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1.
Artículo en Inglés | MEDLINE | ID: mdl-33825947

RESUMEN

The lack of consensual measures to monitor core change in Autism Spectrum Disorder (ASD) or response to interventions leads to difficulty to prove intervention efficacy on ASD core symptoms. There are no universally accepted outcome measures developed for measuring changes in core symptoms. However, the CARS (Childhood Autism Rating Scale) is one of the outcomes recommended in the EMA Guideline on the clinical development of medicinal products for the treatment of ASD. Unfortunately, there is currently no consensus on the response definition for CARS among individuals with ASD. The aim of this elicitation process was to determine an appropriate definition of a response on the CARS2 scale for interventions in patients with Autism Spectrum Disorder (ASD). An elicitation process was conducted following the Sheffield Elicitation Framework (SHELF). Five experts in the field of ASD and two experts in expert knowledge elicitation participated in an 1-day elicitation workshop. Experts in ASD were previously trained in the SHELF elicitation process and received a dossier of scientific evidence concerning the topic. The response definition was set as the mean clinically relevant improvement averaged over all patients, levels of functioning, age groups ***and clinicians. Based on the scientific evidence and expert judgment, a normal probability distribution was agreed to represent the state of knowledge of this response with expected value 4.03 and standard deviation 0.664. Considering the remaining uncertainty of the estimation and the available literature, a CARS-2 improvement of 4.5 points has been defined as a threshold to conclude to a response after an intervention. A CARS-2 improvement of 4.5 points could be used to evaluate interventions' meaningfulness in indivudals. This initial finding represents an important new benchmark and may aid decision makers in evaluating the efficacy of interventions in ASD.

2.
Artículo en Inglés | MEDLINE | ID: mdl-33769388

RESUMEN

Children with neuroblastoma (NB) and opsoclonus-myoclonus-ataxia syndrome (OMAS) have a favorable oncologic outcome and overall survival. In contrast, despite intensive multidrug immunomodulation, the neurologic outcome is complicated by the relapsing nature of the neurologic symptoms and long-term neurobehavioral sequelae. Being associated with low-risk NB, there exists an ambiguity in the current literature regarding the administration of chemotherapy in these children. We reviewed our archives for children with NB-OMAS over a 22-year (January 1996 to January 2018) period. Eighteen children (10 female) with a median age at diagnosis of 23 months had NB-OMAS and were included. They had stage 1 (9/18; 50%), 2 (1/18; 5.5%), 3 (7/18; 39%), and 4 (1/18; 5.5%) disease according to the International Neuroblastoma Staging System. Multimodality therapy included surgery (16/18; 89%), chemotherapy (11/18; 61%), and immunomodulatory therapy (10/18; 55%). Complete oncologic remission was achieved in all children. Relapse of OMAS and presence of neurologic sequelae were observed in 1 (5.5%) and 5 (28%) cases, respectively. Presence of neurologic sequelae was significantly associated with low-tumor stage (P=0.036) and treatment without chemotherapy (P=0.003). Chemotherapy administration was the only variable significantly predicting a favorable neurologic outcome (95% confidence interval: 0.26-1.40, P=0.01). To conclude, our study including a limited cohort of patients highlights a favorable neurologic outcome associated with chemotherapy administration in children with NB-OMAS. However, further studies with larger sample size need to be conducted before drawing any definite conclusions.

