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1.
Epilepsy Res ; 173: 106622, 2021 Mar 23.
Artículo en Inglés | MEDLINE | ID: mdl-33813361

RESUMEN

PURPOSE: Handedness has been strongly linked to cerebral lateralisation for language. Literature on atypical handedness and its association with focal epilepsy starting from early childhood is limited and needs exploration. METHODS: In this cross-sectional study, the prevalence of atypical handedness (left-handedness and ambidexterity) was compared between children with left/right focal epilepsy aged 3-14 years without any definite motor deficit, between August 2019 and July 2020. Cognition, behavioral abnormalities, working memory, and executive function were also compared between the groups. Various clinical, demographic, and radiological variables were explored to determine any association with the presence of atypical handedness. RESULTS: Of the total 79 children (45 boys, 57 % mean age 6.5 ± 2.3 years, 41-left focal epilepsy) with focal epilepsy enrolled for the study, the prevalence of atypical handedness was found to be 12.6 % (10/79). No association between atypical handedness and clinical/demographic variables including left/right focal epilepsy was observed in the study excepting neuroimaging abnormality (p = 0.0008 and 0.02 respectively). This increased prevalence of neuroimaging abnormality in patients with atypical handedness was significant in multivariate analysis, after adjusting for confounding variables. Similarly, intelligence quotient (full scale, verbal and non-verbal, as well as sub-domains), behavioral abnormality (internalizing and externalizing score on CBCL), scores on picture memory, simple copying, and missing scan task were not significantly different between children with left and right focal epilepsy or children with atypical handedness and right-handedness. CONCLUSION: Atypical handedness is not more frequent in children with left focal epilepsy as compared to right focal epilepsy.

3.
Seizure ; 85: 119-126, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33461030

RESUMEN

BACKGROUND: Dravet syndrome (DS) is an infantile-onset developmental and epileptic encephalopathy syndrome with limited treatment options. We aimed to evaluate the efficacy and tolerability of fenfluramine in patients with Dravet syndrome using meta-analytical techniques. METHODS: We searched for relevant randomized controlled trials and non-randomized studies involving children with Dravet syndrome on fenfluramine therapy in MEDLINE, CENTRAL, EMBASE, Google Scholar and Web of Science database (31 July 2020). The primary outcome for the efficacy of fenfluramine was reduction in monthly convulsive seizure frequency. We carried out a random effect meta-analysis focusing on efficacy and safety variables. Only Randomized Controlled Trials (RCT) were included in the meta-analysis. The risk of bias was assessed for each study, and GRADE was used to assess the quality of evidence for each outcome. RESULTS: Of 61 publications initially screened, 12 were reviewed as full-text. Seven articles including 2 RCTs, 4 uncontrolled studies (3 prospective and one retrospective study), and one case report described responses to fenfluramine in 144 DS patients (54 % male, mean age of 8.8 years, median dose of 0.4 mg/kg/day). Fenfluramine was found to be more efficacious than placebo, in terms of mean convulsive and total seizure frequency reduction (mean difference: -45.3 % (95 % CI: -48.1 %, -42.4 %, p < 0.00001) and -39.7 % (-46.7 %, -32.7 %, p < 0.00001)). A greater proportion of patients in the fenfluramine arm achieved >25 %, >50 %, >75 % and 100 % seizure reductions (odds ratios: 6.5 (3.7, 11.5, p < 0.00001), 10.6 (5.3, 21.3, p < 0.00001), 22.7(6.9, 75.3, p < 0.00001) and 9.3(1.7, 51.4, p = 0.01) respectively). The incidence of serious adverse events was not greater in the fenfluramine groups (OR: 1.02 (0.5, 2.19, p = 0.96)). CONCLUSION: Fenfluramine appears to be a safe and efficacious antiseizure medication in patients with Dravet syndrome.

