Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare but treatable, often paraneoplastic neuroimmunologic condition. This is a retrospective chart review of 8 patients diagnosed in the past 11 years at a tertiary care hospital. The mean age of children with OMAS was 21.2 ± 8 months. There was a female preponderance (62.5%). Median symptom duration was 24.5 days (interquartile range [IQR] 12.7; 97.5). All patients had ataxia and irritability; 6 had opsoclonus.An underlying neurogenic tumor was identified in 87.5% (⅞) of the patients by computed tomography (CT)/magnetic resonance imaging. Neuroblastoma was detected in ⅘ with normal 24-hour urinary vanillylmandelic acid and 2 had negative metaiodobenzylguanidine scan.All patients received adrenocorticotropic hormone/steroids for a median of 9.5 months (IQR 5.3; 13.5) with clonazepam. Five received intravenous immunoglobulin (IVIG), including repeated cycles in ⅘. Two received rituximab. One child with relapsing-remitting course received pulse dexamethasone and cyclophosphamide, resulting in improvement. Clonazepam restored ambulation in one with delayed diagnosis and failure of response to steroids. Six patients underwent tumor resection and four needed chemotherapies.Median follow-up was 15 months (IQR 10.7; 23.2). Mean OMAS-severity scale reduced from 10 to 1.4 in the IVIG group and 10.6 to 5.3 in those who did not receive IVIG. Cognitive delay and behavioral issues were seen in 100% treated with steroids only; 50 and 25%, respectively, treated with multimodal immunotherapy. Five had relapses, one with tumor recurrence.Thorax and abdomen CT scan was found to be a sensitive tool for tumor detection. Better motor and cognitive behavioral outcome were noted in patients who received adjunctive IVIG.Future studies on optimum investigation and treatment protocol in various resource settings are needed.

Objective Dravet syndrome (DS) is an epileptic syndrome that shares similarities with febrile seizures (FS), especially before 1 year of age, making it challenging to differentiate the two. We describe the profile of DS, with emphasis on the first year of life that can aid in early diagnosis. Methods The clinical, investigative, treatment, and outcome profiles of DS patients presenting to the outpatient department (OPD) between October 2016 and December 2021 in a single tertiary care center in South India were analyzed. Results Seventeen children were studied, with median age at presentation of 30 (interquartile range [IQR] 10, 47) months. The median age at seizure onset was 5 (IQR 3, 6) months. First seizure semiology were generalized tonic-clonic (GTCS) (35.3%), focal (52.9%), and myoclonic (11.8%). Fever preceding first seizure was seen in 76.5%. Status epilepticus (SE; ≥30 minutes) and prolonged seizures (>10 minutes) were seen in 41.2% each, and >5 seizures were seen in 82.4% in the first year of life. The most frequent subsequent seizure types were focal seizures (76.5%) and GTCS (76.5%). Other seizure triggers included vaccination (52.9%), light (17.6%), and Hot-bath (5.8%). Delayed developmental milestones for age were found in 52.9% at diagnosis. Magnetic resonance imaging (MRI) brain and electroencephalogram were normal in 76.4% each. Pathogenic/likely pathogenic variants in SCN1A gene were seen in 64.7%. Average of 3.9 anti-seizure medications were used. After optimization of treatment seizure frequency reduced in 40% and 4/15 (26.6%) had SE. Conclusion In addition to characteristic clinical profile of DS we observed atypical presentations: an earlier age of seizure onset and afebrile seizure at onset. Delayed diagnosis was noted. Seizure control improved and SE reduced on optimal treatment.

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Background: Vaccination is an integral aspect of a pediatrician's practice. The fear associated withpain is a common problem. Alleviating this pain, not only puts the child at ease but also reduces theapprehension some parents have. Our objective was to evaluate local analgesic use during childhoodimmunization, its efficacy, and assessing how it serves as an advantage to the patient, theparents/guardian, and the medical personnel. Methodology: It was a randomized study. Data wascollected for a period of 1 year, from August 2018 to August 2019. Children from birth to 18 yearswere divided into 2 groups: case (local anesthetic i.e Lidocaine aerosol applied) and control (no localanesthetics applied). They were immunized as per NIS/IAP. The pain was assessed by a standardpain chart (Modified Behavioural Pain Scale (BPS). Result: Totally (including IM, SC, and IDinjections), the Local anesthesia group Median pain score was 6 and the No Local anesthesia groupMedian pain score was 8. There was a significant difference in pain scores between the two groups.Conclusion: The present study showed that local anesthetics could be applied quickly and withease. There was a significant difference in pain scores between the two groups (higher score beingin the group in which local anesthetics weren’t used). The reduction in the pain score, in turn,showed a significant difference in the attitude of the child, parent, as well as medical personnel.