3.
Indian J Pediatr ; 2021 Mar 19.
Artículo en Inglés | MEDLINE | ID: mdl-33740232

RESUMEN

Epilepsy is a common neurological condition in children. It is usually amenable to drug therapy. However, nearly one-third of patients may be refractory to antiseizure drugs. Poor compliance and nonepileptic events should be ruled out as possible causes of drug-resistant epilepsy (DRE). After failing adequate trials of two appropriate antiseizure drugs, patients with focal DRE or poorly classifiable epilepsy or epileptic encephalopathy with focal electro-clinical features should be worked up for surgical candidacy. A randomized controlled trial provided a class I evidence for epilepsy surgery in pediatric DRE. Pre-surgical screening workup typically includes a high-resolution epilepsy protocol brain magnetic resonance imaging (MRI) and a high-quality in-patient video electroencephalography evaluation. Advanced investigations such as positron emission tomography (PET), single-photon emission computed tomography (SPECT), and magnetoencephalography (MEG) may be required in selected cases especially when brain MRI is normal, and further evidence for anatomo-electro-clinical concordance is necessary to refine candidacy for surgery and surgical strategy. Some children may also need functional MRI to map eloquent regions of interest such as motor, sensory, and language functions to avoid unacceptable neurological deficits after surgery. Selected children may need invasive long-term electroencephalographic monitoring using stereotactically implanted intracranial depth electrodes or subdural grids. Surgical options include resective surgeries (lesionectomy, lobectomy, multilobar resections) and disconnective surgeries (corpus callosotomy, etc.) with the potential to obtain seizure freedom. Other surgical procedures, typically considered to be palliative are neuromodulation [deep brain stimulation (DBS), vagal nerve stimulation (VNS), and responsive neural stimulation (RNS)]. DBS and RNS are currently not approved in children. Pediatric DRE should be evaluated early considering the risk of epileptic encephalopathy and negative impact on cognition.

4.
Epilepsy Res ; 171: 106574, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33582533

RESUMEN

BACKGROUND: A classic ketogenic diet, even though effective in children with drug-resistant epilepsy is not tolerated well by them and cumbersome to prepare. Low glycemic index therapy (LGIT), the least restrictive with minimal adverse effects among ketogenic dietary therapies has been proven effective in uncontrolled trials, but a placebo-controlled trial in this regard is still lacking. METHODS: In this open-label randomized controlled study, we randomized children above age two years with drug-resistant epilepsy into two groups (LGIT and control groups). Patients in the LGIT group received an add-on low glycemic index diet for 3 months along with the ongoing antiepileptic drugs and the patients in the control group did not receive any dietary intervention. Seizure frequency was assessed from the seizure diary maintained by the parents. Diet compliance was assessed using the diet diary that was maintained by the parents for three days just before the scheduled monthly visits of the patients. RESULTS: Forty children with drug-refractory epilepsy (20 in each group) were enrolled. While 6/20 children in the LGIT arm have >50 % reduction in seizure frequency, none achieved this in the control arm (p = 0.02). The overall compliance with the low glycemic diet in the intervention group was 88.5 %. Out of six responders to LGIT, one child achieved seizure freedom and one achieved >90 % seizure reduction. Five continued LGIT further for a median duration of 8 months (range-4-12 months) successfully. The number needed to treat for more than 50 % seizure reduction was 3 and for more than 90 % seizure reduction was 10. The mean frequency of seizures for the intervention and control groups at three months of follow-up was not significantly different (p = 0.16), but the change in seizure frequency as compared to baseline was better in the intervention arm (p = 0.01). Three patients in the LGIT arm had non-serious adverse events (lethargy in two, vomiting in one). CONCLUSION: In children aged 2-8 years with drug-refractory epilepsy, the administration of LGIT along with ongoing anti-seizure medications (ASM) is more efficacious in reducing seizure frequency as compared to ASM alone.

5.
J Child Neurol ; : 883073821996095, 2021 Feb 24.
Artículo en Inglés | MEDLINE | ID: mdl-33624545

RESUMEN

This study aimed to assess the neurocognitive outcomes and their diffusion tensor imaging correlates in children (aged 6-16 years) with mild traumatic brain injury. This prospective analysis included 74 children with mild traumatic brain injury (52 boys; mean age: 9.5 [±2.7] years). Wechsler Intelligence Scale for Children-Indian adaptation (WISC-IV), Child Behavior Checklist, and Children's Sleep Habits Questionnaire were administered for 57 cases (at 3 months postinjury) and 51 controls of similar age. The findings of diffusion tensor imaging (done within 7 days of injury) were correlated with various WISC-IV indices. The presenting features at the time of injury were loss of consciousness (53%), confusion or disorientation (47%), and post-traumatic amnesia (10%). Other features in the acute phase included drowsiness (86%), headache (78%), balance problems (62%), nausea (47%), fatigue (45%), vomiting (35%), nasal or ear bleed (12%), sensitivity to sound and light (12%), etc. At 3 months postinjury, the children with mild traumatic brain injury performed poorly in terms of Intelligence Quotient, perceptual reasoning index, and processing speed index as compared to controls. Based on the Child Behavior Checklist, 17% of children with mild traumatic brain injury had internalizing behavioral problems in comparison with 4% of controls. Prevalence of poor sleepers in the mild traumatic brain injury cohort and controls was 12.3% and 2% respectively. Headache, reduced attention span, and fatigue were common postconcussion symptoms. There was a positive correlation between right uncinate fasciculus fractional anisotropy and verbal comprehension index (r = 0.32; P < .05).

