Publications by authors named "Renu Suthar"

103 Publications

A 7-month-old boy with failure to thrive.

Brain Pathol 2021 Jul 14:e12998. Epub 2021 Jul 14.

Department of Pediatrics, Post Graduate Institute of Medical Education & Research, Chandigarh, India.

This autopsy case illustrates the neuropathological including ultrastructural findings in a genetically proven case of GM1 gangliosidosis.
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http://dx.doi.org/10.1111/bpa.12998DOI Listing
July 2021

Effectiveness and Safety of Nitrazepam in Children with Resistant West Syndrome.

Indian J Pediatr 2021 Jun 24. Epub 2021 Jun 24.

Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India.

Objectives: To study the effectiveness, tolerability, and safety of oral nitrazepam in children with resistant West syndrome (WS).

Methods: This prospective observational study was conducted at a tertiary care hospital in North India from January 2019 to October 2020. Children with WS resistant to standard therapy were enrolled within 7 d of initiation of nitrazepam and prospectively followed-up for cessation of spasms and adverse events.

Results: Forty-one children with resistant WS initiated on nitrazepam therapy were evaluated. The median age at onset of spasms was 6 mo (Q1, Q3: 4, 8). There was a preponderance of male gender (71%) and structural causes (78%). More than half of the enrolled children had failed four or more antiseizure medications (ASM) for epileptic spasms. The study participants had a long lead-time-to-treatment (LTTT) for the initial standard therapy (median: 2 mo; Q1, Q3: 1, 5) and nitrazepam (median: 11 mo; Q1, Q3: 8, 16). Nitrazepam was instituted as monotherapy in 7 (17%) children and as an adjunct in the rest. Twenty-one (51%) children achieved persistent cessation of epileptic spasms. However, the electroclinical response was observed in 17 (42%) children. Drowsiness, sialorrhea, and decreased appetite were the most commonly observed adverse events. Most adverse events were mild to moderate in severity and did not require dose reduction or change of medication. There was no significant difference between the responders and nonresponders in terms of LTTT, age at onset, or etiology.

Conclusions: Nitrazepam is a safe and feasible treatment alternative in children with resistant WS resulting in persistent cessation of spasms and electroclinical response in nearly half of patients.
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http://dx.doi.org/10.1007/s12098-021-03823-4DOI Listing
June 2021

Randomized trial of high-dose pyridoxine in combination with standard hormonal therapy in West syndrome.

Seizure 2021 May 29;91:75-80. Epub 2021 May 29.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Objective: To determine whether high-dose, oral pyridoxine in combination with standard adrenocorticotropic hormone (ACTH) therapy has superior effectiveness than ACTH therapy alone in increasing cessation of epileptic spasms for children with West syndrome.

Methods: This study was an open-label, randomized controlled trial with masked endpoint assessments. Eligible children with West syndrome, age ranged 3-18 months, were randomized into the intervention (n = 43) and the standard arm (n = 37) of therapy. The intervention group received oral pyridoxine at 100-300 mg/kg/day in addition to standard therapy of intramuscular ACTH at 150 IU/m/day. Primary effectiveness outcome was a complete cessation of spasms at two weeks and sustained till six weeks.

Results: Comparison of effectiveness measures between intervention and standard groups were : complete cessation of epileptic spasms (48.8% vs 58.3%; group difference -9.6%; 95% confidence interval [CI] -30% to 12.3%; p = 0.4), median EEG scores (Q1-Q3) by Jeavons Score at six weeks [3 (1-5) vs 3 (1-5); p = 0.6], median motor scores (Q1-Q3) by DASII (Development Assessment Scales for Indian Infants) at 12 weeks [35 (29-49) vs 42 (34.3-63.8), p = 0.04], and median mental scores (Q1-Q3) by DASII at 12 weeks [35 (29.5-46) vs 41.5 (31.3-60), p = 0.02]. Adverse events were comparable in both arms.

Conclusions: There was no evidence to suggest the superiority of high-dose pyridoxine in combination with ACTH versus ACTH alone for the treatment of West syndrome, considering the limitations of the study design.
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http://dx.doi.org/10.1016/j.seizure.2021.05.012DOI Listing
May 2021

Efficacy and Safety of Levetiracetam vs. Phenytoin as Second Line Antiseizure Medication for Pediatric Convulsive Status Epilepticus: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

J Trop Pediatr 2021 05;67(2)

Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh 160012, India.

Objective: To evaluate the efficacy and safety of levetiracetam (LEV) in comparison to phenytoin (PHT) as second line antiseizure medication (ASM) for Pediatric convulsive status epilepticus (SE).

