Characterization of the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G). Seven children were retrospectively identified as having COE-G and were followed-up clinically using EEGs. Visual manifestations were the most common ictal event. Eye-associated ictal deviation was associated with ipsilateral turning of the head and migraine-like symptoms were frequent. Hemiconvulsions occurred in two children, and only one child had secondary generalized tonic-clonic seizures. In all patients, seizures occurred while awake, while two patients also had seizures while sleeping. EEG showed five patients with occipital spike-wave discharges when their eyes were closed which disappeared once their eyes were opened. Two cases continued having frequent seizures despite antiepileptic drug treatment. These patients also displayed learning difficulties and behavioral impairments after seizure onset. COE-G is a distinctive epileptic syndrome; however, the long-term prognosis for patients with the condition is unclear.
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http://dx.doi.org/10.1007/s12013-013-9594-8 | DOI Listing |
Dev Cogn Neurosci
December 2024
Division of Psychology and Language Sciences, UCL, London WC1H 0AP, UK. Electronic address:
Executive functions can be classified into processes of inhibition, working memory and shifting, which together support flexible and goal-directed behaviour and are crucial for both current and later-life outcomes. A large body of literature has identified distinct brain regions critical to performing each of these functions. These findings are however predicated on a piecemeal and single-task approach.
View Article and Find Full Text PDFNeurol Sci
December 2024
Neurophysiopathology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Introduction: Biallelic variants in QARS1, a house-keeping gene involved in protein synthesis, cause a rare encephalopathy classically characterized by severe developmental delay, drug-resistant neonatal-onset epilepsy, microcephaly, and brain atrophy. We aim to raise awareness on mild QARS1-related phenotypes describing a 6-year-old patient.
Case Description: Epilepsy onset occurred at 3.
Quant Imaging Med Surg
December 2024
Department of Radiology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Neurodevelopment and Cognitive Disorders, Chongqing, China.
Background: Little is known about the precise impaired patterns of white matter (WM) fiber tracts in preschool-aged children with autism spectrum disorder (ASD). Thus, we used diffusion tensor imaging (DTI)-based automated fiber quantification (AFQ) to explore the changes in WM fiber tracts in preschool-aged children with ASD and its correlation with the severity of clinical manifestations.
Methods: A total of 43 pediatric ASD and 42 age- and sex-matched typical developing children were examined with DTI.
Brain Dev
December 2024
Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry (NCNP), Japan; Department of Pediatrics, Tottori Prefectural Tottori Rehabilitation Center, Japan.
Background: Childhood cerebral type of Adrenoleukodystrophy (CC-ALD) is fatal without hematopoietic stem cell transplantation (HSCT). We consider whether EEGs showing focal paroxysmal delta waves can be a candidate of early detector of the apparent ALD and HSCT therapy.
Methods: Twenty-two male children with ALD (5-16 years; 10.
Zh Vopr Neirokhir Im N N Burdenko
December 2024
Irkutsk State Medical University, Irkutsk, Russia.
Background: Giant non-traumatic and non-iatrogenic cranial vault defects are poorly studied due to their rarity. Therefore, diagnosis and analysis of their causes are difficult. In available literature, we found only 4 cases of giant pericranial sinus accompanied by extensive cranial vault defects.
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