7.
Pediatr Neurol ; 116: 7-13, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33388546

RESUMEN

BACKGROUND: The prevalence of sleep-disordered breathing is high in children with Down syndrome. Although the association between sleep-disordered breathing and developmental delay and behavioral abnormalities are proven among typically developing children, there are few such studies of children with Down syndrome. This study assesses the relationship between the severity of sleep apnea and development and behavioral abnormalities in individuals with Down syndrome. METHODS: In a cross-sectional prospective study, 53 children with Down syndrome were assessed for sleep-disordered breathing by overnight polysomnography. Behavior was assessed using Child Behavior Checklist (CBCL) and developmental quotient (DQ) using Developmental Profile 3. The association between various domains of behavior and development with the Apnea-Hypopnea Index (AHI) was assessed using Spearman rank correlation. Multiple linear regression assessed the independent effects of various factors on development and behavior. RESULTS: Of 53 subjects (three to 11.8 years), 51 (96%) were found to have obstructive sleep apnea (OSA). In both three to five year and six to 12 year age groups, there was a statistically significant positive correlation between the CBCL scores and the AHI (rho = 0.77 and 0.83, respectively). There was a statistically significant negative correlation between the DQ and the AHI (rho = -0.62). In multiple linear regression, AHI was the only independent variable that was associated with CBCL and DQ. CONCLUSIONS: This study provides robust evidence that OSA can negatively influence the development and behavior in children with Down syndrome as in typically developing children. Moreover, with increasing severity of OSA, children with Down syndrome have more behavioral abnormalities, especially attention deficit and hyperactivity, and also have poorer development scores.

8.
J Neuromuscul Dis ; 8(1): 125-136, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33337384

RESUMEN

BACKGROUND: Limb girdle muscular dystrophy recessive type 1 (LGMDR1, Previously LGMD2A) is characterized by inactivating mutations in CAPN3. Despite the significant burden of muscular dystrophy in India, and particularly of LGMDR1, its genetic characterization and possible phenotypic manifestations are yet unidentified. MATERIAL AND METHODS: We performed bidirectional CAPN3 sequencing in 95 LGMDR1 patient samples characterized by calpain-3 protein analysis, and these findings were correlated with clinical, biochemical and histopathological features. RESULTS: We identified 84 (88.4%) cases of LGMDR1 harboring 103 CAPN3 mutations (71 novel and 32 known). At least two mutant alleles were identified in 79 (94.2%) of patients. Notably, 76% exonic variations were enriched in nine CAPN3 exons and overall, 41 variations (40%) correspond to only eight exonic and intronic mutations. Patients with two nonsense/out of frame/splice-site mutations showed significant loss of calpain-3 protein as compared to those with two missense/inframe mutations (P = 0.04). We observed a slow progression of disease and less severity in our patients compared to European population. Rarely, presenting clinical features were atypical, and mimicked other muscle diseases like FSHMD, distal myopathy and metabolic myopathies. CONCLUSION: This is first systematic study to characterize the genetic framework of LGMDR1 in the Indian population. Preliminary calpain-3 immunoblot screening serves well to direct genetic testing. Our findings prioritized nine CAPN3 exons for LGMDR1 diagnosis in our population; therefore, a targeted-sequencing panel of nine exons could serve well for genetic diagnosis, carrier testing, counseling and clinical trial feasibility study in LGMDR1 patients in India.

