Autoimmune Epilepsy in Children: Unraveling the Mystery.

Although many neurologists are familiar with the clinical presentations of anti-N-methyl-d-aspartate receptor or limbic encephalitides, there remains much mystery surrounding autoimmune etiologies of subacute and chronic epilepsies. In addition, the subtleties and differences in presentation in the pediatric population limit diagnosis and challenge clinicians. In the absence of an acute encephalitic picture, it is likely that many clinicians do not test for autoimmune disorders due to the uncertainty surrounding the selection of appropriate candidates for testing and immunomodulation.

Adult-onset Rasmussen's Syndrome with associated cortical dysplasia.

We describe a 23-year-old woman with previous right temporal lobe surgeries for underlying cortical dysplasia, presenting with right hemispheric seizures and epilepsia partialis continua (EPC). After anti-seizure medication adjustments, she developed focal status epilepticus with progressive EEG and neuroimaging changes. Cerebrospinal fluid and serum autoimmune panels were negative except for an elevated serum acetylcholine-receptor antibody titer, but she underwent immunosuppressive therapy.

Ictal laughter and crying: Should they be classified as automatisms?

Gelastic seizures (GS) describe ictal laughter and are associated with hypothalamic lesions, as well as other cortical areas. Dacrystic seizures (DS), characterized by ictal crying, also have been reported in hypothalamic lesions and focal epilepsy. We describe a young girl with drug resistant focal dyscognitive seizures associated with gelastic and dacrystic features.

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

Objective: 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.

Objective: 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.

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.

Methods: We performed a population-based study to determine the incidence of first unprovoked seizure or newly diagnosed epilepsy. Participants were Northern Manhattan residents seen at area hospitals and nursing homes between 2003 and 2005.

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.

Methods: A prospective cohort study, from 1999 to 2004, included children with first FS from one Pediatric Emergency Department.

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.

Nonconvulsive status epilepticus in children: clinical and EEG characteristics.

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

Objective: To analyze the epidemiological, clinical, and electroencephalograpic features in pediatric patients with NCSE.

Methods: We identified 19 pediatric patients with NCSE from the epilepsy database of the Comprehensive Epilepsy Center at, Columbia University between June 2000 and December 2003.

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.

Seizure characterization and electroencephalographic features in Glut-1 deficiency syndrome.

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

Methods: Twenty children with clinical and laboratory features of Glut-1 DS were evaluated. Age at seizure diagnosis, seizure classification, and response to treatment were determined by chart review.

Treatment for latent TB in correctional facilities: a challenge for TB elimination.

Background: To eliminate tuberculosis (TB) in the United States, more information is needed on how to gain access to difficult-to-reach, high-risk populations to evaluate people who would benefit from treatment for latent TB infection (LTBI).

Methods: A field study was conducted of people at risk for co-infection with TB and the human immunodeficiency virus (HIV) and to demonstrate that treating LTBI in inmates is feasible. Inmates were tested for LTBI using the Mantoux tuberculin skin test (TST).

Glucose transporter 1 deficiency syndrome and other glycolytic defects.

Glucose transporter 1 deficiency syndrome is emblematic of a brain energy failure syndrome. Energy failure also results from other genetically determined metabolic disorders, such as hypoglycemic syndromes, hypoketonemic syndromes associated with fatty acid oxidation defects, glycolytic enzymopathies, and mitochondrial defects. Glucose transporter 1 deficiency syndrome is particularly illustrative of this group of disorders and produces an infantile-onset epileptic encephalopathy that responds to a ketogenic diet.