Evaluation of the Effects of Clobazam on Seizure Control and Quality of Life in Children With Lennox-Gastaut Syndrome: A Pilot Study.

Purpose: This is a prospective study of children with Lennox-Gastaut syndrome receiving clobazam as adjunctive therapy. This pilot study aims to examine medication effectiveness as it relates to seizure reduction, as well as improvement in parent-reported behavior and quality of life (QOL).

Methods: Ten patients with Lennox-Gastaut syndrome aged 3-11 years were enrolled in this 6-week, 4 phase study.

Cognitive Functioning in Patients with Pediatric-Onset Multiple Sclerosis, an Updated Review and Future Focus.

Pediatric-onset multiple sclerosis (POMS) is relatively rare, but as technology and neuroimaging advance, an increasing number of cases are identified, and our understanding of how multiple sclerosis (MS) impacts the developing brain improves. There are consistent findings in the literature highlighting the impact of MS and other demyelinating diseases on cognitive functioning and cognitive development. We also have a better understanding of how POMS impacts psychosocial functioning and functional outcomes in daily living.

Long-Term Neurocognitive, Psychosocial, and Magnetic Resonance Imaging Outcomes in Pediatric-Onset Acute Disseminated Encephalomyelitis.

Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder that is usually self-limited. Recent studies have suggested ongoing neurological deficits and neurocognitive impairment in these patients. Little information on the correlation of clinical and neuroimaging markers in ADEM is available.

Cognitive deficits in pediatric-onset multiple sclerosis: what does the future hold?

Pediatric-onset multiple sclerosis is relatively rare and research regarding disease characteristics is in its infancy. Regardless, there are a growing number of studies finding early cognitive deficits in this population. There are some similarities in outcomes to those seen in the adult-onset multiple sclerosis population, but also several important differences.

Cognitive impairment occurs in children and adolescents with multiple sclerosis: results from a United States network.

In the largest sample studied to date, we measured cognitive functioning in children and adolescents with pediatric multiple sclerosis (n = 187) as well as those with clinically isolated syndrome (n = 44). Participants were consecutively enrolled from six United States Pediatric Multiple Sclerosis Centers of Excellence. Participants had a mean of 14.

Fatigue and depression in children with demyelinating disorders.

Fatigue and depression have been shown to be significant problems in children with multiple sclerosis. The rate at which these conditions occur in children with other acquired demyelinating syndromes is unknown. In this cross-sectional study, the authors evaluated 49 children with demyelinating disorders (multiple sclerosis and acute disseminated encephalomyelitis) and 92 healthy controls for depression and/or fatigue using the Behavior Assessment System for Children, Second Edition behavior and mood rating scale and Varni PedsQL Multidimensional Fatigue Scale.

Acuted disseminated encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is a disorder of the central nervous system (CNS) characterized by an acute event, typically with encephalopathy, in which diffuse CNS involvement occurs. It may follow an infectious event and occurs more commonly in young children. Pulse steroid treatment is frequently used to treat ADEM.

Disease progression in pediatric multiple sclerosis: disparities between physical and neurocognitive outcomes.

Growing information regarding the neurocognitive burden of pediatric multiple sclerosis (MS) has become available in recent years. Whereas natural history studies suggest a longer time to irreversible disability in the pediatric population with MS than in the adult MS population, increasing knowledge suggests a relatively rapid accrual of cognitive deficits in this young population. This article will explore this paradox, taking into account studies that have focused on neuroimaging, cognition and outcomes in pediatric MS.

Cerebellar mutism in pediatric acute disseminated encephalomyelitis.

Acute disseminated encephalomyelitis is a demyelinating process affecting multiple areas of the central nervous system, frequently including the cerebellum. Cerebellar insult may lead to absence of speech or cerebellar mutism. Cerebellar mutism often occurs in young children after posterior fossa tumor resection, and generally appears as part of a larger subset of neurobehavioral signs and personality changes known as posterior fossa syndrome.

Thalamic atrophy and cognition in unilateral temporal lobe epilepsy.

This study examined quantitative magnetic resonance volumes of the thalamus and hippocampus and determined their relationship with cognitive function and clinical seizure characteristics in a sample of 46 unilateral temporal lobe epilepsy (TLE) subjects (20 left and 26 right) and 29 controls. The hippocampus and thalamus exhibited different patterns of volume abnormality, different associations with clinical seizure characteristics, and different patterns of relationship with cognitive measures. Hippocampal volume reduction was primarily ipsilateral to the seizure focus, and thalamic volume reduction was bilateral.

MRI volume loss of subcortical structures in unilateral temporal lobe epilepsy.

Few studies have examined the relative degree of brain volume loss in both the hippocampi and subcortical structures in unilateral temporal lobe epilepsy (TLE) and their association with clinical seizure correlates. In this study, quantitative MRI volumes were measured in the hippocampus, thalamus, caudate, putamen, and corpus callosum in 48 patients with unilateral TLE (26 right, and 22 left) and compared with the volumes of 29 healthy controls. The ipsilateral hippocampus, corpus callosum, and bilateral thalami exhibited the greatest volume loss, reflected by large to moderate effect size differences compared with controls.

Executive functioning in childhood epilepsy: parent-report and cognitive assessment.

There is considerable interest in the assessment of executive function (EF) in pediatric clinical populations but only a few well-standardized measures exist. We examined EF in 53 children aged 8 to 18 years with recent onset epilepsy (31 males, 22 females) and 50 control children (23 males, 27 females) using the Behavior Rating Inventory of Executive Function (BRIEF) and the Delis-Kaplan Executive Function System (D-KEFS). Thirty children had localization-related epilepsy and 23 had idiopathic generalized epilepsy; average duration of 10 months (SD 4y 1mo) and onset age of 11 years 6 months (SD 3y 6mo).

Cerebellar atrophy in temporal lobe epilepsy.

Purpose: The goal of this work was to determine the presence and degree of cerebellar atrophy in chronic temporal lobe epilepsy, its clinical seizure correlates, and its association with general cortical atrophy.

Methods: Study participants were 78 persons with temporal lobe epilepsy and 63 age- and gender-matched healthy controls. All subjects underwent high-resolution MRI with manual tracing of the cerebellum.

Ipsilateral and contralateral MRI volumetric abnormalities in chronic unilateral temporal lobe epilepsy and their clinical correlates.

Purpose: To assess the presence, extent, and clinical correlates of quantitative MR volumetric abnormalities in ipsilateral and contralateral hippocampus, and temporal and extratemporal lobe regions in unilateral temporal lobe epilepsy (TLE).

Methods: In total, 34 subjects with unilateral left (n = 15) or right (n = 19) TLE were compared with 65 healthy controls. Regions of interest included the ipsilateral and contralateral hippocampus as well as temporal, frontal, parietal, and occipital lobe gray and white matter.

Working memory and processing speed deficits in systemic lupus erythematosus as measured by the paced auditory serial addition test.

As many as 66% of systemic lupus erythematosus (SLE) patients have been reported to have cognitive deficits. These deficits are often associated with information processing speed and working memory. Similarly, processing speed and working memory impairments are the hallmark of cognitive dysfunction in multiple sclerosis (MS).