9.
Brain Dev ; 43(1): 152-156, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32855000

RESUMEN

BACKGROUND: Infections are a common cause of childhood stroke with variable presentation. The current case describes a rare infective cause of venous and arterial stroke in an immunocompetent girl with management implications. CASE DESCRIPTION: A 12 year old girl, presented with history of fever for 10 days, painful swelling of right eye for 7 days and altered sensorium for 2 days. On examination, she had right eye orbital cellulitis and fullness of right paranasal area. On nervous system examination, she was delirious, had right eye ophthalmoparesis, left upper motor neuron facial palsy and signs of meningeal inflammation. Her contrast enhanced CT head and subsequent MRI brain with arteriography and venography revealed right cavernous sinus and distal internal carotid artery thrombosis. She was started on intravenous ceftriaxone and vancomycin and subcutaneous heparin. In view of persistent symptoms, endoscopic debridement of right nasal cavity was done, which showed growth of aspergillus flavus. Subsequently, she was started on intravenous voriconazole. Within a week, she was afebrile, her inflammatory and neurological signs started improving. She was discharged after 3 weeks of intravenous voriconazole which was continued for 3 more weeks orally. Her procoagulant and immunodeficiency work up were normal. At 4 months follow up, she showed both clinical and radiological resolution. CONCLUSIONS: Despite high mortality described in sino-orbital aspergillosis, early and appropriate treatment led to optimal outcome. In deep seated infections, isolation of etiological organism should be attempted, particularly when patient doesn't respond to conventional antimicrobial therapy.

10.
J Trop Pediatr ; 67(1)2021 01 29.
Artículo en Inglés | MEDLINE | ID: mdl-33367907

RESUMEN

BACKGROUND: During the current ongoing COVID-19 pandemic, psychological problems like anxiety, depression, irritability, mood swings, inattention and sleep disturbance are fairly common among quarantined children in several studies. A systematic review of these publications to provide an accurate burden of these psychiatric/behavioral problems is needed for planning mitigating measures by the health authorities. METHODS: Different electronic databases (MEDLINE, EMBASE, Web of Science, CENTRAL, medRxiv and bioRxiv) were searched for articles describing psychological/behavioral complications in children/adolescents with/without pre-existing behavioral abnormalities and their caregivers related to the COVID-19 pandemic. Only original articles with/without comparator arms and a minimum sample size of 50 were included in the analysis. The pooled estimate of various psychological/behavioral problems was calculated using a random-effect meta-analysis. RESULTS: Fifteen studies describing 22 996 children/adolescents fulfilled the eligibility criteria from a total of 219 records. Overall, 34.5%, 41.7%, 42.3% and 30.8% of children were found to be suffering from anxiety, depression, irritability and inattention. Although the behavior/psychological state of a total of 79.4% of children was affected negatively by the pandemic and quarantine, at least 22.5% of children had a significant fear of COVID-19, and 35.2% and 21.3% of children had boredom and sleep disturbance. Similarly, 52.3% and 27.4% of caregivers developed anxiety and depression, respectively, while being in isolation with children. CONCLUSION: Anxiety, depression, irritability, boredom, inattention and fear of COVID-19 are predominant new-onset psychological problems in children during the COVID-19 pandemic. Children with pre-existing behavioral problems like autism and attention deficit hyperactivity disorder have a high probability of worsening of their behavioral symptoms.


Asunto(s)
/psicología , Cuidadores/psicología , Pandemias , Cuarentena/psicología , Adolescente , Ansiedad/epidemiología , Niño , Estudios Transversales , Depresión/epidemiología , Humanos , Salud Mental , Problema de Conducta
11.
Epilepsia ; 61(12): 2763-2773, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33188527

RESUMEN

OBJECTIVE: The objectives of this study were to evaluate ENDIT score and develop a novel outcome prediction score for outcome of pediatric convulsive status epilepticus (CSE) at the hospital and 3 months postdischarge. METHODS: Children and adolescents aged 1 month to 14 years, presenting with CSE to a tertiary care teaching center in North India from January 2017 to March 2019, were screened for enrollment. In-hospital and 3-month postdischarge outcome were defined as poor if Pediatric Cerebral Performance Category Scale (PCPCS) score dropped by ≥2 levels. RESULTS: Overall, 61 patients were enrolled for final analysis after applying exclusion and inclusion criteria. The area under the receiver operating characteristic (ROC) curve for ENDIT score in predicting mortality and differentiating good from poor outcome at the hospital and at 3 months postdischarge was 0.74 (95% confidence interval [CI] = 0.58-0.89), 0.7 (95% CI = 0.57-0.83), and 0.72 (95% CI = 0.6-0.82), respectively. Based on predictors in the present cohort that were significantly different between good and poor outcome cases at the hospital and 3 months postdischarge, a new six-point score named PEDSS (pre-status epilepticus PCPCS, background electroencephalographic abnormalities, drug refractoriness, semiology, and critical sickness) was developed. The area under ROC curves for PEDSS score in predicting mortality and differentiating good from poor outcome at the hospital and at 3 months postdischarge were 0.93 (95% CI = 0.87-0.99), 0.8 (95% CI = 0.7-0.9), and 0.89 (95% CI = 0.8-0.96), respectively. The best cutoff PEDSS scores for predicting mortality and poor outcome at the hospital and at 3 months postdischarge were ≥4, ≥3, and ≥3, respectively. SIGNIFICANCE: The PEDSS score has high predictive accuracy for mortality and differentiating good from poor outcome at the hospital and 3 months postdischarge in pediatric CSE. Future studies should be planned to validate it in various geographical and health care settings and in adults.