Data Source: PubMed, Embase, Google scholar/Google, Scopus, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Cochrane Database of Systematic Reviews and Cochrane Central Register of Controlled Trials.

Study Selection: Randomized controlled trials (RCTs) assessing LEV and PHT as second line agent for convulsive SE in children <18 years published between 1 January 2000 and 30 November 2020.

Data Extraction: The data were pooled regarding the proportion of children achieving seizure cessation within 5-60 min of completion of study drug infusion (primary outcome); and seizure cessation within 5 min, time to achieve seizure cessation, seizure recurrence between 1 to 24 h, intubation and cardiovascular instability (secondary outcomes). Data were analyzed using RevMan version 5.4 and quality analysis was done using Cochrane risk-of-bias tool. The study protocol was registered with PROSPERO.

Data Synthesis: Twelve RCTs with 2293 children were included. Seizure cessation within 5-60 min was similar with both the drugs [82% in LEV vs. 77.5% in PHT, risk ratio (RR) = 1.04, 95% confidence interval (95% CI) 0.97-1.11, p = 0.30]. Seizure recurrences within 1-24 h was higher with PHT in comparison to LEV (16.6% vs. 9.7%, RR = 0.63, 95% CI 0.44-0.90, p = 0.01). Higher proportion of children in PHT group required intubation and mechanical ventilation (21.4% vs. 14.2%, RR = 0.54, 95% CI 0.30-0.98, p = 0.04). Seizure cessation within 5 min, time to achieve seizure cessation, and cardiovascular instability were similar with both the drugs. Three RCTs were at low risk of bias and nine were at high risk of bias.

Conclusion: The efficacy of LEV is similar to PHT as second line ASM for Pediatric convulsive SE. Seizure recurrences between 1 to24 h and requirement of intubation and mechanical ventilation were significantly higher with PHT in comparison to LEV.
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http://dx.doi.org/10.1093/tropej/fmab014DOI Listing
May 2021

Metabolic epilepsy in hyperprolinemia type II due to a novel nonsense ALDH4A1 gene variant.

Metab Brain Dis 2021 Aug 26;36(6):1413-1417. Epub 2021 May 26.

Pediatric Biochemistry Unit, Department of Pediatrics, PGIMER, Chandigarh, India.

Hyperprolinemia type II (HPII) is a rare autosomal recessive disorder of proline degradation pathway due to deficiency of delta-1-pyrroline-5-carboxylate dehydrogenase. Pathogenic variants in the ALDH4A1 gene are responsible for this disorder. We here describe an 11-month-old infant with recurrent seizures refractory to multiple antiepileptic drugs. She was hospitalized in view of acute-onset encephalopathy, exacerbation of generalized seizures following an upper respiratory infection. Laboratory investigation revealed significantly elevated proline levels in dried blood spots. DNA sample of the child was subjected to a targeted next-generation sequencing gene panel for hyperprolinemias. We detected a novel nonsense homozygous variant in the ALDH4A1 gene in the child and the heterozygous variant of the same in both the parents. Based on the location of the variant i.e. in the last exon, truncated protein is expected to be expressed by skipping nonsense-mediated decay and such point-nonsense variants could be an ideal target for readthrough drugs to correct genetic defects.
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http://dx.doi.org/10.1007/s11011-021-00757-wDOI Listing
August 2021

Clinical profile, aetiology, short-term outcome and predictors of poor outcome of neonatal seizures among out-born neonates admitted to a neonatal unit in Paediatric emergency of a tertiary care hospital in North India: A prospective observational study.

Trop Doct 2021 May 21:494755211016226. Epub 2021 May 21.

Assistant Professor, Division of Paediatric Critical Care, Department of Paediatrics, Advanced Paediatric Centre, Postgraduate Institute of Medical Education and Research (29751PGIMER), Chandigarh, India.

Neonatal seizures are common manifestations of several neurological or systemic disorders and associated with high morbidity, mortality and poor short- and long-term developmental outcomes. It is important to determine the aetiology and factors that determine the poor outcome, more so in a newly developed setting. The early detection of predictors of poor outcome will help in planning acute management, counselling, follow-up and rehabilitation services. In this prospective observational study, we looked at the clinical profile, aetiology, short-term outcomes and predictors of poor outcome of neonatal seizures among out-born neonates. The common causes were hypoxic ischaemic encephalopathy, sepsis and metabolic disturbances. One-third of neonates had poor outcome. Abnormal neurological and cardiorespiratory examination at admission; low oxygen saturation, glucose and pH; and hypoxic ischemic encephalopathy-III were predictors of poor outcome.
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http://dx.doi.org/10.1177/00494755211016226DOI Listing
May 2021

Long-term epilepsy control, motor function, cognition, sleep and quality of life in children with West syndrome.

