Managing Status Epilepticus in a Child with Dravet Syndrome: How Difficult It Could Be?

2021 ◽  
Author(s):  
Reza Shervin Badv ◽  
Azin Ghamari ◽  
Mahmoud Reza Ashrafi ◽  
Mahmoud Mohammadi ◽  
Reza Azizi Malamiri ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Continuous Infusion ◽  
Ketogenic Diet ◽  
Sodium Valproate ◽  
Normal Development ◽  
Case Reports ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy ◽  
Myoclonic Epilepsy Of Infancy ◽  
Probable Diagnosis

AbstractPreviously known as severe myoclonic epilepsy of infancy, Dravet syndrome is characterized by febrile or afebrile prolonged hemiconvulsive seizures or generalized status epilepticus in an infant with previously normal development. Immediate management of status epilepticus is critical in these patients. Early control of status epilepticus prevents further brain damage; however, there is no consensus regarding the management of status epilepticus in children with Dravet syndrome, as many conventional antiseizure medications that are recommended in the management of status epilepticus worsen the seizures in these patients. A 2.5-year-old girl was referred due to status epilepticus which was refractory to antiseizure medications. Sodium valproate, nitrazepam, ketogenic diet, intravenous phenytoin, and midazolam continuous infusion were administered. After controlling status epilepticus, the probable diagnosis of Dravet syndrome was proposed and confirmed by a mutation in SCN1A. As previously stated in numerous case reports, phenytoin worsens seizures in patients with Dravet syndrome. Therefore, it seems logical that in every infant with status epilepticus and probable Dravet syndrome, the practicing physician considers administering intravenous valproate or even midazolam continuous infusion instead of intravenous phenytoin.

Accuracy of Flash Glucose Monitoring in a Patient with Dravet Syndrome on a Ketogenic Diet

2019 ◽  
Vol 51 (01) ◽  
pp. 045-048
Author(s):  
Aline Kortas ◽  
Katharina Schiller ◽  
Gabriele Unterholzner ◽  
Markus Rauchenzauner
Keyword(s):  
Ketogenic Diet ◽  
Glucose Monitoring ◽  
Dravet Syndrome ◽  
Observational Period ◽  
Flash Glucose Monitoring ◽  
Low Glucose ◽  
Severe Myoclonic Epilepsy ◽  
Flash Glucose Monitoring System ◽  
Myoclonic Epilepsy Of Infancy ◽  
Over Time

AbstractWe herein report the case of a 3-year-old girl with severe myoclonic epilepsy of infancy known as Dravet syndrome (DS) on a ketogenic diet (KD) whose glucose concentrations were controlled by using a flash glucose monitoring system. Two-hundred ninety-three events of moderate hypoglycemia with a minimum of 45 mg/dL, not related to day or night, were recorded during the observational period. Hypoglycemia rate declined from 24.5% of all measurements to 11.8% over time; one hypoglycemia-associated seizure and one seizure due to ketone concentrations below the therapeutic range were observed. In summary, this case report broadens our understanding of hypoglycemia risk in patients with DS on a KD. Especially in childhood, the painless and easy detection of low glucose concentrations might lead to improved cognitive performance, and the reduction of hypoglycemia-induced seizures.

scholarly journals The use of the ketogenic diet in the treatment of epileptic encephalopathies

2018 ◽  
Author(s):  
Roberto Caraballo
Keyword(s):  
Ketogenic Diet ◽  
Case Reports ◽  
Case Series ◽  
Dravet Syndrome ◽  
Epilepsy Syndrome ◽  
Behavioral Disturbances ◽  
Paroxysmal Activity ◽  
Epileptic Encephalopathies ◽  
Progressive Encephalopathy ◽  
Epileptic Syndromes

Epileptic encephalopathies(EE) are severe conditions characterized by paroxysmal activity on the electroencephalogram (EEG) that is often aggressive, seizures that are commonly multi-form and intractable, and severe cognitive and behavioral disturbances that present or worsen after the onset of epilepsy. The ketogenic diet (KD) has been shown to be effective in the treatment of refractory epileptic encephalopathies, and has been suggested as an early treatment option in very young children. Although the experience is often anecdotal and mostly consists of case reports and case series, the aim of this study was to present our own experience and an overview of the current literature on the diet in patients with EE. Some encephalopathies, such as epilepsy with myoclonic and atonic seizures, West syndrome, Dravet syndrome, or Lennox-Gastaut syndrome, are well recognized and known to have a good response to the ketogenic diet, while others are more rare or only recently identified, such as epilepsy with focal migrating seizures in infancy, febrile infection-related epilepsy syndrome, or myclonic status in non-progressive encephalopathy, about which little is known and the KD is only tried in sporadic cases. EE are typically refractory to the antiepileptic drugs and the KD  should be considered earlier in the therapeutic scheme of these severe epileptic syndromes. The KD including the oral formula may be considered in all pediatric patients with EE even in infancy.

Vagus Nerve Stimulation in Intractable Epilepsy Associated With SCN1A Gene Abnormalities

2017 ◽  
Vol 32 (5) ◽  
pp. 494-498 ◽  
Author(s):  
Stephen P. Fulton ◽  
Kate Van Poppel ◽  
Amy L. McGregor ◽  
Basanagoud Mudigoudar ◽  
James W. Wheless
Keyword(s):  
Vagus Nerve ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Seizure Control ◽  
Intractable Epilepsy ◽  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy ◽  
Scn1a Gene ◽  
Myoclonic Epilepsy Of Infancy

Mutations in the SCN1A gene cause a spectrum of epilepsy syndromes. There are 2 syndromes that are on the severe end of this spectrum. The classic severe form, Dravet syndrome, is an epileptic encephalopathy of childhood, causing cognitive decline as well as intractable seizures. Severe Myoclonic Epilepsy of Infancy–Borderline (SMEIB) is a term used to include cases with similar severities as those with Dravet syndrome, but lacking a single feature of classic severe myoclonic epilepsy of infancy. Vagus nerve stimulation is a nonpharmacologic treatment for intractable epilepsy. A retrospective review was conducted of patients with deleterious SCN1A mutations who had vagus nerve stimulation placement for treatment of their intractable epilepsy. These children had onset of their epilepsy between 3 and 29 months of age. Seizure control was assessed 6 months after implantation. Twenty patients are included in the study, with 12 implanted at our institution. Nine of the 12 patients implanted at our institution, who had confirmed pre- and post-implantation seizure assessments, showed improvement in seizure control, which was defined as >50% reduction in generalized tonic-clonic seizures, and 4 of those 12 reported improvement in cognitive or speech development. Seven of the 8 patients not implanted at our institution reported subjective benefit, with 4 relating “marked improvement” or seizure freedom. Vagus nerve stimulation appears to impart a benefit to children with deleterious SCN1A gene abnormalities associated with intractable epilepsy.

Severe myoclonic epilepsy of infancy (Dravet syndrome): Recognition and diagnosis in adults

Neurology ◽  
2006 ◽  
Vol 67 (12) ◽  
pp. 2224-2226 ◽  
Author(s):  
F. E. Jansen ◽  
L. G. Sadleir ◽  
L. A. Harkin ◽  
L. Vadlamudi ◽  
J. M. McMahon ◽  
...  
Keyword(s):  
Myoclonic Epilepsy ◽  
Dravet Syndrome ◽  
Severe Myoclonic Epilepsy ◽  
Myoclonic Epilepsy Of Infancy
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