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Background : Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim : We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods : In this prospective observational study, eligible infants and children < 21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental "delay" and "no delay" groups. Results: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental "delay" and "no delay" groups (P = 0.04). Conclusion : Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Corticosteroids prolong ambulation and improve muscle power among boys with Duchenne muscular dystrophy (DMD). However, the optimal steroid regimen remains unclear. Hence, this study was undertaken to compare the efficacy of daily‐ versus intermittent‐steroid regimens in ambulatory boys with DMD. Methods: In this single‐center, open‐label randomized trial, 72 children were randomized to receive either daily prednisolone (0.75 mg/kg/day) or intermittent prednisolone (0.75 mg/kg/day, for first 10 days of every month). The primary outcome measure was the difference in average score on manual muscle testing (MMT) at baseline and after 6 mo of steroids. A difference of >0.2 was hypothesized to be significant. Secondary outcomes included changes in timed functions, muscular dystrophy‐specific functional‐rating scale score, peak torque, average power, and pulmonary function. Results: In the intention‐to‐treat analysis, the mean (SD) change in MMT scores was 0.17 (0.15) and 0.08 (0.10) for the daily and intermittent steroid groups, respectively. The mean difference between the two interventions was 0.10 (95% confidence interval [CI] = 0.04–0.16; P =.003), which although significant was less than the predefined value of 0.2. Statistically significantly improvements were observed with daily‐steroid regimen in the Gowers time (P =.01), nine‐metre walk test (P =.02) and average power (P =.02) as compared to intermittent‐steroid regimen. A total of 19/32 (52.8%) children in the daily‐steroid group and 8/29 (27%) children in the intermittent‐steroid group experienced some form of adverse effect (P =.02). Discussion: Over a short‐term period, the intermittent‐steroid regimen was non‐inferior to the daily‐steroid regime in preserving muscle strength among children with DMD. However, better improvement of functional measures was observed with daily‐steroid administration. The frequency of individual side effects was similar between the two groups. [ABSTRACT FROM AUTHOR]

Byline: Jagdish. Meena, Rachna. Seth, Biswaroop. Chakrabarty, Sheffali. Gulati, Sandeep. Agrawala, Priyanka. Naranje The opsoclonus-myoclonus ataxia syndrome (OMAS) also called “Kinsbourne syndrome” or “dancing eye syndrome” is a rare but [...]

Byline: Lokesh. Saini, Biswaroop. Chakrabarty, Atin. Kumar, Sheffali. Gulati Sir, A 5-month-old boy presented with global developmental delay, generalized looseness of the body, and jerky, chaotic eye movements. There was [...]

Byline: Anil. Israni, Rachana. Dubey, Biswaroop. Chakrabarty, Atin. Kumar, Sheffali. Gulati Introduction Epidermal nevus is a congenital hamartoma of embryonal ectodermal origin. It can be classified on the basis of [...]

Byline: Sheffali. Gulati, Puneet. Jain, Biswaroop. Chakrabarty, Atin. Kumar, Neerja. Gupta, Madhulika. Kabra Objective: The objective of this study is to retrospectively collect and then describe the clinico-radiographical profile of [...]

Byline: Anil. Israni, Biswaroop. Chakrabarty, Atin. Kumar, Sheffali. Gulati Oculomotor nerve palsy can be due to varied causes that include diabetic neuropathy, myasthenia gravis, brainstem infarction, demyelinating conditions, and cerebral [...]

Byline: Sheffali. Gulati, Jaya. Kaushik An epileptic seizure is defined as transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in brain. There are diverse [...]

Byline: Lokesh. Saini, Ranjith. Kumar, Biswaroop. Chakrabarty, Sheffali. Gulati Headache is infrequent in early childhood. Headache and neurological deficits associated with cerebrospinal fluid (CSF) lymphocytosis (HaNDL), a variant of migraine, [...]

Byline: Lokesh. Saini, Biswaroop. Chakrabarty, Sheffali. Gulati, Atin. Kumar Dear Sir, A premorbidly normal, 13-year-old boy, presented with complaints of headache for last 1 month. The headache was intermittent, moderate [...]

Byline: Sheffali. Gulati, Biswaroop. Chakrabarty, Atin. Kumar, Puneet. Jain, Harsh. Patel, Lokesh. Saini Objective : This is a retrospective chart review of consecutive children with acquired demyelinating disorders presenting to [...]

Byline: Aruna. Nambirajan, Vaishali. Suri, Mehar. Sharma, Ranjith. Kumar, Ajay. Garg, Sheffali. Gulati, Vivek. Tandon Clinical Summary A 7-year-old, previously well, female child presented with sudden onset recurrent episodes of [...]

Byline: Anil. Israni, Biswaroop. Chakrabarty, Sheffali. Gulati, Atin. Kumar Clinical Description A 10 year old, premorbidly normal girl presented with history of weakness of right half of the body, right [...]

Byline: Puneet. Jain, Shivaram. Shastri, Sheffali. Gulati, Thomas. Kaleekal, Madhulika. Kabra, Neerja. Gupta, Y. Gupta, Ravindra. Pandey Background: Valproate is a commonly used anticonvulsant drug. Uridine 5?-diphospho (UDP)-glucuronosyltransferase (UGT) contributes [...]

Byline: Lakshminarayanan. Kannan, Puneet. Jain, Suvasini. Sharma, Sheffali. Gulati Sir, Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disorder caused by a persistent infection of the brain by an aberrant [...]