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Epilepsy Behav 2022 Jun 22;133:108784. Epub 2022 Jun 22.

Monroe Carell Jr. Children's Hospital at Vanderbilt University Medical Center, 2200 Children's Way, Nashville, TN 37232, USA. Electronic address:

The benefits of continuous electroencephalography (cEEG) monitoring in the intensive care unit (ICU) are increasingly appreciated, though expanding indications for cEEG may strain resources. The current standard of care in babies with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia (TH) includes cEEG monitoring throughout the entire TH and rewarming process (at least 72 h). Recent cEEG data demonstrate that most seizures occur within the first 24 h of monitoring. Read More

Neurol Clin Pract 2022 Jun;12(3):e22-e24

Department of Neuropediatric (LA), Instituto de Medicina Integral Prof. Fernando Figueira (IMIP); Department of Neonatology (TA, JM), Instituto de Medicina Integral Prof. Fernando Figueira (IMIP); Department of Radiology (AH), Instituto de Medicina Integral Prof. Fernando Figueira (IMIP); Department of Radiology (LL), Hospital das Clínicas da Universidade de São Paulo; Department of Virology (JJFM), Pernambuco State Central Laboratory (LACEN/PE); and Department of Pediatrics (JA), Instituto de Medicina Integral Prof. Fernando Figueira (IMIP), Recife, Brazil.

Because the COVID-19 pandemic evolves, reports of neurologic manifestations and neuroimaging associated with SARS-CoV-2 have been reported in children and in neonates, although very few when compared with adults.

Objectives: To describe a 3-day-old neonate presenting afebrile seizures with a persistent positive nasopharyngeal swab for SARS-CoV-2 and neuroimaging demonstrating extensive cerebral white matter injury.

Methods: Consent was obtained to report this case. Read More

Children (Basel) 2022 May 24;9(6). Epub 2022 May 24.

Study Group for Intensive and Integrated Care of Pediatric Central Nervous System, Department of Pediatrics (iCNS Study Group), Chang Gung Memorial Hospital, Taoyuan 333, Taiwan.

Background: Neonatal encephalopathy is caused by a wide variety of acute brain insults in newborns and presents with a spectrum of neurologic dysfunction, such as consciousness disturbance, seizures, and coma. The increased excitability in the neonatal brain appears to be highly susceptible to seizures after a variety of insults, and seizures may be the first clinical sign of a serious neurologic disorder. Subtle seizures are common in the neonatal period, and abnormal clinical paroxysmal events may raise the suspicion of neonatal seizures. Read More

Maedica (Bucur) 2022 Mar;17(1):107-115

Nice Hospitals, Hyderabad, India.

Doppler ultrasound of fetal vessels plays an important role in diagnosing fetal growth restriction (FGR). It also aids in early detection of fetal compromise and clinical decision making. To determine the efficacy of the pulsatility index (PI) of the fetal umbilical artery (UA) and middle cerebral artery (MCA) in the third trimester of pregnancy for predicting adverse perinatal outcomes in the growth restricted fetuses. Read More

Front Endocrinol (Lausanne) 2022 3;13:818252. Epub 2022 Jun 3.

Pediatric Academic Clinical Programme, Lee Kong Chian School of Medicine, Singapore, Singapore.

Recurrent and profound hypoglycemia is a leading cause of neonatal brain injury. Small-for-gestational-age infants are at risk of hypoglycemia due to substrate deficiency and hyperinsulinism. Inappropriate insulin secretion by the β-cells of the pancreas results in hypoglycemia, neuronal energy deprivation, and parieto-occipital brain injury. Read More

Neurophysiol Clin 2022 Jun 16. Epub 2022 Jun 16.

Children's Neuroscience Service, Department of Neurology, Perth Children's Hospital, Nedlands, Australia; Perron Institute for Neurological and Translational Science, University of Western Australia, Perth, Australia.

Objectives: The important role of the EEG in preterm and term babies in investigating brain function and seizures, predicting outcomes, evaluating therapeutic interventions and decision-making is being increasingly acknowledged. Development of the brain in the last trimester of pregnancy results in rapid changes in the EEG patterns in this period. Acquiring and interpreting the EEG of a preterm baby can be challenging. Read More

Comput Methods Programs Biomed 2022 Jun 10;222:106950. Epub 2022 Jun 10.