12.
Seizure ; 83: 175-180, 2020 Oct 31.
Artículo en Inglés | MEDLINE | ID: mdl-33161247

RESUMEN

PURPOSE: To describe and correlate the clinical, radiological and EEG findings in children with lissencephaly. METHOD: Retrospective record analysis of children with lissencephaly presenting to tertiary health centre in Northern India was performed. Radiological classification and severity scoring were done. EEG findings were categorized into three patterns and its association with clinical severity was studied. RESULTS: Twenty-eight children (males = 17) with lissencephaly were enrolled. Median age at diagnosis was 6.5months (range 3days-3years). Global developmental delay (median social quotient (SQ) = 25 (range15-68) was seen in all; motor deficits in 23 (82 %); epilepsy in 21 (75 %); behavioural problems in 18 (64 %); ophthalmic problems in 17 (61 %); microcephaly in 13 (46 %); feeding difficulty in 12 (43 %). Radiologically, classical Type I lissencephaly was seen in 18(64 %), cobblestone variant (Type II) in 5 (18 %) and microlissencephaly in 5 (18 %). Grade 4 (diffuse pachygyria) radiologic severity was most common (severity grade 1-6); no cases with severity score 5 or 6 were seen. The clinical profile did not correspond with radiological severity grading. EEG pattern recognition revealed pattern I in 14 (50 %); pattern II in 6 (21 %); pattern III in 8 (29 %). Children with pattern III EEG had drug resistant epilepsy and severe developmental delay. No relationship between EEG patterns and radiological severity grading was evident. CONCLUSION: EEG is better predictor of clinical status and outcome rather than radiological severity grading. EEG pattern III is associated with severe developmental delay and drug resistant epilepsy.

13.
Seizure ; 82: 59-64, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33011589

RESUMEN

PURPOSE: This study aims to assess the prevalence of sleep abnormalities in children with drug-resistant epilepsy (DRE) and characterize their polysomnographic profile and to further compare it with well-controlled epilepsy (WCE) and age-matched typically developing children (TDC). METHODS: A cross-sectional study consisting of 40 children in each group (DRE, WCE, and TDC) was conducted. Children's sleep habits questionnaire (CSHQ) and modified pediatric Epworth daytime sleepiness scale (MPEDSS) were administered to all three groups. Thirty-five children each in the DRE and WCE group and 17 TDC underwent single night polysomnography (PSG). RESULTS: The prevalence of sleep abnormalities by the administration of CSHQ in DRE group was 72.5% (95% C.I-58.7 to 86.3%, mean score: 47.5 ± 7.1) compared to 32.5% (42.4 ± 6.2) and 15% (37.3 ± 5) in WCE and TDC groups respectively (P = 0.01). On MPEDSS, 52.5% of children in the DRE group had excessive daytime sleepiness compared to 12.5% in WCE and 5% in TDC groups respectively (p-0.03). On overnight PSG, sleep efficiency and REM sleep duration were significantly reduced in the DRE group in comparison to WCE and TDC. N2 duration, REM latency, arousal, and apnea-hypopnea index were significantly increased in the DRE group when compared to WCE and TDC groups. CONCLUSION: Sleep-related problems are major comorbidity in up to three-fourths of patients with DRE and sleep architecture is significantly affected particularly in the DRE group.

