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

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 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

scholarly journals Rare variants of small effect size in neuronal excitability genes influence clinical outcome in Japanese cases of SCN1A truncation-positive Dravet syndrome

PLoS ONE ◽  
2017 ◽  
Vol 12 (7) ◽  
pp. e0180485 ◽  
Author(s):  
Michael F. Hammer ◽  
Atsushi Ishii ◽  
Laurel Johnstone ◽  
Alexander Tchourbanov ◽  
Branden Lau ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Effect Size ◽  
Neuronal Excitability ◽  
Rare Variants ◽  
Dravet Syndrome ◽  
Small Effect Size

scholarly journals Septic Encephalopathy Characterized by Acute Encephalopathy with Biphasic Seizures and Late Reduced Diffusion and Early Nonconvulsive Status Epilepticus

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Hiroshi Yamaguchi ◽  
Tsukasa Tanaka ◽  
Azusa Maruyama ◽  
Hiroaki Nagase
Keyword(s):  
Status Epilepticus ◽  
Febrile Seizure ◽  
Immune Reaction ◽  
Brain Dysfunction ◽  
Nonconvulsive Status Epilepticus ◽  
Acute Encephalopathy ◽  
Septic Encephalopathy ◽  
Impaired Consciousness ◽  
Neurologic Symptom ◽  
Early Seizure

Infection, whether viral or bacterial, can result in various forms of brain dysfunction (encephalopathy). Septic encephalopathy (SE) is caused by an excessive immune reaction to infection, with clinical features including disturbed consciousness and seizures. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is usually accompanied by viral infection in children and is characterized by biphasic seizures and impaired consciousness. The initial neurologic symptom of AESD is typically a febrile seizure that frequently lasts longer than 30 minutes. However, the possible forms this seizure takes are unclear. For example, it is unknown if nonconvulsive status epilepticus (NCSE) could be an early seizure symptomatic of AESD. In addition, thus far no cases of combined SE and AESD have been reported. Here, we describe the first reported case of SE with AESD that notably demonstrated NCSE as an early seizure.

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