4.
Epilepsy Behav ; : 107620, 2020 Nov 30.
Artículo en Inglés | MEDLINE | ID: mdl-33268014

RESUMEN

PURPOSE: Spectral resolution is imperative for complex listening tasks such as understanding speech in the presence of background noise and has a significant role in children, particularly classroom learning. The present study evaluated the auditory spectral resolution abilities of children with Benign epilepsy with centrotemporal spikes (BECTS). METHOD: This cross-sectional study conducted from August 2017 to March 2020 recruited 23 children with clinical and electrographic features consistent with BECTS as cases. Fifteen age and sex matched typically developing children (TDC) were taken as controls. Spectral resolution abilities were evaluated using the recently developed Spectral temporally modulated Ripple test (SMRT). RESULTS: The mean age of the cases was 10.63 ±â€¯1.91 years with a slight male preponderance (69%). The mean (±SD) SMRT thresholds in the cases and controls were 5.90 (±1.91) and 7.21 (±1.03) respectively. The auditory spectral resolution threshold measured by SMRT in children with BECTS was observed to be significantly lower when compared to the controls (p of 0.021). CONCLUSION: Children with BECTS have a lower spectral resolution threshold by SMRT.

5.
Am J Trop Med Hyg ; 2020 Nov 09.
Artículo en Inglés | MEDLINE | ID: mdl-33219642

RESUMEN

Scrub typhus, a vector-borne rickettsiosis, is the leading treatable cause of non-malarial febrile illness in Asia. The myriad of typical and atypical features poses a clinical conundrum. We aimed to study the clinical and laboratory profile of children with scrub typhus infection diagnosed by IgM ELISA. Data of children < 12 years presenting with undifferentiated fever to the pediatric services of a tertiary teaching institute between January 2012 and December 2018 were retrieved. Children with seropositive IgM ELISA (InBios International Kit) for scrub typhus were enrolled in the study. Clinical features, laboratory investigations, treatment received, and the outcome recorded were obtained. Objective evidence of organ dysfunction was taken as severe scrub typhus. In total, 262 children were diagnosed with scrub typhus. The mean age was 5 years, with male preponderance (65%). And, 13 children presented during infancy. Fever was universal, and generalized lymphadenopathy (93.5%) and hepatomegaly (70%) were the common clinical signs. Eschar was identified in 31%, with greater predilection for groin and axilla. Thrombocytopenia was striking in one-third of children. Also, 25 children (9.5%) had severe scrub typhus and 18 required intensive care stay. Elevated aspartate aminotransferase enzyme levels was a predictor of severity ([OR 3.9], P value 0.005) by multivariate analysis. Lymphadenopathy was found significantly associated with eschar (P < 0.005). No mortality was recorded. This 6-year study underscores the varied spectrum of pediatric scrub typhus infection. Zero mortality in our cohort signifies the excellent outcome with judicious first-line antibiotics.

6.
J Neurosci Rural Pract ; 11(4): 597-600, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-33144797

RESUMEN

Objective To estimate the serum zinc levels in children under the age of 5 years with febrile seizures and febrile children without seizures Materials and Methods In this cross-sectional study from 2017 to 2018, 40 children with febrile seizures (simple and complex) were taken as cases. Forty age- and sex-matched febrile children without convulsions were recruited as controls. Serum zinc estimates were analyzed using a spectrophotometer (Biolis 50i-Autoanalyzer). Statistical Analysis The demographic variables and serum zinc estimates were analyzed using the Mann-Whitney test. The odds ratio was used to calculate the association of zinc deficiency in febrile seizures; 5% level of significance was considered. Results The mean serum concentrations of zinc in the cases and controls were 83.8 ± 33.1 µg/dL and 116.3 ± 30.3 µg/dL, respectively ( p = 0.002). Hypozincemia defined by "a serum zinc level of less than 63 µg/dL" was found in 12 (30%) cases and 2(5%) controls with an odds ratio of 8:1. Conclusion Children with febrile seizures had significantly reduced concentrations of zinc in the serum.