Epilepsy Res 2021 Jul 2;173:106629. Epub 2021 Apr 2.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India. Electronic address:

Purpose: To assess epilepsy, motor function, cognitive, sleep, and quality of life outcomes and their predictors in a follow-up cohort with West syndrome (WS) at ≥5 years of age.

Methods: Cross-sectional evaluation in a follow-up cohort of WS (aged 5-14 years), between July 2018 and December 2019, was performed at a tertiary-care referral center in Northern India. 164 children were assessed in-person for epilepsy severity, functional status (gross motor and hand function), social quotient, behavioral comorbidities, sleep problems, and quality of life (QoL) using Early Childhood Epilepsy Severity Scale, Gross Motor Function Classification System, Manual Ability Classification System, Vineland Social Maturity Scale, Diagnostic and Statistical Manual of Mental disorders-5 criteria, Children's Sleep Habits Questionnaire, and PedsQL-Epilepsy module respectively. Furthermore, 238 children with the inability to visit the hospital were assessed through telephonic interview along with retrospective case record review for epilepsy control, gross and fine motor measures.

Results: 402 children with WS (75 % boys) with regular follow-up were studied and the majority (80 %) had underlying structural etiology. The median age (interquartile range) of the cohort was 92 (78-107) months. Of these, 60 % had evolved to Lennox-Gastaut syndrome (LGS). The following long-term outcomes were observed: ongoing epilepsy (261/402), unfavorable motor status (130/402), moderate to profound intellectual disability (111/164), autistic spectrum disorder (42/164), attention-deficit hyperactivity disorder (18/164), poor sleep (135/164), and impaired QoL (115/164). Non-structural etiology (odds ratio [OR] = 3.8, 95 % confidence interval [CI]: 2.1-5.5, p=<0·0001) and older age (>5 months) at the onset of epileptic spasms (OR = 2·9, 95 % CI: 1.5-5.0, p=<0·0001) were associated with enduring seizure freedom for more than two years.

Conclusion: The present study revealed a preponderance of structural etiology and a high rate of transition to LGS. Early age at onset of spasms (before five months) and structural etiology were the predictors of unfavorable long-term epilepsy outcome. QoL was impaired in more than two-thirds of patients and it correlated significantly with cognitive, sleep, motor, and behavioral outcomes. However, the results of our study should be interpreted in the context of significant attrition of the original cohort.
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http://dx.doi.org/10.1016/j.eplepsyres.2021.106629DOI Listing
July 2021

Bone density and bone health alteration in boys with Duchenne Muscular Dystrophy: a prospective observational study.

J Pediatr Endocrinol Metab 2021 May 12;34(5):573-581. Epub 2021 Apr 12.

Pediatric Neurology Unit, Department of Pediatrics,APC, PGIMER, Chandigarh, India.

Objectives: Boys with Duchenne Muscular Dystrophy (DMD) are at increased risk for compromised bone health, manifesting as low-impact trauma long bone fractures and vertebral compression fractures.

Methods: In a prospective observational study, we studied bone health parameters in North Indian boys with DMD. We consecutively enrolled ambulatory boys with DMD on glucocorticoid therapy. Bone health was evaluated with X-ray spine, Dual-energy X-ray absorptiometry (DXA), serum calcium, vitamin D3 (25[OH]D), 1,25-dihyroxyvitamin D3 (1,25[OH]D), serum osteocalcin, osteopontin, and N terminal telopeptide of type 1 collagen (Ntx) levels.

Results: A total of 76 boys with DMD were enrolled. The median age was 8.5 (interquartile range [IQR] 7.04-10.77) years. Among these, seven (9.2%) boys had long bone fractures, and four (5.3%) had vertebral compression fractures. Fifty-four (71%) boys underwent DXA scan, and among these 31 (57%) had low bone mineral density (BMD, ≤-2 z-score) at the lumbar spine. The mean BMD z-score at the lumbar spine was -2.3 (95% confidence interval [CI] = -1.8, -2.8), and at the femoral neck was -2.5 (95% CI = -2, -2.9). 25(OH)D levels were deficient in 68 (89.5%, n=76) boys, and 1,25(OH)D levels were deficient in all. Mean serum osteocalcin levels were 0.68 ± 0.38 ng/mL (n=54), serum osteopontin levels were 8.6 ± 4.6 pg/mL (n=54) and serum Ntx levels were 891 ± 476 nmol/L (n=54). Boys with low BMD received glucocorticoids for longer duration, in comparison to those with normal BMD (median, IQR [16.9 (6-34) months vs. 7.8 (4.8-13.4) months]; p=0.04).

