Related Experiment Video
Updated: May 8, 2026
09:57
Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
Vitamin-responsive epileptic encephalopathies in children
Satish Agadi1, Michael M Quach, Zulfi Haneef
1Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA ; Department of Neurology, Baylor College of Medicine, Houston, TX 77030, USA ; Texas Children's Hospital, 6621 Fannin Street, CCC 1250.03, 12th Floor, Houston, TX 77030, USA.
Epilepsy Research and Treatment
|August 29, 2013
Summary
Epileptic encephalopathies in children can have severe outcomes. This paper reviews specific vitamin-responsive conditions that offer targeted treatments beyond symptomatic seizure control.
Area of Science:
- Pediatric Neurology
- Biochemistry
- Genetics
Background:
- Untreated epileptic encephalopathies in children can lead to severe, long-term consequences.
- Current antiepileptic drugs (AEDs) primarily manage seizures symptomatically, without addressing underlying causes.
- Identifying specific treatable conditions is crucial for potentially altering disease progression.
Purpose of the Study:
- To highlight rare pediatric epileptic encephalopathies with specific, treatable underlying causes.
- To discuss conditions responsive to vitamin or vitamin derivative therapies.
- To emphasize the importance of etiological diagnosis for effective treatment.
Main Methods:
- Literature review of pediatric epileptic encephalopathies.
- Focus on conditions with treatments that modify the natural disease course.
- Analysis of vitamin and vitamin derivative responsive disorders.
Main Results:
- Several specific underlying conditions causing epileptic encephalopathies have been identified.
- These conditions demonstrate responsiveness to targeted vitamin or vitamin derivative treatments.
- Such treatments offer a potential to modify the disease's natural progression.
Conclusions:
- Early diagnosis of specific treatable causes of epileptic encephalopathy is vital.
- Vitamin-responsive disorders represent a key group where specific treatments can significantly impact outcomes.
- Therapeutic strategies targeting underlying deficiencies can offer disease-modifying benefits in pediatric epilepsy.
More Related Videos
Related Concept Videos
01:24Epilepsy and Seizures: Overview
Epilepsy is a chronic neurological disease marked by recurrent, unpredictable seizures. These seizures are caused by abnormal electrical discharges in the brain, leading to behavior, sensation, or consciousness alterations. They can also cause transient impairment of awareness, interfering with daily activities.
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
01:22Epilepsy ll: Types
Recurrent seizures, stemming from abnormal electrical activity in the brain, are the defining characteristic of epilepsy, a chronic neurological condition. Because seizure features vary greatly, epilepsy is classified using two systems: by seizure type and by epilepsy syndromes. These classifications enable clinicians to describe seizure patterns and select suitable treatment strategies.I. Classification by Seizure Type1. Focal EpilepsyFocal epilepsy begins in one hemisphere of the brain.
01:20Antiepileptic Drugs: Modulators of Neurotransmitter Release Mediated by SV2A Protein
Antiepileptic drugs, such as levetiracetam (Keppra) and brivaracetam (Briviact), have emerged as crucial tools in managing epilepsy. These medications exert their therapeutic effects by targeting the synaptic vesicle protein SV2A, a transmembrane glycoprotein primarily found in the brain.
SV2A is a transmembrane glycoprotein located predominantly in the brain, modulating the release of neurotransmitters for neuronal communication. Both levetiracetam and brivaracetam exhibit a high affinity for...
SV2A is a transmembrane glycoprotein located predominantly in the brain, modulating the release of neurotransmitters for neuronal communication. Both levetiracetam and brivaracetam exhibit a high affinity for...
01:13Seizures: Classification
Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
01:19Encephalitis l: Introduction
Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
01:20Antiepileptic Drugs: Potassium Channel Activators
Ezocgabine or retigabine, an antiepileptic drug of remarkable efficacy, has revolutionized the management of seizures. It is a potassium channel activator, explicitly targeting the family of Q subtype potassium channels. It enhances the transmembrane potassium currents, regulating neuronal excitability. This action stabilizes the resting membrane potential, a pivotal factor in mitigating the hyperexcitability that characterizes epilepsy.
Ezogabine has gained approval as an adjunctive treatment...
Ezogabine has gained approval as an adjunctive treatment...