7.
J Pediatr Neurosci ; 15(2): 94-98, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33042238

RESUMEN

Aims and Objectives: Pontocerebellar hypoplasia (PCH) constitutes a heterogeneous group of neurodegenerative/neurodevelopmental disorder of pons and cerebellum with onset in prenatal period. This study aimed to discuss the clinical, radiological profile, and outcome of four infants with PCH attending our center. Materials and Methods: Data of children with psychomotor retardation seen between January 2015 and December 2015 at neurodevelopmental clinic was retrieved. PCH was defined by clinical and radiological criteria. Clinical features included were delay in attainment of milestones in more than two developmental domains accompanied by severe microcephaly. Radiological evidence of cerebellar volume loss with hypoplasia of pons was included. Patient charts were reviewed for clinical features, neuroimaging, electroencephalography, and biochemical investigations including serum and cerebrospinal lactate. Molecular genetic testing for the common p.A307S mutation in TSEN54 of the cases and their parents were also analyzed. Results: During this period, 101 children with psychomotor retardation were evaluated at our center. Of the 101, four children were with clinical and radiological evidence of PCH. In addition to psychomotor retardation and severe microcephaly, spasticity, bipyramidal signs, and epileptic spasms were universal in all four children. Three of the four children had optic atrophy and two had sensorineural hearing loss. Severe cerebellar hypoplasia with attenuated pons was seen in all four children. Two children had dragonfly appearance of cerebellum on coronal section. The commonest TSEN54 p.A307S mutation in children and their parents was not detected. Conclusion: A heightened index of suspicion for PCH is merited in infants with progressive psychomotor retardation and severe microcephaly. Cerebellar hypoplasia with pontine attenuation forms the mainstay of diagnosis of PCH.

8.
Neurocrit Care ; 2020 Oct 07.
Artículo en Inglés | MEDLINE | ID: mdl-33025545

RESUMEN

BACKGROUND: To study the incidence and time of onset of intensive care unit-acquired weakness in a prospective cohort of children (2-12 years) by serial simplified electrophysiological assessment (Pediatric Critical Illness Myopathy Polyneuropathy study, PEDCIMP). METHODS: A single-center, prospective cohort study (Trial Registry Number: NCT02763709; PEDCIMP2016) was conducted at the pediatric intensive care unit of a tertiary care hospital in North India. A complete electrophysiological evaluation (4 motor nerves and 2 sensory nerves) was performed at baseline in children (2-12 years) admitted to the ICU with a pediatric risk of mortality (PRISM) of > 20 with more than 24-h stay. Following the entry evaluation, a minimal alternate day simplified electrophysiological testing of the unilateral common peroneal nerve and the sural nerve was assessed. A 25% reduction in compound muscle action potential (CMAP) and sensory nerve action potential from baseline was considered significant for ICUAW and was confirmed by complete electrophysiological re-evaluation. RESULTS: Of the total 481 children assessed for eligibility, 97 were enrolled. The median age of the cohort was 7 years. Sepsis (81%); need for vasoactive support (43%); multiorgan dysfunction (26%) were the common reasons for admission. Of the 433 eligible patient ICU days, 380 electrophysiological observations were done. A significant decrease of > 25% in CMAP of common peroneal nerve was not detected in any of the 380 observations. However, two children unfit for inclusion were diagnosed with ICUAW during the study period. CONCLUSIONS: Children admitted with PRISM > 20 have a very low incidence of intensive care unit-acquired weakness by serial clinical and abbreviated electrophysiological evaluation.