Conclusions: Bone health is compromised in North Indian boys with DMD. BMD at the lumbar spine is reduced in more than half of boys with DMD and nearly all had vitamin D deficiency on regular vitamin D supplements. Longer duration of glucocorticoid therapy is a risk factor for low BMD in our cohort.
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http://dx.doi.org/10.1515/jpem-2020-0680DOI Listing
May 2021

Effect of early withdrawal of phenobarbitone on the recurrence of neonatal seizures: An open-label randomized controlled trial.

Epilepsy Behav 2021 04 8;117:107875. Epub 2021 Mar 8.

Division of Neonatology, Department of Pediatrics, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Background: The long-term administration of phenobarbitone in neonates may be associated with adverse neurological outcome. The timing of stopping phenobarbitone maintenance after acute seizure control in neonates is a matter of debate.

Objectives: To study the effect of early withdrawal of phenobarbitone on recurrence of neonatal seizures.

Study Design: Open-label randomized controlled trial.

Participants: Outborn neonates (≥34 weeks of gestation to <28 days of postnatal period) with seizures (n = 221) admitted to Neonatal unit in Pediatric emergency of a tertiary care hospital in north India over 1 year.

Intervention: After a loading dose of phenobarbitone (20 mg/kg), neonates who remained seizure free for at least 12 h were enrolled after written informed consent from parents, and randomized (computer generated block randomization) to 'phenobarbitone withdrawal group' (n = 112) where phenobarbitone maintenance was stopped and 'phenobarbitone continued group' (n = 109) where phenobarbitone maintenance was continued until discharge and further as per clinician's discretion.

Outcomes: The primary outcome was seizure recurrence until discharge and secondary outcomes were time to reach full enteral feeds, duration of hospital stay, abnormal neurological status at discharge, and mortality in two groups.

Results: The baseline variables were comparable in 2 groups. The incidence of seizure recurrence was similar in the phenobarbitone withdrawal and phenobarbitone continued groups (50% vs. 37.6%, respectively, p = 0.078). Among secondary outcomes, the phenobarbitone withdrawal and continued groups had similar time to reach full enteral feeds (4.02 days vs. 4.2 days, p = 0.75), duration of hospital stay (6.3 days vs. 6.5 days, p = 0.23), abnormal neurological status at discharge (45.6% vs. 38%, p = 0.39), and mortality (11.6% vs. 8.3%, p = 0.50).

Conclusion: Early withdrawal of phenobarbitone in neonatal seizures does not lead to a significant increase in the rate of seizure recurrence.
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http://dx.doi.org/10.1016/j.yebeh.2021.107875DOI Listing
April 2021

Unusual Cause of Encephalopathy and Seizures in a Child.

Neurol India 2021 Jan-Feb;69(1):167-169

Department of Radiodiagnosis and Pediatric Neurology, PGIMER, Chandigarh, Punjab, India.

Toxic encephalopathy is an important differential diagnosis in a child with encephalopathy and seizures. Subtle circumstantial evidence and apt neuroimaging features can significantly contribute to management, especially in a case of accidental exposure. 2, 4-D (ethyl ester) poisoning is a rare diagnosis, despite the common usage of this toxic compound as weedicide in northern India. The clinical similarity to the anticholinesterase poisoning, especially in the setting of agrochemical exposure is the main cause of under-diagnosis with usually fatal outcomes. We present an interesting case of accidental 2, 4-D (ethyl ester) poisoning in a child with typical neuroimaging features. A review of the literature regarding neuroimaging patterns of bilaterally symmetrical signal abnormalities involving basal ganglia in brain magnetic resonance imaging (MRI) from the point of view of clinical significance, is also discussed.
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http://dx.doi.org/10.4103/0028-3886.310065DOI Listing
June 2021

MR vessel wall imaging in tubercular meningitis.

Neuroradiology 2021 Feb 27. Epub 2021 Feb 27.

Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.

Purpose: Tubercular meningitis (TBM) has the propensity to cause secondary vasculitis through various mechanisms leading to development of cerebrovascular complications. These vascular involvements can be detected by vessel wall imaging (VWI). In this study, we aimed to study detailed findings of vessel wall imaging in cases of tubercular meningitis.

Methods: All consecutive patients of suspected tubercular meningitis in whom diagnosis of TBM could be made according to diagnostic criteria given by Ahuja et al. were included in the study. High-resolution MR VWI and time of flight (TOF) magnetic resonance angiography (MRA) were done along with routine MRI sequences. Arteries up to second-order branches were studied, and statistical analyses were done with respect to stage of tubercular meningitis, infarctions and TOF MRA findings.

