Chellamani Harini
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A research roadmap for SCN8A-related disorders: addressing knowledge gaps and aligning research priorities across stakeholders Open
Background Despite significant scientific progress since the 2012 discovery that variants in the SCN8A gene can cause human epilepsy, disease mechanisms and best practices for management of SCN8A-related disorders (SCN8A-RD) remain incompl…
View article: Comparative effectiveness of second‐line treatments for epileptic spasms
Comparative effectiveness of second‐line treatments for epileptic spasms Open
Objective This study was undertaken to evaluate the response to second treatments for infantile epileptic spasms syndrome (IESS). Methods Infants aged 2–24 months with IESS were prospectively enrolled in the National Infantile Spasms Cohor…
View article: Outcomes following resective and disconnective strategies in the treatment of epileptic spasms: a systematic review of the literature and individual patient data meta-analysis
Outcomes following resective and disconnective strategies in the treatment of epileptic spasms: a systematic review of the literature and individual patient data meta-analysis Open
Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. Whil…
A Multicenter Training and Interrater Reliability Study of the BASED Score for Infantile Epileptic Spasms Syndrome Open
Purpose: The best possible outcomes in infantile epileptic spasms syndrome require electroclinical remission; however, determining electrographic remission is not straightforward. Although the determination of hypsarrhythmia has inadequate…
View article: The spectrum of movement disorders in young children with <scp><i>ARX</i></scp>‐related <scp>epilepsy‐dyskinesia</scp> syndrome
The spectrum of movement disorders in young children with <span><i>ARX</i></span>‐related <span>epilepsy‐dyskinesia</span> syndrome Open
Children with developmental and epileptic encephalopathies often present with co‐occurring dyskinesias. Pathogenic variants in ARX cause a pleomorphic syndrome that includes infantile epilepsy with a variety of movement disorders ranging f…
Initial combination versus early sequential standard therapies for Infantile Epileptic Spasms Syndrome—Feedback from stakeholders Open
We read with great interest the report by Sourbron et al. 1 entitled "Medical Treatment in Infants and Young Children with Epilepsy: Off-label Use of Antiseizure Medications.Survey Report of ILAE Task Force Medical Therapies in Children".W…
Influence of extent and age at corpus callosotomy on seizure outcomes. A single center experience Open
Corpus callosotomy (CC) is a palliative treatment for drop seizures in patients with drug‐resistant nonlocalizable epilepsy. We compared drop seizure outcomes between patients undergoing anterior CC versus complete CC and examined factors …
View article: Utility of Exome Sequencing for Diagnosis in Unexplained Pediatric-Onset Epilepsy
Utility of Exome Sequencing for Diagnosis in Unexplained Pediatric-Onset Epilepsy Open
Importance Genomic advances inform our understanding of epilepsy and can be translated to patients as precision diagnoses that influence clinical treatment, prognosis, and counseling. Objective To delineate the genetic landscape of pediatr…
Influence of different feeding rates of polychaete worms, Eulalia viridis (Annelida, Polychaeta), on the gonad development of male Penaeus semisulcatus (Decapoda, Dendrobranchiata) under captive conditions Open
Penaeus semisulcatus is a large growing species, attaining a maximum size of about 250 mm in total length. It is more resistant to white spot syndrome virus (WSSV) than other cultivable penaeid shrimps, which makes it a potential candidate…
View article: Epileptic spasms in CDKL5 deficiency disorder: Delayed treatment and poor response to first‐line therapies
Epileptic spasms in CDKL5 deficiency disorder: Delayed treatment and poor response to first‐line therapies Open
Objective We aimed to assess the treatment response of infantile‐onset epileptic spasms (ES) in CDKL5 deficiency disorder (CDD) vs other etiologies. Methods We evaluated patients with ES from the CDKL5 Centers of Excellence and the Nationa…
Clinical characteristics of children with infantile epileptic spasms syndrome from a tertiary-care hospital in Dhaka, Bangladesh Open
This study highlights the long lead times to treatment for IESS in a low- and middle-income country, and the need for early referral of children with suspected epileptic spasms to epilepsy care centers.
