet Italia Onlus and EpiCARE network (European Reference Network for Rare and Complex Epilepsies). We collated responses on seven DS SCN1A+ patients who died following refractory SE (mean age 6.9 year, range 1.3–23.4 year); six were on valproate, clobazam, and stiripentol. All patients had previous SE. Fatal SE was always triggered by fever: either respiratory infection or one case of hexavalent vaccination. SE lasted between 80 min and 9 h and all patients received IV benzodiazepines. Four patients died during or within hours of SE; in three patients, SE was followed by coma with death occurring after 13–60 days. Our survey supports the hypothesis that unresponsive fever is a core characteristic feature of acute encephalopathy. We highlight the need for management protocols for prolonged seizures and SE in DS.

scholarly journals Perfusion imaging with arterial spin labeling in acute encephalopathy with reduced subcortical diffusion following secondary generalized status epilepticus

2017 ◽  
Vol 9 (1) ◽  
pp. 32-39 ◽  
Author(s):  
Kenta Takahara ◽  
Takato Morioka ◽  
Takafumi Shimogawa ◽  
Toshiyuki Amano ◽  
Aoi Kawakita ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Perfusion Imaging ◽  
Arterial Spin Labeling ◽  
Spin Labeling ◽  
Acute Encephalopathy

How Different Are the Patients With Bilateral Hippocampal Sclerosis From the Unilateral Ones Clinically?

2016 ◽  
Vol 48 (3) ◽  
pp. 209-216 ◽  
Author(s):  
Ebru Nur Vanli-Yavuz ◽  
Betul Baykan ◽  
Serra Sencer ◽  
Altay Sencer ◽  
Isin Baral-Kulaksizoglu ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Status Epilepticus ◽  
Epilepsy Surgery ◽  
Hippocampal Sclerosis ◽  
Control Group ◽  
High Signal ◽  
Binary Logistic Regression Analysis ◽  
Laboratory Findings ◽  
Patients With Epilepsy ◽  
Febrile Status Epilepticus

Purpose. There is a lack of knowledge on consecutive patients with epilepsy associated with bilateral hippocampal sclerosis (BHS). We aimed to investigate the differentiating features of BHS in comparison with unilateral HS (UHS). Method. We investigated our database for patients with epilepsy fulfilling the major magnetic resonance imaging criteria for BHS; namely, presence of bilateral atrophy and high signal changes on T2 and FLAIR series in the hippocampi. UHS patients seen in past 2 years were included as the control group. Clinical, EEG, and other laboratory findings, data on treatment response and epilepsy surgery were investigated from their files. Results. A total of 124 patients (31 with BHS and 93 with UHS; 49 right-sided and 44 left-sided) were included. We found that 16.1% of the BHS and 18.3% of the UHS groups were not drug-refractory. A binary logistic regression analysis performed with significant clinical features disclosed that history of febrile status epilepticus, mental retardation, and status epilepticus were statistically more common in BHS group. Moreover, diagnosis of psychosis established by an experienced psychiatrist and slowing of the EEG background activity were both found significantly more frequent in BHS. 66.67% of the operated BHS patients showed benefit from epilepsy surgery. Conclusions. BHS is a heterogeneous group, showing significant differences such as increased frequencies of mental retardation, status epilepticus, febrile status epilepticus and psychosis, in comparison to UHS. In all, 16.1% of the BHS cases showed a benign course similar to the UHS group and some patients with drug-resistant epilepsy may show benefit from epilepsy surgery.

scholarly journals Etiology, Risk Factors, Mortality and Morbidity of Status Epilepticus in Children: A Retrospective Cross-Sectional Study in Tehran, Iran

2020 ◽  
Vol 14 (1) ◽  
pp. 95-102
Author(s):  
Mohammad Vafaee-Shahi ◽  
Elaheh Soltanieh ◽  
Hossein Saidi ◽  
Aina Riahi
Keyword(s):  
Risk Factors ◽  
Status Epilepticus ◽  
Brain Imaging ◽  
Morbidity And Mortality ◽  
Cross Sectional ◽  
Mortality And Morbidity ◽  
Imaging Results ◽  
History Of ◽  
Abnormal Brain ◽  
Febrile Status Epilepticus

Background: Risk factors identification associated with status epilepticus is valuable in order to prevent morbidity and mortality in children. This study aimed to consider the etiology, risk factors, morbidity and mortality in children with status epilepticus. Methods: This retrospective cross-sectional study was performed on 119 patients aged from one month old to 15 years old. Patients’ data were recorded, including basic demographic, etiology and clinical information. The different risk factors correlated to morbidity and mortality were evaluated in this study. Results: The most common etiologies were acute symptomatic and febrile status epilepticus by 32.8% and 22.7%, respectively. Abnormal brain imaging results were reported far more frequently in patients with a history of neurodevelopmental delay and previous status epilepticus (p<0.001). The overall morbidity and mortality rates were 18.9% and 10.9%, respectively; while these rates in patients with delayed development (45.16% and 18.42%, respectively) were significantly higher than patients with normal development (8% and 7.4%, respectively). The morbidity rates in patients with previous seizures and previous status epilepticus were remarkably higher than those without previous history of seizure (26.41% vs 11.32%; p=0.047) and without previous status epilepticus (36.36% versus 14.28%; p=0.018). The length of hospital stay in patients with mortality was considerably longer than patients without mortality (12.30 ± 16.1 days vs 7.29 ± 6.24 days; p=0.033). The mortality rate in patients with normal Lumbar Puncture result was notably lower than those with abnormal LP result (2.9% vs 50%). The morbidity rate in patients with abnormal brain imaging results (p<0.001) was significantly greater than those in patients with normal results. The mortality rate was relatively higher in patients with abnormal imaging results compared to those normal results. Etiology was an important predictor of mortality and morbidity rates; acute symptomatic (32.8%), febrile status epilepticus (22.7%) and remote symptomatic (16.8%) etiologies were the most common underlying causes of S.E. While in children less than 3 years old, the acute symptomatic etiology and febrile status epilepticus etiologies were estimated as the most common, in most patients older than 3 years old the most common etiology of status epilepticus was unknown. Congenital brain defects etiology had the highest mortality (36.36%) and morbidity (42.85%) rate. The lowest morbidity (3.84%) and mortality (0%) rates were for patients with febrile status epilepticus etiology. Conclusion: Age, developmental delay, history of previous status epilepticus, the length of hospital stay, abnormal brain imaging results and the underlying etiology of status epilepticus were associated with increased morbidity and mortality among children with status epilepticus.

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