Results: Out of all 101 cases of TBM, infarctions were found in 49 cases (48.5%), and vessel wall enhancement was seen in 67 cases (66.3%). With increasing severity of disease, more severe vascular involvement was seen on VWI. There was significant association between enhancement of individual arteries and infarctions in their territories. VWI had better sensitivity than the MRA, while MRA had better specificity than VWI for detection of vascular complications.

Conclusion: Tubercular vasculitis can be detected by VWI in the form of nodular or smooth segmental enhancement of vessel wall with or without stenosis. Incorporation of VWI in routine MR imaging can play a greater role in early detection and management of cerebrovascular complications which can help to improve prognosis of the disease.
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http://dx.doi.org/10.1007/s00234-021-02678-yDOI Listing
February 2021

Celiac Disease in a Boy with Duchenne Muscular Dystrophy: A Double Jeopardy!

Ann Indian Acad Neurol 2020 Sep-Oct;23(5):731-732. Epub 2020 Dec 8.

Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.4103/aian.AIAN_40_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887502PMC
December 2020

Pyridoxine Trial in Early Infantile Epileptic Encephalopathy: Never Forget!

Ann Indian Acad Neurol 2020 Sep-Oct;23(5):727-729. Epub 2020 Dec 8.

Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.4103/aian.AIAN_204_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887496PMC
December 2020

Brain MRI in Epstein-Barr Virus Meningoencephalitis in Children.

Ann Indian Acad Neurol 2020 Sep-Oct;23(5):621-624. Epub 2020 Feb 7.

Department of Pediatrics, Medanta, The Medicity, Gurugram, Haryana, India.

Background And Purpose: Epstein-Barr virus (EBV) meningoencephalitis can have variable and nonspecific brain magnetic resonance imaging (MRI) findings in children. This study was done with the purpose of describing brain MRI findings in children with EBV meningoencephalitis.

Materials And Methods: The study included 45 pediatric patients that presented with variable neurological symptoms and were found to have EBV meningoencephalitis based on positive EBV deoxyribonucleic acid (DNA) in the cerebrospinal fluid. All these patients had undergone brain MRI. Clinical and radiological features were evaluated.

Results: Fever was a presenting feature in all cases. Signs of meningitis and raised intracranial pressure (ICP) were seen in 24 (53.3%) cases, encephalopathy in 15 (33.3%), and seizures were present in 33 (73.3%). MRI was abnormal in 29 (64.4%) patients. The cortical/subcortical pattern was diagnosed in 9 (20%) cases, white matter involvement in 7 (15.5%), basal ganglia in 5 (11.1%), thalamic involvement in 4 (8.8%), brain stem involvement in 2 (6.2%), substantia nigra involvement in 2 (4.4%), and cerebellar involvement in 2 (4.4%). Diffusion restriction was present in 11 (24.4%) cases and susceptibility changes in 7 (15.5%). Meningeal enhancement was present in 10 (22.2%) cases. In addition, brain abscess and subdural effusion/empyema were present in 1 (2.2%) case each.

Conclusion: Pediatric EBV meningoencephalitis has varied clinicoradiological spectrum and there is no specific MRI pattern to characterize the meningoencephalitis on imaging. Common MRI findings include cortical-subcortical involvement, white matter changes, basal ganglia, and thalamic involvement.
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http://dx.doi.org/10.4103/aian.AIAN_537_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887499PMC
February 2020

MRI Spectrum of Meningoencephalitis in Children.

Ann Indian Acad Neurol 2020 Sep-Oct;23(5):616-620. Epub 2019 Dec 24.

Department of Pediatrics, Medanta, The Medicity, Gurugram, NCR, Haryana, India.

Background And Purpose: type b (Hib) infection occurs mostly in children and is transmitted from person to person through the respiratory pathway. Hib strain is associated with meningitis or encephalitis. It is not an uncommon infection, particularly, in the developing world. This prospective cohort study was done with the aim of describing imaging findings in patients with Hib meningoencephalitis.

Materials And Methods: In a prospective cohort study, consecutive children admitted in the pediatric emergency unit with acute febrile encephalopathy were enrolled. The clinical details, CSF analysis, and microbiological and serological investigations were recorded on a case record proforma. Children with confirmed Hib meningoencephalitis were included in this study. Clinicoradiological features were assessed.

Results: A total of 16 patients with acute febrile encephalopathy, in whom CSF latex agglutination, CSF culture, or CSF multiplex PCR were positive for were included in this study. All these children were investigated with magnetic resonance imaging (MRI) brain. Important imaging findings were meningitis, predominantly around frontoparietal lobes (43%), cerebritis (28%), ventriculitis (14%), and subdural collections (21.5%). One patient had features consistent with acute disseminated encephalomyelitis (ADEM) while four patients had normal MRI scan.

