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

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 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.

Dravet Syndrome: Early Diagnosis and Emerging Therapies

2019 ◽  
Vol 08 (02) ◽  
pp. 031-037
Author(s):  
Tyler J. Burr ◽  
Karen L. Skjei
Keyword(s):  
Status Epilepticus ◽  
Early Diagnosis ◽  
Seizure Control ◽  
Febrile Seizures ◽  
Epileptic Encephalopathy ◽  
Dravet Syndrome ◽  
Long Term Outcomes ◽  
Treatment Algorithms ◽  
Emerging Therapies

AbstractDravet's syndrome (DS) or severe myoclonic epilepsy of infancy is a rare, genetic, and infantile-onset epileptic encephalopathy. DS presents with recurrent febrile seizures and/or febrile status epilepticus in developmentally normal infants, and subsequently evolves into a drug-resistant mixed-seizure disorder with developmental arrest or regression. As many defining clinical features of DS do not become evident until 3 to 4 years of age, diagnosis is often delayed. Early seizure control, particularly the prevention of status epilepticus in infancy, has been shown to correlate with better long-term outcomes. Thus, early diagnosis and seizure control is crucial. Several treatment algorithms have been published in recent years to guide antiepileptic drug selection and escalation. Last year, two agents, stiripentol and cannabidiol, were approved by the U.S. Food and Drug Administration specifically for use in DS, and a third has been submitted (fenfluramine). Additional therapies, including serotonin modulators lorcaserin and trazodone, verapamil, and several first-in-class medications, are currently in various phases of investigation.

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