Fenfluramine in the successful treatment of super-refractory status epilepticus in a patient with Dravet syndrome.

A 20-year-old woman with Dravet syndrome and multiple prior episodes of status epilepticus presented to our hospital in November 2018 in super-refractory status epilepticus. After 5 weeks of unsuccessful continuous treatment with anesthetics, including pentobarbital and ketamine, we sought and were granted an emergency approval by the United States Food and Drug Administration to administer fenfluramine, an investigational new drug, to this patient. One week of treatment with fenfluramine at 0.

Pregnancy outcomes of refractory epilepsy patients treated with Brain-responsive neurostimulation.

Objective: To study the pregnancy outcomes, including obstetric complications and fetal outcomes, in pregnant women with epilepsy (WWE) treated with direct brain-responsive neurostimulation (RNS System).

Methods: Retrospective review of obstetric outcomes and fetal outcomes in WWE treated with the RNS System at nine comprehensive epilepsy centers in the United States from 2014-2020. In addition, changes in seizure frequency, anti-seizure medications, and RNS System setting adjustments during pregnancy were investigated.

Epilepsy surgery in the underserved Hispanic population improves depression, anxiety, and quality of life.

Objective: The objective of this study was to investigate the effect of epilepsy surgery on depression, anxiety, and quality of life (QOL) in a Hispanic, primarily immigrant, Spanish-speaking population with intractable epilepsy (IE).

Methods: Patients with IE from a comprehensive epilepsy treatment center in an urban, public healthcare setting who underwent resective brain surgery between 2008 and 2014 (N=47) and completed presurgical and postsurgical neuropsychological evaluation were retrospectively identified. Presurgical and 1-year postsurgical Beck Depression Inventory-II (BDI-II), Beck Anxiety Inventory (BAI), and QOLIE-31 ratings were analyzed as postsurgical outcome measures.

The Weekend Effect on Morbidity and Mortality Among Pediatric Epilepsy Admissions.

Background: Pediatric epilepsy is one of the most common neurological disorders with low mortality and high morbidity, often requiring hospitalization. Weekend admissions have been shown to be associated with worse outcomes compared with their weekday counterparts. To date, no study has assessed the impact of weekend admission on clinical and quality outcomes in the pediatric epilepsy population.

Characterization of electrocorticogram high-gamma signal in response to varying upper extremity movement velocity.

The mechanism by which the human primary motor cortex (M1) encodes upper extremity movement kinematics is not fully understood. For example, human electrocorticogram (ECoG) signals have been shown to modulate with upper extremity movements; however, this relationship has not been explicitly characterized. To address this issue, we recorded high-density ECoG signals from patients undergoing epilepsy surgery evaluation as they performed elementary upper extremity movements while systematically varying movement speed and duration.

Potentially preventable complications in epilepsy admissions: The "weekend effect".

Introduction: Epilepsy affects approximately 1% of the population in the United States with frequent hospital admissions accounting for a significant burden on patients and society as a whole. Weekend admissions have generally been found to have poorer outcomes compared to weekday admissions with increased rates of preventable complications, such as nationally identified "hospital-acquired conditions" (HAC).

Objective: This study aimed to assess the impact of weekend admission on HACs and mortality in the adult epilepsy population.

Dysregulation of PINCH signaling in mesial temporal epilepsy.

Mounting evidence suggests that inflammation is important in epileptogenesis. Particularly Interesting New Cysteine Histidine-rich (PINCH) protein is a highly conserved, LIM-domain protein known to interact with hyperphosphorylated Tau. We assessed PINCH expression in resected epileptogenic human hippocampi and further explored the relationships among PINCH, hpTau and associated kinases.

Unstable periodic orbits in human epileptic hippocampal slices.

Inter-ictal activity is studied in hippocampal slices resected from patients with epilepsy using local field potential recording. Inter-ictal activity in the dentate gyrus (DG) is induced by high-potassium (8 mM), low-magnesium (0.25 mM) aCSF with additional 100 μM 4-aminopyridine(4-AP).

A testbed to explore the optimal electrical stimulation parameters for suppressing inter-ictal spikes in human hippocampal slices.

New interventions using neuromodulatory devices such as vagus nerve stimulation, deep brain stimulation and responsive neurostimulation are available or under study for the treatment of refractory epilepsy. Since the actual mechanisms of the onset and termination of the seizure are still unclear, most researchers or clinicians determine the optimal stimulation parameters through trial-and-error procedures. It is necessary to further explore what types of electrical stimulation parameters (these may include stimulation frequency, amplitude, duration, interval pattern, and location) constitute a set of optimal stimulation paradigms to suppress seizures.

Electrocorticogram encoding of upper extremity movement duration.

Electrocorticogram (ECoG) is a promising long-term signal acquisition platform for brain-computer interface (BCI) systems such as upper extremity prostheses. Several studies have demonstrated decoding of arm and finger trajectories from ECoG high-gamma band (80-160 Hz) signals. In this study, we systematically vary the velocity of three elementary movement types (pincer grasp, elbow and shoulder flexion/extension) to test whether the high-gamma band encodes for the entirety of the movements, or merely the movement onset.

An in vitro seizure model from human hippocampal slices using multi-electrode arrays.

Temporal lobe epilepsy is a neurological condition marked by seizures, typically accompanied by large amplitude synchronous electrophysiological discharges, affecting a variety of mental and physical functions. The neurobiological mechanisms responsible for the onset and termination of seizures are still unclear. While pharmacological therapies can suppress the symptoms of seizures, typically 30% of patients do not respond well to drug control.

Extracting kinetic information from human motor cortical signals.

Brain machine interfaces (BMIs) have the potential to provide intuitive control of neuroprostheses to restore grasp to patients with paralyzed or amputated upper limbs. For these neuroprostheses to function, the ability to accurately control grasp force is critical. Grasp force can be decoded from neuronal spikes in monkeys, and hand kinematics can be decoded using electrocorticogram (ECoG) signals recorded from the surface of the human motor cortex.

Sensitivity and specificity of upper extremity movements decoded from electrocorticogram.

Electrocorticogram (ECoG)-based brain computer interfaces (BCI) can potentially be used for control of arm prostheses. Restoring independent function to BCI users with such a system will likely require control of many degrees-of-freedom (DOF). However, our ability to decode many-DOF arm movements from ECoG signals has not been thoroughly tested.

Spatio-temporal inter-ictal activity recorded from human epileptic hippocampal slices.

Epilepsy is a medical syndrome that produces seizures affecting a variety of mental and physical functions. The actual mechanisms of the onset and termination of the seizure are still unclear. While medical therapies can suppress the symptoms of seizures, 30% of patients do not respond well.

Status epilepticus amauroticus revisited: ictal and peri-ictal homonymous hemianopsia.

Objective: To describe the clinical, electrographic, and radiographic features of status epilepticus amauroticus, or homonymous hemianopsia associated with partial status epilepticus, in 3 patients w:h subsequent resolution of radiographic abnormalities and visual deficits.

Design: Case series.

Setting: Rancho Los Amigos National Rehabilitation Center in Downey, California, and the Los Angeles County + University of Southern California Medical Center.

Correlation between BOLD fMRI and theta-band local field potentials in the human hippocampal area.

The relation between the blood-oxygen-level-dependent (BOLD) signal, which forms the basis of functional magnetic resonance imaging (fMRI), and underlying neural activity is not well understood. We performed high-resolution fMRI in patients scheduled for implantation with depth electrodes for seizure monitoring while they navigated a virtual environment. We then recorded local field potentials (LFPs) and neural firing rate directly from the hippocampal area of the same subjects during the same task.