11.
Pediatr Crit Care Med ; 21(11): 959-965, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32852360

RESUMEN

OBJECTIVES: To compare the diagnostic accuracy of the ultrasonography-guided optic nerve sheath diameter with transcranial Doppler-guided middle cerebral artery flow indices against the gold standard invasive intraparenchymal intracranial pressure values in children. DESIGN: A single-center prospective cohort study. SETTING: PICU of a tertiary care teaching hospital in North India. PATIENTS: Eligible children (2-12 yr) are admitted to ICU and are undergoing intracranial pressure monitoring using an intraparenchymal catheter. Observations with a parallel measured intracranial pressure greater than or equal to 20 mm Hg were included as case-observations. Children with an invasive intracranial pressure of less than or equal to 15 mm Hg were taken as neurologic-control-observations and healthy children served as healthy-control-observations. INTERVENTIONS: The horizontal and vertical diameters of the optic nerves were measured, and averages were calculated and compared. Middle cerebral artery flow indices (pulsatility index and resistive index) were measured bilaterally and averages were calculated and compared in the three groups. Twenty-two measurements of optic nerve sheath diameter were assessed by two different observers in quick succession for interrater reliability. MEASUREMENTS AND MAIN RESULTS: A total of 148 observations were performed in 30 children. Four observations were excluded (intracranial pressure between 16 and 19 mm Hg). Of the 144 observations, 106 were case-observations and 38 were neurologic-control-observations. Additional 66 observations were healthy-control-observations. The mean optic nerve sheath diameter was 5.71 ± 0.57 mm in the case-observations group, 4.21 ± 0.66 mm in the neurologic-control-observations group, and 3.71 ± 0.27 mm in the healthy-control-observations group (p < 0.001 for case-observations vs neurologic-control-observations/healthy-control-observations). The mean pulsatility index in case-observations was 0.92 ± 0.41 compared with controls 0.79 ± 0.22 (p = 0.005) and the mean resistive index was 0.56 ± 0.13 in case-observations compared with 0.51 ± 0.09 (p = 0.007) in controls (neurologic-control-observations and healthy-control-observations). For the raised intracranial pressure defined by intracranial pressure greater than or equal to 20 mm Hg, the area under the curve for optic nerve sheath diameter was 0.976, while it was 0.571 for pulsatility index and 0.579 for resistive index. Furthermore, the optic nerve sheath diameter cutoff of 4.0 mm had 98% sensitivity and 75% specificity for raised intracranial pressure, while the pulsatility index value of 0.51 had 89% sensitivity and 10% specificity by middle cerebral artery flow studies. The sensitivity and specificity of 0.40 resistive index value in the raised intracranial pressure were 88% and 11%, respectively. Kendall correlation coefficient between intracranial pressure and optic nerve sheath diameter, pulsatility index, and resistive index was 0.461, 0.148, and 0.148, respectively. The Pearson correlation coefficient between two observers for optic nerve sheath diameter, pulsatility index, and resistive index was 0.98, 0.914, and 0.833, respectively. CONCLUSIONS: Unlike transcranial Doppler-guided middle cerebral artery flow indices, ultrasonography-guided optic nerve sheath diameter was observed to have a good diagnostic accuracy in identifying children with an intracranial pressure of greater than or equal to 20 mm Hg.

14.
Indian J Tuberc ; 67(1): 136-138, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32192608

RESUMEN

Tuberculosis (TB) is a disease of diverse manifestations. In children, neurotuberculosis is the severest form, which when left untreated can have deleterious consequences. There has been reports on pediatric TB meningitis manifesting with fever and seizures, altered sensorium or focal deficits. There are reports on TB meningitis presenting with cognitive decline in adults. We are reporting a eleven month old girl child who presented with acute regression of attained developmental milestones of one month duration as the only presenting complaint and MRI brain revealed basal exudates with hydrocephalus which nailed the diagnosis of tuberculous meningitis. CSF (Cerebro Spinal Fluid) tested by CBNAAT (Cartridge Based Nucleic Acid Amplification Testing) for TB was negative, but gastric aspirate tested for the same, came positive. Tuberculin skin testing was also positive. Chest X-ray was normal. The child had not received BCG (Bacillus Calmette Guerin)vaccine, thereby increasing her risk of complicated TB. The contact couldn't be traced. The child was started on ATT (Anti Tubercular Treatment) as soon as the diagnosis was made and she improved, thus signifying the better outcome with early initiation of ATT. This case reporting is intended to highlight the unusual presentation of TB meningitis in children, which when clinicians are aware of will lead to early treatment and better prognosis.

16.
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