Department of Neuroscience, Imaging and Clinical Sciences, University "Gabriele d'Annunzio" of Chieti-Pescara, Chieti, Italy; Behavioral Imaging and Neural Dynamics Center, University "Gabriele d'Annunzio" of Chieti-Pescara, Chieti, Italy; Institute for Advanced Biomedical Technologies, University "Gabriele d'Annunzio" of Chieti-Pescara, Chieti, Italy.

Background And Objective: Neonatal seizures are the most common clinical presentation of neurological conditions and can have adverse effects on the neurodevelopment of the neonatal brain. Visual detection of these events from continuous EEG recordings is a laborious and time-consuming task. We propose a novel algorithm for the automated detection of neonatal seizures. Read More

Dev Med Child Neurol 2022 Jun 16. Epub 2022 Jun 16.

Clinical Neurophysiology, Roger Salengro Hospital, Centre, Lille, France.

Aim: To determine the prognostic value of conventional electroencephalography (EEG) monitoring in neonatal hypoxic-ischemic encephalopathy (HIE).

Method: In this multicentre retrospective study, 95 full-term neonates (mean of 39.3wks gestational age [SD  1. Read More

Am J Perinatol 2022 Jun 16. Epub 2022 Jun 16.

Pediatrics, Saint Louis University School of Medicine, Saint Louis, United States.

Background: Infants with neonatal opioid withdrawal syndrome (NOWS) have disrupted neurobehavior that requires hospitalization and treatment.

Objective: To evaluate EEG abnormalities using amplitude integrated EEG (aEEG) in NOWS.

Design/methods: Eighteen term born infants with NOWS were recruited prospectively for an observational pilot study. Read More

Mol Syndromol 2022 May 1;13(3):226-234. Epub 2022 Feb 1.

Department of Pediatric Metabolism, Ankara University Faculty of Medicine, Ankara, Turkey.

Pontocerebellar hypoplasia (PCH) is a heterogeneous neurodevelopmental disorder that is characterized by decreased brainstem and cerebellum volume. Pontocerebellar hypoplasia type 6 (PCH6) is a mitochondrial disease associated with autosomal recessive inheritance that results from mutations in the gene. In this case report, we describe a new clinical presentation with a novel pathogenic variant. Read More

J Coll Physicians Surg Pak 2022 Jun;32(6):823-825

Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, Pakistan.

This study aimed to determine the clinical spectrum and biochemical findings on urine organic acids (UOA) in Biotin-responsive multiple carboxylase deficiency (MCD) patients presenting to the biochemical genetics laboratory (BGL). Patients reported as MCD, from January 2013-December 2020 were included. The UOA was analysed by gas chromatography mass spectrometer. Read More

J Clin Med 2022 May 27;11(11). Epub 2022 May 27.

Department of Obstetrics and Gynecology, University Hospital Rechts der Isar, Technical University of Munich, 80333 Munich, Germany.

Obese women are at high risk of developing pre-eclampsia (PE). As an altered angiogenic profile is characteristic for PE, measurement of soluble fms-like tyrosine kinase-1 (sFlt-1)/placental growth factor (PIGF) ratio in the maternal serum can be helpful for PE diagnosis, as well as for adverse perinatal outcome (APO) prediction. There is growing evidence that obesity might influence the level of sFlt-1/PIGF and, therefore, the aim of the study was the evaluation of sFlt-1/PIGF as an APO predictor in obese women with PE. Read More

Clin Chim Acta 2022 Jul 6;532:115-122. Epub 2022 Jun 6.

Department of Genetics, College of Basic Medical Sciences, Jilin University, Changchun, Jilin 130021, China. Electronic address:

Background: Isolated sulfite oxidase deficiency (ISOD) is a rare type of life-threatening neurometabolic disorders characterized by neonatal intractable seizures and severe developmental delay with an autosomal recessive mode of inheritance. Germline mutation in SUOX gene causes ISOD. Till date, only 32 mutations of SUOX gene have been identified and reported to be associated with ISOD. Read More

J Neurophysiol 2022 Jun 8. Epub 2022 Jun 8.

Department of Neurosurgery, University of Utah School of Medicine, Salt Lake City, UT, United States.

Electrographic seizures and abnormal background activity in the neonatal electroencephalogram (EEG) may differentiate between harmful versus benign brain insults. Using two animal models of neonatal seizures, electrical activity was recorded in freely behaving rats and examined quantitatively during successive time periods with field-potential recordings obtained shortly after the brain insult (i.e. Read More

Hosp Pediatr 2022 Jun 8. Epub 2022 Jun 8.