Conclusions: is still a common cause of meningitis in infants and children in the developing world. We have tried to study the most common MRI features associated with Hib infection to help radiologists alert the treating clinicians to further investigate these patients for appropriate prognostication.
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http://dx.doi.org/10.4103/aian.AIAN_500_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887487PMC
December 2019

Etiologic Profile of Childhood Stroke from North India: Is It Different from Developed World?

J Child Neurol 2021 Jul 23;36(8):655-663. Epub 2021 Feb 23.

Pediatric Neurology Unit, Department of Pediatrics, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Objective: To describe the etiology of childhood arterial-ischemic stroke from a developing country and assess short-term neurologic outcome.

Methods: Prospective observational study. Consecutive children between the age of >28 days to <12 years, admitted with the diagnosis of arterial-ischemic stroke were enrolled during the study period from January 2017 to December 2018. Short-term neurologic outcome was assessed with Pediatric Cerebral Performance Category (PCPC) scale and Pediatric Stroke Outcome Measure (PSOM).

Results: We enrolled 76 children with arterial-ischemic stroke, with a median age of 24 months (interquartile range 12-69), and 43 (57%) were boys. The most common risk factor for childhood arterial-ischemic stroke was arteriopathy in 59 (77%), followed by cardiovascular disorder in 12 (16%) children. Among 59 children with arteriopathy, 32 (42%) had infection-associated arteriopathies, 10 (13%) had mineralizing angiopathy, 10 (13%) had moyamoya disease. Pediatric stroke risk factors were classified according to Pediatric Stroke Classification and CASCADE primary classification. Short-term neurologic outcome was assessed at 3 months in 62 (82%) survivors. Among stroke survivors, 33 (61%) had sensory-motor deficits, and 24 (39%) had severe neurologic disability (PCPC ≥ 4). The presence of fever, encephalopathy, low Glasgow coma score at presentation, seizures, and infection-associated arteriopathy predicted severe neurologic disability at follow-up.

Conclusion: The risk factors for pediatric arterial-ischemic stroke are different from developed countries in our cohort. Infection-associated arteriopathies, mineralizing angiopathy, and moyamoya disease are the most common risk factors in our cohort. Two-thirds of pediatric stroke survivors have neurologic disability at short-term follow-up.
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http://dx.doi.org/10.1177/0883073821991291DOI Listing
July 2021

Neurodevelopmental Outcome in Neonates with Acinetobacter Sepsis with Meningitis and/or Ventriculitis at 2-5 years.

Indian J Pediatr 2021 04 7;88(4):400-401. Epub 2021 Jan 7.

Department of Microbiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1007/s12098-020-03573-9DOI Listing
April 2021

A prospective cohort study to assess the frequency and risk factors for calcification in single lesion parenchymal neurocysticercosis.

Seizure 2020 Dec 18;83:132-138. Epub 2020 Oct 18.

Director Pediatric Neurology and Neurodevelopment, Medanta, The Medicity, Gurgaon, Haryana, India.

Background: Calcified neurocysticercosis (NCC) predisposes patients to an enduring state of epilepsy. The predictors for calcification in parenchymal neurocysticercosis are not well defined.

Method: In this prospective cohort study, consecutive children with single-lesion parenchymal NCC were enrolled and followed up for one year. All patients were investigated with brain 3 T-MRI and electroimmunotransfer blot (EITB). Clinical follow-ups were performed every 3 months. Radiology was repeated at the 6-month and one-year follow-ups. The proportion of calcified lesions at one year and the predictors of calcification were studied.

Result: During the study period from June 2013 to December 2015, 93 children with single lesion parenchymal NCC were enrolled. At presentation, 90 % of the lesions were in the colloidal stage, and 71 % of the lesions had moderate to severe perilesional oedema. All children had 6 months of follow-up, and 86 (92.5 %) had one year of follow-up. Seizure recurrence was present in 13 (14 %) children. Follow-up radiology at one year showed lesion resolution in 51 (59 %) lesions and calcification in 28 (32.5 %) lesions. Children with calcified lesions during follow-up had a higher odds of seizure recurrences {OR, 95 % CI 3.6(2.3-5.6)}. The presence at baseline of diffusion restriction {OR, 95% CI 2.9 (1.01-8.8)}, scolex or wall calcification in the T2 Star weighted angiography MRI images {OR, 95% CI 3.7 (1.7-8.2)} and >10 mm size of the lesion {OR, 95 % CI 2.4 (1.2-5.01)} predicted lesion calcification.

