Linda Leary

Nonconvulsive Status Epilepticus in Children: Clinical and EEG Characteristics

Summary:  Background: Nonconvulsive status epilepticus (NCSE) is a highly heterogeneous clinical condition that is understudied in the pediatric population.

Seizure characterization and electroencephalographic features in Glut-1 deficiency syndrome

Summary:  Purpose: To characterize seizure types and electroencephalographic features of glucose transporter type 1 deficiency syndrome (Glut‐1 DS).

Prevalence of self-reported epilepsy in a multiracial and multiethnic community in New York City.

PURPOSE To estimate the prevalence of epilepsy in a racially and ethnically diverse neighborhood in New York City. METHODS We used random-digit dialing to identify people with a history of epilepsy. We estimated the prevalence of active epilepsy and lifetime epilepsy. RESULTS The age-adjusted prevalence of active epilepsy was 5.0 per 1000, and that of lifetime epilepsy was 5.9 per 1000. Prevalence appeared higher in Hispanics (active prevalence: 6.3 per 1000; lifetime prevalence: 7.5 per 1000) than in non-Hispanics (active prevalence: 4.1 per 1000; lifetime prevalence: 4.7 per 1000). Blacks appeared to have a lower prevalence of active epilepsy (5.2 per 1000) than whites (5.9 per 1000), but a higher lifetime prevalence (7.5 per 1000 vs. 5.9 per 1000). Ethnic and racial differences in access to epilepsy care were evident both in terms of drug treatment and use of emergency departments for care. CONCLUSIONS The prevalence of epilepsy in this predominantly minority urban community is similar to that reported in other contemporary studies. Less access to health care for black and Hispanic respondents, compared with white respondents, may have influenced self-reported active epilepsy prevalence estimates since the definition includes recent use of antiseizure medication.

Estimating the incidence of first unprovoked seizure and newly diagnosed epilepsy in the low-income urban community of Northern Manhattan, New York City

Purpose: To estimate the incidence and mortality associated with first unprovoked seizure or newly diagnosed epilepsy in a low‐income, predominantly Hispanic community in Northern Manhattan, New York City.

Risk of Intracranial Pathologic Conditions Requiring Emergency Intervention After a First Complex Febrile Seizure Episode Among Children

OBJECTIVE. To determine the likelihood of intracranial pathologic conditions requiring emergency neurosurgical or medical intervention among children without meningitis who presented to the pediatric emergency department after a first complex febrile seizure. METHODS. We performed a retrospective review of prospectively collected data for children in neurologically normal condition who presented to a single pediatric emergency department after a first complex febrile seizure (focal, multiple, or prolonged). The complex febrile seizure classification was determined independently by 2 epileptologists. The presence of intracranial pathologic conditions was determined through review of neuroimaging results, telephone interviews, or medical record review. RESULTS. Data for 71 children with first complex febrile seizures were analyzed. Fifty-one (72%) had a single complex feature (20 focal, 22 multiple, and 9 prolonged), and 20 (28%) had multiple complex features. None of the 71 patients (1-sided 95% confidence interval: 4%) had intracranial pathologic conditions that required emergency neurosurgical or medical intervention. CONCLUSIONS. For children with first complex febrile seizures, the risk of intracranial pathologic conditions that require emergency neurosurgical or medical intervention is low, which suggests that routine emergency neuroimaging for this population is unnecessary.

Are MRI‐detected brain abnormalities associated with febrile seizure type?

Objective: Whether magnetic resonance imaging (MRI) is informative in febrile seizures (FS) is unknown. We undertook a study to determine the frequency of MRI‐detected brain abnormalities and to evaluate their association with FS type and with specific features of complex FS.

Electromyography-based seizure detector: Preliminary results comparing a generalized tonic-clonic seizure detection algorithm to video-EEG recordings

Automatic detection of generalized tonic–clonic seizures (GTCS) will facilitate patient monitoring and early intervention to prevent comorbidities, recurrent seizures, or death. Brain Sentinel (San Antonio, Texas, USA) developed a seizure‐detection algorithm evaluating surface electromyography (sEMG) signals during GTCS. This study aims to validate the seizure‐detection algorithm using inpatient video–electroencephalography (EEG) monitoring.

Acute hyperglycemia produces transient improvement in glucose transporter type 1 deficiency

Glucose transporter type 1 deficiency syndrome (Glut1‐DS) is characterized clinically by acquired microcephaly, infantile‐onset seizures, psychomotor retardation, choreoathetosis, dystonia, and ataxia. The laboratory signature is hypoglycorrhachia. The 5‐hour oral glucose tolerance test (OGTT) was performed to assess cerebral function and systemic carbohydrate homeostasis during acute hyperglycemia, in the knowledge that GLUT1 is constitutively expressed ubiquitously and upregulated in the brain.

Underlying cause of death in incident unprovoked seizures in the urban community of Northern Manhattan, New York City

We determined underlying cause‐specific mortality for incident unprovoked seizures from Northern Manhattan, New York City. We calculated the case fatality, proportionate mortality, and the underlying cause‐specific standardized mortality ratios (SMRs), with U.S. death rates as the standard. Thirty‐two deaths were observed between 2003 and 2007 among 209 participants. Case fatality was significantly lower for idiopathic/cryptogenic seizures versus symptomatic seizures. About 31.3% of the deaths were attributed to malignant neoplasms, 25.0% to diseases of the heart, 15.6% to influenza and pneumonia, 3.1% to cerebrovascular diseases, and 25.0% to other causes. Significant SMRs were observed for all causes (SMR = 1.6), influenza and pneumonia (SMR = 7.1), and malignant neoplasms (SMR = 2.9). Younger cases (<65 years) had increased SMRs for all causes, malignant neoplasms, and other causes. Older cases (≥65 years) had increased SMRs for influenza and pneumonia. Underlying cause of death paralleled the underlying cause of seizure in patients with symptomatic etiologies.

Subdural electrode recording of generalized photoepileptic responses

We evaluated the spatiotemporal distribution of photic driving (PDR), photoparoxysmal (PPR), and photoconvulsive (PCR) responses recorded by intracranial electrodes (ic-EEG) in a patient with generalized photosensitivity and right frontal lobe cortical dysplasia. Intermittent light stimulation (ILS) was performed thirteen times in nine days. Cortical responses to ILS recorded by ic-EEG were reviewed and classified as PDRs, PPRs, and PCRs. Photic driving responses were restricted to the occipital lobe at ILS frequencies below 9 Hz, spreading to the parietal and central regions at > 9 Hz. Photoparoxysmal responses commonly presented as focal, medial occipital, and parietal interictal epileptic discharges (IEDs), the latter propagating to the sensorimotor cortices. Generalized IEDs were also generated in the setting of PPRs. Photoconvulsive responses, characterized by repetitive bilateral upper extremity myoclonus sustained until the end of the stimulus, were associated with propagation of the medial parieto-occipital discharge to the primary sensorimotor and supplementary area cortices, while generalized myoclonic seizures were associated with a generalized spike-and-wave discharge with an interhemispheric posterior cingulate onset sparing the sensorimotor cortices. Both types of PCR could occur during the same stimulus. Regardless of the pathway, PCRs only occurred when PDRs involved the parietal cortices. While there may be more than one pathway underlying PCRs, parietal lobe association cortices appear to be critical to their generation.