Background And Objective: Neonatal seizures are one of the most challenging problems for experts across the globe. Although there is no consensus on the "ideal" treatment of neonatal seizures, phenobarbitone has been the drug of choice for decades. Unfortunately, although extensively studied in adults and children, levetiracetam lacks rigorous evaluation in the neonatal population, despite its frequent use as an off-label drug. Read More

Eur Heart J Case Rep 2022 Jun 24;6(6):ytac209. Epub 2022 May 24.

Nork-Marash Medical Center, Armenak Armenakyan St. 13, 0047 Yerevan, Armenia.

Background: Tetralogy of Fallot (TOF) is one of the most widespread cyanotic congenital heart disease (CHD), which can be successfully repaired in the neonatal period. However, residual problems and the surgical technique itself can create a favourable basis for arrhythmias and conduction abnormalities in these patients. Sometimes, these arrhythmias may worsen during pregnancy and require urgent intervention. Read More

Am J Obstet Gynecol MFM 2022 Jun 3;4(5):100672. Epub 2022 Jun 3.

Division of Maternal-Fetal Medicine, Department of Obstetrics, Gynecology, and Reproductive Sciences, McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX.

Background: Small-for-gestational-age neonates (birthweight of <10th percentile for gestational age) are significantly more likely to have multiple adverse outcomes than appropriate-for-gestational-age neonates (birthweight of 10th-90th percentile). Most small-for-gestational-age neonates are undetected during pregnancy (ie, not diagnosed as fetal growth restriction), but the sequela of being undetected remains uncertain.

Objective: The primary objective of this study was to compare the composite neonatal adverse outcomes among singleton pregnancies that were at least 37 weeks and delivered small-for-gestational-age neonates, which were diagnosed as either fetal growth restriction during pregnancy (detected small for gestational age) or not (undetected small for gestational age). Read More

Cureus 2022 Apr 30;14(4):e24629. Epub 2022 Apr 30.

Microbiology, Byramjee Jeejeebhoy (BJ) Government Medical College & Sassoon General Hospitals, Pune, IND.

Background The Omicron variant of SARS-CoV-2 infection was seen to be more infectious but less severe in children than adults with reduced hospitalization rates. There is a paucity of data on hospitalized children with confirmed Omicron variant. Objective We describe demographic, epidemiologic, clinical, radiological, laboratory features and outcomes of children with confirmed Omicron variant of SARS-CoV-2 infection admitted to a tertiary care teaching hospital in Pune, India. Read More

Am J Obstet Gynecol 2022 Jun 2. Epub 2022 Jun 2.

Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.

Background: Although there is a well-known association of fetal bradycardia associated with maternal eclampsia, the characteristics of fetal heart rate tracings following an eclamptic seizure have not previously been thoroughly described. Fetal heart rate changes are thought to be related to maternal lactic acidemia caused by vasospasm and uterine hyperactivity leading to placental hypoperfusion and fetal hypoxia. The decision to intervene in the case of an abnormal fetal heart rate tracing following an eclamptic seizure is often difficult, however, maternal resuscitation should be the primary focus. Read More

World Neurosurg 2022 Jun 2. Epub 2022 Jun 2.

Department of Neurological Surgery, Policlinico "G. Rodolico-S. Marco" University Hospital, Catania, Italy; Interdisciplinary Research Center on Brain Tumors Diagnosis and Treatment, University of Catania, Catania, Italy.

Ping-pong fractures (PPF) have become less frequent and no definite predictors to determine which fractures will elevate spontaneously and which should undergo surgical treatment have not been clearly defined. Herein, the authors present a revision of the literature, in which 54 papers were included, with a total of 228 children studied. Patients who underwent surgery accounted for 30%; elevation through obstetrical vacuum or other aspiration systems was applied in 30%, spontaneous resolution occurred in 40%; in 4 patients percutaneous microscrew elevation was applied. Read More

Front Genet 2022 13;13:880464. Epub 2022 May 13.

Genetics and Precision Medicine Department, King Abdullah Specialized Children Hospital, King Abdulaziz Medical City MNG-HA, Riyadh, Saudi Arabia.

3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (HMG-CoA lyase) is a rare inborn error of leucine degradation and ketone body synthesis, caused by homozygous or compound heterozygous disease-causing variants in . To understand the natural history of this disease, we reviewed the biochemical, clinical, and molecular data of 62 patients from 54 different families with confirmed HMG-CoA lyase deficiency (HMGCLD) diagnosis from Saudi Arabia. The majority of the affected individuals were symptomatic. Read More

Curr Drug Saf 2022 May 31. Epub 2022 May 31.