14.
Muscle Nerve ; 62(6): 699-704, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33002199

RESUMEN

Smooth muscle dysfunction in Duchenne muscular dystrophy (DMD) has been rarely studied. A cross-sectional study was conducted to estimate the prevalence of smooth muscle dysfunction (vascular, upper gastrointestinal, and bladder smooth muscle) in children with DMD using questionnaires (Pediatric Bleeding Questionnaire, Pediatric Gastroesophageal Symptom Questionnaire, and Dysfunctional Voiding Symptom Score). Investigations included bleeding time estimation, nuclear scintigraphy for gastroesophageal reflux, and uroflowmetry for urodynamic abnormalities. Ninety-nine subjects were included in the study. The prevalence of vascular, upper gastrointestinal, and bladder smooth muscle dysfunction was 27.2%. Mean bleeding time was prolonged by 117.5 seconds. The prevalence of gastroesophageal reflux was 21%. Voided volume/estimated bladder capacity over 15% and abnormal flow curves on uroflowmetry were seen in 18.2% and 9.7% of the subjects, respectively. Our study highlights the need for addressing issues related to smooth muscle dysfunction in the routine clinical care of patients with DMD.

15.
Indian J Pharmacol ; 52(4): 283-289, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33078729

RESUMEN

OBJECTIVES: The present study aimed to determine the pattern of prescription of antiepileptic drugs (AEDs) in a cohort of patients with epilepsy (PWE) attending a tertiary care center of North India. MATERIALS AND METHODS: Demographic variables including age, gender, age at onset, type and frequency of seizures, and prescription of all AEDs (dose and duration) were noted. Descriptive analysis of the use of AEDs was done, and their different combinations were studied. RESULTS: A total of 1187 prescriptions were evaluated. Demography showed 65.7% of males; mean age of 21.9 years (range: 2-77 years), generalized seizures (53%), and focal seizures (47%). Only 21.8% of the patients were seizure free with no seizure in 1 year of treatment. The five most frequently prescribed AEDs out of 12 AEDs were sodium valproate (VPA) (49.6%), clobazam (CLB) (39.3%), levetiracetam (LEV) (28.4%), carbamazepine (CBZ) (27.3%), and phenytoin (PHT) (26.5%). Monotherapy was effective in 36.6% of the patients. Sodium VPA (39.4%), PHT (25.6%), and CBZ (20.1%) were commonly used as monotherapy. Polytherapy was required in 63.4% of the patients, and most commonly prescribed combinations were PHT + CLB (n = 53), sodium VPA + CLB (n = 62), CBZ + CLB (n = 45), PHT + sodium VPA + CLB (n = 28), and CBZ + sodium VPA + CLB (n = 31). CONCLUSIONS: Polytherapy is a very common practice in our tertiary care center. Sodium VPA, a highly prescribed AED, results in good control of generalized seizures, whereas focal seizures are well controlled by CBZ alone as well as in combination. The present study highlights the commonly prescribed combinations of AEDs resulting in control of different types of seizures.

16.
Seizure ; 83: 98-103, 2020 Oct 06.
Artículo en Inglés | MEDLINE | ID: mdl-33120328

RESUMEN

BACKGROUND: Childhood epilepsy forms a significant burden on the health-care delivery system. Only a few pediatric neurologists available in most of the developing countries and caregivers face a lot of financial and logistic hardships, apart from a long waiting period for initial and follow up visits. Telemedicine is a proposed effective alternative in overcoming this burden. METHODS: Telephonic consultation by a pediatric neurology fellow was compared with that of a specialty nurse; both against face-to-face consultation (gold standard). Care-givers of children 4 months-18 years with epilepsy were telephonically consulted 24-48 hours before their scheduled hospital appointment by one specialty nurse and one pediatric neurology fellow at least 24 h apart in a random sequence. During the hospital visit, another pediatric neurology fellow blinded to the telephonic consultation, documented the same after Face-to-Face interview. RESULTS: In 141 children with epilepsy, 504 critical clinical events were identified. Telephonic consultation by pediatric neurology fellow had a sensitivity of 99 %, 97 %, and 100 % and specificity of 100 % each in detecting whether the child had any breakthrough seizure, any adverse event and whether the drug compliance was adequate or poor respectively, as compared to face-to-face consultation. Telephonic consultation by specialty nurse had a sensitivity of 91 %, 84 %, and 98 % and specificity of 97 %, 99 %, and 81 % in detecting whether the child had any breakthrough seizure, adverse event and whether the drug compliance was adequate or poor respectively. But the specialty nurses fared poorly in identifying atypical seizure semiologies like atonic and myoclonic seizures and documenting an exact number of breakthrough seizures, as well as few subjective adverse effects like behavioral abnormality and scholastic worsening, which was performed excellently by the pediatric neurology fellow. CONCLUSIONS: Telephonic consultation in childhood epilepsy by pediatric neurology fellow has excellent sensitivity and specificity. A specialty nurse has also acceptable sensitivity and specificity in comparison with a face-to-face consultation.

