98%
921
2 minutes
20
Abnormal cortical excitation in response to photic stimulation (photosensitivity) has historically been associated with generalized epilepsies, in patients outside of infancy. At our tertiary centre, we encountered a patient with infantile spasms secondary to a mutation in ALG13 (c320A>G) who had photic stimulation-induced epileptic spasms over a broad range of frequencies on multiple EEGs, which were worse without treatment and decreased as treatment was escalated. This is the first reported case of epileptic spasms triggered by photic stimulation and it is unclear whether the phenomenon is unique to this patient, to those with this mutation or whether it is present in a broader group of patients with infantile spasms.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1684/epd.2021.1370 | DOI Listing |
Behav Brain Res
September 2025
Department of Pharmacology, Research Block B, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India. Electronic address:
Infantile Epileptic Spasms Syndrome (IESS), also referred to as West syndrome, is a severe epileptic disorder that emerges during early childhood. It is marked by characteristic epileptic spasms, developmental stagnation or regression, and a distinctive electroencephalogram (EEG) pattern known as hypsarrhythmia. To better understand the underlying mechanisms of IESS, various genetic and chemically induced animal models have been developed.
View Article and Find Full Text PDFEpilepsy Behav
September 2025
Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada. Electronic address:
Objective: Electronic health records offer the opportunity for quality-improvement (QI) initiatives addressing health disparities in epilepsy care. The Pediatric Epilepsy Outcome-Informatics Project (PEOIP) at Alberta Children's Hospital implemented point-of-care data entry into a standardized pediatric epilepsy electronic note as part of routine clinical care to support QI initiatives. Our study validated collected data by assessing the prevalence of patient characteristics, ethno-racial background, and 4 of the most common severe epilepsy syndromes: infantile epileptic spasms, Dravet, Lennox-Gastaut, and developmental epileptic encephalopathy with spike-wave action in sleep.
View Article and Find Full Text PDFPediatr Neurol
August 2025
Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Background: The Burden of AmplitudeS and Epileptiform Discharges (BASED) and "Hypsarrhythmia Scoring System" (HSS) serve to evaluate the interictal EEG in infantile epileptic spasms (ES) syndrome (IESS). We aimed to assess these scoring systems' reliability and diagnostic utility in infants with IESS and other developmental and epileptic encephalopathies (DEEs) without ES.
Methods: Three epileptologists from a single medical center scored the deidentified EEG tracings of 110 infants, 58 with IESS, and 52 with other DEEs (of similar age and sex distributions), according to the BASED and HSS scoring systems.
Dev Med Child Neurol
September 2025
Xiangya Hospital, Central South University - Pediatric Neurology, Changsha, Hunan, China.
Behav Neurol
September 2025
Department of Electronics and Communication Engineering, Chettinad Academy of Research and Education, Manamai Campus, Chennai, Tamil Nadu, India.
Temporary disturbances in brain function are caused by epilepsy, a chronic disorder resulting from sudden abnormal firing of brain neurons. This research introduces an innovative real-time methodology representing detecting epileptic spasms from electroencephalogram (EEG) data. It employs a support vector machine (SVM) alongside embedded zero tree wavelet (EZW) transform.
View Article and Find Full Text PDF