Department of Pharmacology, SRM Medical College Hospital and Research Centre, Kattankulathur, TamilNadu, India.

Background: Drug-induced hypersensitivity reaction is a potentially life-threatening condition reported among patients of different age groups. Phenytoin is a prototypic drug prescribed for the treatment of a variety of seizure disorders. Allergic reaction to phenytoin therapy in a newborn is relatively a rare clinical manifestation that is not frequently reported. Read More

Eur J Paediatr Neurol 2022 May 7;39:25-29. Epub 2022 May 7.

Department of Pediatrics & Human Genetics, Emma Children's Hospital, Amsterdam University Medical Center, University of Amsterdam, the Netherlands; United for Metabolic Diseases, the Netherlands; Emma Center for Personalized Medicine, Amsterdam Reproduction and Development, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands. Electronic address:

Background: A rare subset of vitamin B6 responsive seizure disorders does not respond to pyridoxine, and requires the active form of vitamin B6, pyridoxal-5'-phosphate (PLP), to maintain seizure control. Patients with PLP-responsive seizures are dependent on chronic PLP treatment, yet no licensed PLP product is available. PLP food supplements, a product category regulated less stringently than medication, may prove of insufficient effectiveness and safety. Read More

Ann Clin Transl Neurol 2022 May 28. Epub 2022 May 28.

Department of Pediatric Neurology, Queen Fabiola Children's Hospital-ULB, Brussels, Belgium.

Recessive mutations in the SLC13A5 gene encoding the sodium-dependent citrate transporter are a recently identified cause of developmental and epileptic encephalopathy. Here, we describe a child harboring a novel homozygous loss-of-function mutation in the SLC13A5 gene (c.1496C>T-p. Read More

Children (Basel) 2022 May 5;9(5). Epub 2022 May 5.

Department of Neonatology, Reims University Hospital Alix de Champagne, 51100 Reims, France.

Pediatric morbidity from meningitis remains considerable. Preventing complications is a major challenge to improve neurological outcome. Seizures may reveal the meningitis itself or some complications of this disease. Read More

Pediatr Endocrinol Diabetes Metab 2022 May 27. Epub 2022 May 27.

Department of Pediatrics, Nutrition and Metabolic Disorders, Children's Memorial Health Institute, Warsaw, Poland.

Glycogen storage disease type 1b (GSD 1b) is an inherited metabolic defect caused by a deficiency of microsomal glucose-6-phosphate (G6P) transport protein across the endoplasmic reticulum membrane. Patients with GSD 1b have hypoglycemia episodes, lactate acidosis, hypertriglyceridemia, hypercholesterolemia, hyperuricemia, neutropenia and in imaging studies hepatomegaly and/or nephromegaly. The primary goals of treatment are to maintain proper blood glucose levels and to increase the number of properly functioning neutrophils. Read More

Epilepsy Behav Rep 2022 8;19:100549. Epub 2022 May 8.

Department of Neurology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, United Kingdom.

Pathogenic variants in are associated with a spectrum of clinical syndromes ranging from Lethal Neonatal Rigidity and Multifocal Seizure syndrome (RMFSL) to Neurodevelopmental Disorder with Cerebellar Atrophy and with or without Seizures (NEDCAS). RMFSL is characterized by early-onset multifocal seizures with microcephaly. Death occurs during infancy although a less severe course with later onset seizures and longer survival into childhood has been described. Read More

MMWR Morb Mortal Wkly Rep 2022 May 27;71(21):703-708. Epub 2022 May 27.

Seizures, transient signs or symptoms caused by abnormal surges of electrical activity in the brain, can result from epilepsy, a neurologic disorder characterized by abnormal electrical brain activity causing recurrent, unprovoked seizures, or from other inciting causes, such as high fever or substance abuse (1). Seizures generally account for approximately 1% of all emergency department (ED) visits (2,3). Persons of any age can experience seizures, and outcomes might range from no complications for those with a single seizure to increased risk for injury, comorbidity, impaired quality of life, and early mortality for those with epilepsy (4). Read More

Zh Nevrol Psikhiatr Im S S Korsakova 2022 ;122(5):128-131

National Medical Research Center of Children's Health, Moscow, Russia.

A clinical case of a patient with neonatal epilepsy at the age of 5 months, with impaired bone formation, early osteoporosis and frequent limb fractures is described. Panel sequencing confirmed by Sanger sequencing revealed two independent hereditary diseases - osteogenesis imperfect type 1, associated with a mutation in the gene (c.2010delT) and benign non-familial infantile seizures associated with a mutation in the gene (c. Read More