19.
JAMA Pediatr ; 174(10): 944-951, 2020 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-32761191

RESUMEN

Importance: The ketogenic diet (KD) has been used successfully to treat children with drug-resistant epilepsy. Data assessing the efficacy of the modified Atkins diet (MAD) and low glycemic index therapy (LGIT) diet compared with the KD are scarce. Objective: To determine whether the MAD and LGIT diet are noninferior to the KD among children with drug-resistant epilepsy. Design, Setting, and Participants: One hundred seventy children aged between 1 and 15 years who had 4 or more seizures per month, had not responded to 2 or more antiseizure drugs, and had not been treated previously with the KD, MAD, or LGIT diet were enrolled between April 1, 2016, and August 20, 2017, at a tertiary care referral center in India. Exposures: Children were randomly assigned to receive the KD, MAD, or LGIT diet as additions to ongoing therapy with antiseizure drugs. Main Outcomes and Measures: Primary outcome was percentage change in seizure frequency after 24 weeks of dietary therapy in the MAD cohort compared with the KD cohort and in the LGIT diet cohort compared with the KD cohort. The trial was powered to assess noninferiority of the MAD and LGIT diet compared with the KD with a predefined, noninferiority margin of -15 percentage points. Intention-to-treat analysis was used. Results: One hundred fifty-eight children completed the trial: KD (n = 52), MAD (n = 52), and LGIT diet (n = 54). Intention-to-treat analysis showed that, after 24 weeks of intervention, the median (interquartile range [IQR]) change in seizure frequency (KD: -66%; IQR, -85% to -38%; MAD: -45%; IQR, -91% to -7%; and LGIT diet: -54%; IQR, -92% to -19%) was similar among the 3 arms (P = .39). The median difference, per intention-to-treat analysis, in seizure reduction between the KD and MAD arms was -21 percentage points (95% CI, -29 to -3 percentage points) and between the KD and LGIT arms was -12 percentage points (95% CI, -21 to 7 percentage points), with both breaching the noninferiority margin of -15 percentage points. Treatment-related adverse events were similar between the KD (31 of 55 [56.4%]) and MAD (33 of 58 [56.9%]) arms but were significantly less in the LGIT diet arm (19 of 57 [33.3%]). Conclusions and Relevance: Neither the MAD nor the LGIT diet met the noninferiority criteria. However, the results of this study for the LGIT diet showed a balance between seizure reduction and relatively fewer adverse events compared with the KD and MAD. These potential benefits suggest that the risk-benefit decision with regard to the 3 diet interventions needs to be individualized. Trial Registration: ClinicalTrials.gov Identifier: NCT02708030.

20.
J Child Neurol ; 35(14): 970-974, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-32748676

RESUMEN

Traumatic brain injury is an important cause of acquired brain injury. The current study brings to light the clinicoepidemiologic profile of pediatric traumatic brain injury in India. Retrospective record analysis of children (aged ≤ 16 years) with traumatic brain injury presenting to an apex-trauma-center in North India over 4 years was done. Of more than 15 000 patients with a suspected head injury, 4833 were children ≤16 years old. Of these, 1074 were admitted to the inpatient department; 65% were boys with a mean age at presentation being 6.6 years. Most patients (85%) had a Glasgow Coma Scale score of 13 to 15 at presentation while Glasgow Coma Scale scores of ≤8 was seen in 10% of patients. Neuroimaging (computed tomography [CT]) abnormalities were seen in 12% of patients, with the commonest abnormality being skull fracture, followed by contusions, and extradural hemorrhage. Around 2% of patients required decompressive craniotomy whereas 3% of patients succumbed to their illness.Among the inpatients with pediatric traumatic brain injury, two-thirds were boys with a mean age at presentation of 7.6 years. Severity of traumatic brain injury varied as mild (64%), moderate (11%), and severe (25%). The most common mode of injury was accidental falls (59%) followed by road traffic and rail accidents (34%). Neuroimaging abnormalities were seen in half of inpatients with pediatric traumatic brain injury, with the commonest abnormality being skull fracture. Pediatric head injuries are an important public health problem and constitute a third of all head injuries. They are more common in boys, and the most common modes of injury are accidental falls, followed by road traffic accidents.

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