Malignant migrating partial seizures in infancy are rare epilepsy syndrome that begins in the first 6 months of life and characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns which involved different independent areas of both hemispheres with arrest of psychomotor development. The present detailed review is based on the personal observation of 8 patients newly diagnosed at the Russian Children Clinical hospital, Moscow, Russia. At least three ictal patterns recorded from different independent areas of both hemispheres were fixed by video-EEG-monitoring in all patients. The high polymorphism and very frequent seizures (not less than five types at every child) were observed. The cases were pharmacoresistant, with the absence of reaction to antiepileptic therapy and progressive deterioration in 4 (50%) patients. Decreasing of seizure frequency by 50% was achieved in 3 (37.5%) patients treated with the combination of valproates, benzodiazepines and barbiturates and by 75% in 1 (12.5%) patient case treated with valproates, benzodiazepines and levetyracetam (keppra). The authors proposed a definition of this epileptic syndrome as: "malignant epilepsy of infancy with migrating multifocal seizures".
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Epilepsy Behav
January 2025
Faculty of Medicine, Nursing, and Public Health Universitas Gadjah Mada, Yogyakarta, Special Region of Yogyakarta, Indonesia; Department of Pediatrics, Division of Pediatric Neurology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada - Sardjito General Hospital, Special Region of Yogyakarta, Indonesia.
Objective: To investigate the impact of epilepsy surgery on the developmental outcome in infancy with pharmacoresistant epilepsy and its associated factors.
Method: This systematic review and meta-analysis was conducted in adherence with PRISMA 2020. Literature searching was done using PubMed, CENTRAL, and Scopus database.
Pediatr Neurol
December 2024
Zickler Family Prenatal Pediatrics Institute, Children's National Hospital, Washington, District of Columbia; Department of Neurology, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia; Department of Pediatrics, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia. Electronic address:
Background: Congenital disorders of glycosylation (CDG) are a group of metabolic disorders related to dysfunctional glycoprotein and glycolipid biosynthesis. ALG11-related CDG is a rare member of this group, characterized by severe neurodevelopmental impairment, progressive microcephaly, sensorineural hearing loss, and epilepsy. The objective of this report is to provide an update on the phenotype and brain magnetic resonance imaging (MRI) at age seven years for a patient initially described in early infancy with fetal brain disruption sequence.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Department of Inorganic and Organic Chemistry, Faculty of Experimental Sciences, University of Jaén, Campus of International Excellence in Agri-Food (ceiA3), 23071 Jaén, Spain.
Human lactate dehydrogenase A (LDHA) is a homotetrameric isozyme involved in the conversion of glyoxylate into oxalate in the cytosol of liver cells (hepatocytes) and partially responsible for the overproduction of oxalate in patients with the rare disease called primary hyperoxaluria (PH). Recently, LDHA inhibition has been validated as a safe therapeutic method to try to control the PH disease. Stiripentol (STP) is an approved drug used in the treatment of seizures associated with Dravet's syndrome (a severe form of epilepsy in infancy) which, in addition, has been drawing interest in recent years also for potentially treating PH, due to its LDHA inhibitory activity.
View Article and Find Full Text PDFLife (Basel)
December 2024
Department of Neonatology, Emergency County Hospital Bihor, 410167 Oradea, Romania.
Background And Objective: The effects of neonatal hypoglycemia on the developing brain are well known, resulting in poor neurological outcomes. We aimed to perform an updated meta-analysis on neonatal hypoglycemia, the severity of hypoglycemia, and the associated neurodevelopmental outcomes from infancy to adulthood.
Methods: A systematic literature search was conducted from inception until March 2024, using the PubMed, CINAHL, Embase, and the CENTRAL databases.
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