Hospital readmissions in children with new‐onset infantile epileptic spasms syndrome Open
Objective To describe inpatient resource use in the 2 years following infantile epileptic spasms syndrome (IESS) diagnosis, examine the association between clinical/demographic variables and incidence of readmission, and identify risk fact…
Association of Time to Clinical Remission With Sustained Resolution in Children With New-Onset Infantile Spasms Open
Remission after first infantile spasms treatment can be identified by treatment day 7 in most children. Given the importance of early and effective treatment, these data suggest that children who do not respond to standard infantile spasms…
Interictal Connectivity Revealed by Granger Analysis of Stereoelectroencephalography: Association With Ictal Onset Zone, Resection, and Outcome Open
BACKGROUND: Stereoelectroencephalography (sEEG) facilitates electrical sampling and evaluation of complex deep-seated, dispersed, and multifocal locations. Granger causality (GC), previously used to study seizure networks using interictal …
View article: Inequities in Therapy for Infantile Spasms: A Call to Action
Inequities in Therapy for Infantile Spasms: A Call to Action Open
Objective The aim of this study was to determine whether selection of treatment for children with infantile spasms (IS) varies by race/ethnicity. Methods The prospective US National Infantile Spasms Consortium database includes children wi…
Confirmation of infantile spasms resolution by prolonged outpatient EEGs Open
Objective There is no consensus on the type or duration of the posttreatment EEG needed for assessing treatment response for infantile spasms (IS). We assessed whether outpatient electroencephalograms (EEGs) are sufficient to confirm infan…
View article: Comparative Effectiveness of Initial Treatment for Infantile Spasms in a Contemporary US Cohort
Comparative Effectiveness of Initial Treatment for Infantile Spasms in a Contemporary US Cohort Open
This study provides Class III evidence that for children with new-onset infantile spasms, ACTH or oral steroids were superior to nonstandard therapies.
Management of Infantile Spasms During the COVID-19 Pandemic Open
Circumstances of the COVID-19 pandemic have mandated a change to standard management of infantile spasms. On April 6, 2020, the Child Neurology Society issued an online statement of immediate recommendations to streamline diagnosis and tre…
Crisis Standard of Care: Management of Infantile Spasms during <span>COVID</span>‐19 Open
Crisis standards of care" are guidelines to help health care providers deliver the best possible medical care when resources are acutely limited, such as by war, natural disaster, or public health catastrophe. Public health officials and e…
Assessment of Physico-chemical Characteristics of Mangrove Region in the Krishnapatnam Coast, India Open
The present investigation was carried out to assess the physico-chemical characteristics of Mangrove region in the Krishnapatnam coastal region (Southeast coast of India) for a period of ten (10) months from June 2016 to March 2017. Surfac…
View article: White matter spongiosis with vigabatrin therapy for infantile spasms
White matter spongiosis with vigabatrin therapy for infantile spasms Open
Summary The histopathology, “white matter spongiosis,” defined by electron microscopy ( EM ) as “intramyelinic edema,” has been associated with vigabatrin therapy in various animal models, but its role or significance in clinical studies i…
Expanding the phenotypic spectrum of<i>GABRG2</i>variants: a recurrent<i>GABRG2</i>missense variant associated with a severe phenotype Open
Pathogenic missense and truncating variants in the GABRG2 gene cause a spectrum of epilepsies, from Dravet syndrome to milder simple febrile seizures. In most cases, pathogenic missense variants in the GABRG2 gene segregate with a febrile …
Role of Biofloc in the Growth and Survival of Blue morph, <i>Pseudotropheus saulosi<i> Open
Objective: To evaluate the effect of biofloc on the growth and survival of ornamental cichlid, Blue morph. Methods/ Statistical analysis: Thousand number of Blue morph were stocked in circular cement tanks of 14000 l capacity in control an…