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Distinct T Cell Dysregulation Reflects Disease Severity and Progression in Infantile Epileptic Spasms Syndrome and Lennox-Gastaut Syndrome. [PDF]

open access: yesImmune Netw
Chang L   +11 more
europepmc   +1 more source
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Infantile epileptic spasms syndrome: When spasms come out of the blue

Epilepsy and Behavior
This study evaluates the electroclinical features of infantile epileptic spasms syndrome (IESS) suddenly appearing in previously normal patients, aiming to describe clinical outcomes and independent predictors.We retrospectively selected a homogeneous group of patients with IESS from two Italian centers.
Federico Vigevano   +2 more
exaly   +3 more sources

Cohort study of infantile epileptic spasms syndrome: etiological analysis and treatment of corticosteroids

Seizure: the Journal of the British Epilepsy Association, 2022
Infantile epileptic spasms syndrome (IESS) is the most common type of severe epilepsy in infants. However, etiological frequency and optimized therapy, particularly corticosteroid regimen and dose, remain unknown.An ambispective study of an IESS-diagnosed cohort was conducted.
Xiaohua Liang   +2 more
exaly   +3 more sources

Timing the clinical onset of epileptic spasms in infantile epileptic spasms syndrome: A tertiary health center's experience

Epilepsia
AbstractObjectiveLead time to treatment (clinical onset of epileptic spasms [ES] to initiation of appropriate treatment) is known to predict outcomes in infantile epileptic spasms syndrome (IESS). Timing the clinical onset of ES is crucial to establish lead time. We investigated how often ES onset could be established to the nearest week.
Heather Olson   +2 more
exaly   +3 more sources

Etiology of Infantile Epileptic Spasms Syndrome and Clinical Response With Vigabatrin as the First Treatment

Pediatric Neurology
Etiology is an important predictor for treatment outcomes of infantile epileptic spasms syndrome (IESS). In Thailand, vigabatrin (VGB) is the first-line treatment for all patients due to the unavailability of adrenocorticotropic hormone. We aimed to determine the etiology of IESS using the 2017 International League Against Epilepsy classification and ...
Kullasate Sakpichaisakul   +2 more
exaly   +3 more sources

Response to nitrazepam in infantile epileptic spasms syndrome after failed standard treatment

Epilepsy and Behavior
Over half of children with infantile epileptic spasms syndrome (IESS) do not respond to standard treatments. Nitrazepam has shown efficacy similar to adrenocorticotrophic hormone. This study evaluated response rates, adverse effects of nitrazepam, and factors associated with responders in children with IESS who failed standard treatment.We conducted a ...
Kullasate Sakpichaisakul   +1 more
exaly   +3 more sources

Infantile epileptic spasm syndrome as a new NR2F1 gene phenotype

International Journal of Developmental Neuroscience, 2023
AbstractIntroductionNR2F1 pathogenetic variants are associated with the Bosch–Boonstra–Schaaf optic atrophy syndrome (BBSOAS). Recent studies indicate that BBSOAS patients not only have visual impairments but may also have developmental delays, hypotonia, thin corpus callosum and epileptic seizures.
Yan Liang   +5 more
openaire   +2 more sources

Infantile Epileptic Encephalopathy with Hypsarrhythmia (Infantile Spasms/West Syndrome) and Immunity

open access: yesCentral Nervous System Agents in Medicinal Chemistry, 2008
West syndrome is a severe epilepsy, occurring in infancy, that comprises epileptic seizures known as spasms, in clusters, and a unique EEG pattern, hypsarrhythmia, with psychomotor regression. Maturation of the brain is a crucial component.
Terezinha Cresci Braga Montelli   +1 more
openaire   +2 more sources

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