Conclusion: Children with calcification of the parenchymal NCC lesions have a higher risk for seizure recurrence during follow-up. The presence of diffusion restriction, calcified nidus in the colloidal nodular stage, and >10 mm size of the lesion at baseline predicted calcification of the lesion during follow-up.
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http://dx.doi.org/10.1016/j.seizure.2020.10.012DOI Listing
December 2020

Primary Hypokalemic Periodic Paralysis: Long-term Management and Complications in a Child.

J Pediatr Neurosci 2020 Apr-Jun;15(2):132-134. Epub 2020 Jun 27.

Pediatric Neurology Unit, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Hypokalemic periodic paralysis (HPP) is a rare genetically determined neuromuscular disorder caused by mutation in skeletal muscles calcium and sodium channels. It presents with recurrent episodes of flaccid paralysis. A 9-year-old girl presented with recurrent episodic flaccid quadriparesis with complete recovery in-between the episodes. Investigations during the acute episode revealed marked hypokalemia with electrocardiogram changes. Next-generation sequencing showed pathogenic missense mutation in gene. She responded well to oral potassium supplementation, acetazolamide, and spironolactone therapy. Muscle weakness in HPP is reversible, and long-term management reduces frequency of paralysis and prevents permanent weakness.
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http://dx.doi.org/10.4103/jpn.JPN_101_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7519733PMC
June 2020

Randomized Controlled Trial Evaluating Levetiracetam as First-line Therapy for Seizures in Neonates: Pediatric Neurologist's Viewpoint.

Authors:
Renu Suthar

Indian Pediatr 2020 09;57(9):851-852

Pediatric Neurology Unit, Department of Pediatrics, PGIMER, Chandigarh, India.

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September 2020

Acinetobacter Sepsis Among Out-born Neonates Admitted to Neonatal Unit in Pediatric Emergency of a Tertiary Care Hospital in North India.

Indian J Pediatr 2021 02 7;88(2):127-133. Epub 2020 Aug 7.

Department of Microbiology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Objectives: To study the clinical profile, complications, antibiotic resistance pattern, treatment, and outcome of out-born neonates with Acinetobacter spp. sepsis admitted in Pediatric emergency of a tertiary care hospital in North India.

Methods: In this subgroup analysis of a prospective study (conducted over 1 y, February 2018 through January 2019), neonates with Acinetobacter spp. sepsis were included. The data collection included demographic details, clinical features, pre-referral treatment, complications, antibiotic resistance pattern, treatment, and final outcome.

Results: Acinetobacter spp. accounted for 10.6% (43/406) of all isolates and 22.7% (43/189) of Gram-negative isolates. The median (IQR) age at presentation was 1 (1-2) d, 2/3rd were male, and 46.5% were preterm. All were admitted in peripheral hospitals before referral to authors' centre and all received intravenous antibiotics and fluids. The resistance to different antibiotics was: Ciprofloxacin 82%, cephalosporins 78-100%, amikacin 75%, pipercillin-tazobactum 62%, carbapenems 50-85%, chloramphenicol 83%, and tetracycline 50-60%. All isolates were sensitive to colistin. The survival rate was 37.2% (n = 16) and 62.8% (n = 27) had poor outcome [death and Left against medical advice (LAMA)]. Higher proportion of neonates with Acinetobacter sepsis had septic shock, multi-organ dysfunctional syndrome (MODS), and disseminated intravascular coagulation (DIC); and higher proportion required mechanical ventilation, vasoactive drugs, and had poor outcome compared to those with sepsis due to other organisms.

Conclusions: Acinetobacter spp. accounts for high burden of sepsis among out-born neonates and is associated with alarmingly high resistance to cephalosporins, fluroquinolones, aminoglycosides, pipercillin-tazobactum, tetracyclines, and carbapenems. Neonates with Acinetobacter spp. sepsis had higher rates of complications, requirement of mechanical ventilation and vasoactive drugs, and poor survival.
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http://dx.doi.org/10.1007/s12098-020-03460-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7410966PMC
February 2021

Delayed Myelination Pattern and an Abnormal Thyroid Profile Caused by a Novel Mutation in the SLC16A2 Gene.

Indian J Pediatr 2020 09 6;87(9):764-765. Epub 2020 Aug 6.

Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.1007/s12098-020-03459-wDOI Listing
September 2020

Acute Necrotizing Encephalopathy of Childhood with H1N1 Infection.

J Pediatr Intensive Care 2020 Sep 6;9(3):222-224. Epub 2020 Mar 6.

