Objective: Fast oscillations (FOs) were first explored from scalp electroencephalographic (EEG) data from hypsarrhythmia in West syndrome (infantile spasms) to investigate the meaning of FOs in this epileptic encephalopathy.
Methods: In 17 infants with West syndrome, we conservatively detected fast frequency peaks that stood out from the time-frequency spectral background with square root power > 1µV (spectral criterion) and corresponded to clear FOs with at least 4 oscillations in the filtered EEG traces (waveform criterion) in sleep EEGs.
Results: We found a total of 1,519 interictal FOs that fulfilled both the spectral and waveform criteria. The FOs with a median frequency of 56.6Hz (range = 41.0-140.6Hz) were dense, with a median rate of 66 (range = 24-171) per minute before adrenocorticotropic hormone (ACTH) treatment, which was significantly higher than that in control infants without seizures (median = 1, p < 0.001). The FOs were reduced by treatment. The mean gamma and ripple oscillation rates that were detected using the waveform criterion alone were 40.62/min and 15.75/min, respectively, per channel; these results were 112.8 and 98.4 times higher, respectively, than the previously reported corresponding rates in adult epilepsy patients.
Interpretation: The observed FOs corresponded to epileptogenicity because of their close relation to the severity of hypsarrhythmia during the course of ACTH treatment. The very high epileptic FO rates in hypsarrhythmia are thought to affect the process of neurodevelopment by interfering with physiological functions in West syndrome, taking into account that high frequencies are also important in physiological higher brain functions.
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http://dx.doi.org/10.1002/ana.24299 | DOI Listing |
Seizure
January 2025
Division of Neurology, Saitama Children's Medical Center, Saitama, Japan.
Neurology
February 2025
Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
Pathogenic variants in cause congenital muscular dystrophy through hypoglycosylation of alpha-dystroglycan (OMIM #615350). The established phenotypic spectrum of GMPPB-related disorders includes recurrent rhabdomyolysis, limb-girdle muscular dystrophy, neuromuscular transmission abnormalities, and congenital muscular dystrophy with variable brain and eye anomalies. We report a 9-month-old male infant with congenital muscular dystrophy, infantile spasms, and compound heterozygous pathogenic variants (c.
View Article and Find Full Text PDFJ Neurol
January 2025
Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, 610041, China.
Background: Tourette syndrome (TS) is a prevalent neurodevelopmental disorder with an uncertain etiology. Numerous neuroimaging studies have investigated patients with TS, but their conclusions remain inconsistent. The current study attempted to provide an unbiased statistical meta-analysis of published neuroimaging studies of TS.
View Article and Find Full Text PDFeNeurologicalSci
March 2025
Department of Neurology, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan.
L-type calcium channel antagonists are uncommon causes of myoclonus, and the underlying mechanism remains unclear. Here, we report a case of parkinsonian syndrome with deterioration of preexisting myoclonus after nifedipine use. A 96-year-old woman was administered a single dose of sustained-release nifedipine for chest pain.
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January 2025
Treatment Center for Traumatic Injuries, The Third Affiliated Hospital, Southern Medical University, No. 183, Zhongshan Avenue West, Tianhe District, Guangzhou, 510063, Guangdong, China.
In the past two decades, record-breaking heat waves have caused an increasing number of heat-related deaths, including heatstroke, globally. Heatstroke is a life-threatening systemic condition characterized by a core body temperature >40°C and the subsequent development of multiple organ dysfunction syndrome. Lung injury is a well-documented complication of heatstroke and is usually the secondary cause of patient death.
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