Division of Pediatric Critical Care, Department of Pediatrics, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Acute necrotizing encephalopathy of childhood (ANEC) is an uncommon and fulminant complication of seasonal influenza infection associated with high mortality and poor neurological outcome. We report a 4.5-year-old female who had pneumonia, ANEC, and raised intracranial pressure (ICP) with polymerase chain reaction proven H1N1 infection. Management included mechanical ventilation, invasive monitoring and control of ICP, oseltamivir, methylprednisolone, and supportive care in pediatric intensive care unit. She survived with poor neurological status at discharge.
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http://dx.doi.org/10.1055/s-0040-1705182DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7360391PMC
September 2020

Enigma of Rabies: Prolonged Survival in a Boy with Rabies Brachial Plexitis and Encephalomyelitis.

Neurol India 2020 May-Jun;68(3):673-676

Department of Pediatrics, PGIMER, Chandigarh, India.

Rabies encephalitis is a universally fatal disease. Prolonged survival in children with rabies encephalitis has only been anecdotally reported. Case report: An 11-year-old boy presented with right-handed paraesthesia followed by flaccid weakness, progressive quadriparesis and encephalopathy following an unprovoked, class III dog bite over the right wrist 1 month previously. He received five doses of the rabies vaccine as post exposure prophylaxis. Diagnosis of rabies encephalitis was supported by typical MRI brain and spine findings in addition to marked elevation of anti-rabies neutralizing antibody titers in serum and CSF. He was treated with supportive care, methylprednisolone, dexamethasone and simvastatin and was discharged after 6 weeks of hospital stay in a minimally conscious state, with tracheostomy and naso-gastric feeding tubes. At 9 months follow-up, his neurological status showed minimal improvement. Paralytic rabies with brachial plexitis and encephalomyelitis is an atypical presentation of rabies. Very few surviving cases have been reported from India. Survival from rabies is possible with effective clearing of virus with post exposure prophylaxis, but with severe neurological sequelae.
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http://dx.doi.org/10.4103/0028-3886.288993DOI Listing
June 2021

Ataxia as Forme Fruste of Opsoclonus Myoclonus Ataxia Syndrome.

Ann Indian Acad Neurol 2020 May-Jun;23(3):415-417. Epub 2020 Jun 10.

Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.4103/aian.AIAN_111_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313590PMC
June 2020

CAPOS Syndrome: A Rare ATP1A3-Related Disorder.

Ann Indian Acad Neurol 2020 May-Jun;23(3):397-398. Epub 2020 Jun 10.

Department of Pediatrics, Pediatric Neurology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.4103/aian.AIAN_41_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313598PMC
June 2020

COVID-19 Pandemic: The Concerns of Pediatric Neurologists.

Ann Indian Acad Neurol 2020 May-Jun;23(3):358-359. Epub 2020 Jun 10.

Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatric Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

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http://dx.doi.org/10.4103/aian.AIAN_407_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313594PMC
June 2020

Acute Demyelinating Polyneuropathy in a Child with Dengue Shock Syndrome.

Indian J Pediatr 2021 02 16;88(2):165-166. Epub 2020 Jun 16.

Division of Pediatric Critical Care, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.

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http://dx.doi.org/10.1007/s12098-020-03396-8DOI Listing
February 2021

Calcified neurocysticercosis: The calcified dot is not so innocuous!

Seizure 2020 08 8;80:65-66. Epub 2020 Jun 8.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.seizure.2020.05.020DOI Listing
August 2020

Managing syndromic congenital ichthyosis at a tertiary care institute-Genotype-phenotype correlations, and novel treatments.

Dermatol Ther 2020 11 7;33(6):e13816. Epub 2020 Jul 7.

Department of Paediatric Genetics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Syndromic congenital ichthyoses (CI) are genetically determined disorders of cornification that are characterized by generalized scaling along with systemic symptoms. Data on congenital syndromic ichthyosis from developing countries are scarce. We aimed to assess the prevalence, phenotype-genotype correlation, and management of syndromic CI patients presenting to our outpatient during the specified period this was a retrospective study of congenital syndromic ichthyosis patients attending a dermatology clinic in a tertiary care center from 2105-2018. We reviewed epidemiological and comorbidities data, phenotype-genotype correlations, and treatments of syndromic congenital ichthyosis patients. Six patients of Syndromic CI were diagnosedamongst 86 patients of CI (8.1%). Amongst these, three patients of Sjogren-Larrson syndrome (SLS), two patients of Netherton syndrome (NS), and one of Chanarin-Dorfman disease (CDD) were reported. Next-generation sequencing (NGS) was performed with novel variants reported in one patient each of SLS, NS, and CDD. An atypical phenotype was observed in a patient with NS with associated growth hormone and adrenocorticotropic hormone deficiency but with favorable clinical response to intravenous immunoglobulin. Our reports point towards the unreported pool of genetic mutations in CI from India. Novel mutations were associated with variable cutaneous and systemic involvement.
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http://dx.doi.org/10.1111/dth.13816DOI Listing
November 2020
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