Efficacy of verbal suggestion, verbal suggestion with a tuning fork, and verbal suggestion with a cotton swab for inducing the paroxysmal event during video-EEG recording in children with suspected psychogenic nonepileptic seizures.

INTRODUCTION: Video-electroencephalogram (EEG) with suggestion is widely considered the gold standard for diagnosing psychogenic nonepileptic seizures (PNES). However, ethical concerns and uncertainties persist regarding the most minimally invasive and least deceptive suggestion approach. MATERIALS AND METHODS: In an open-label randomized controlled trial, we evaluated the effectiveness of three suggestion methods (verbal suggestion, verbal suggestion with a tuning fork, and verbal suggestion with a cotton swab) during short-term video-EEG (STVEEG) recordings to induce PNES in children aged 5-18 years. If the paroxysmal event couldn't be elicited with the assigned method, alternative techniques were employed. RESULTS: Out of 97 initially screened children, 75 were enrolled, with 25 in each group. The efficacy of all three suggestion methods was comparable in reproducing paroxysmal events (success rate of 16/25, 17/25 and 17/25 in verbal suggestion only, verbal suggestion with tuning fork and sterile cotton swab group respectively, p = 0.83) and the time required for induction (median of 2, 3 and 3 min respectively, p = 0.21). After trying alternative methods, 20 %, 12 %, and 12 % more patients in these three groups, respectively, were able to reproduce the paroxysmal event, with the differences not reaching statistical significance (p = 0.74). The assigned induction method or the success/failure of event reproduction did not significantly impact clinical outcomes at 12 weeks, and none of the patients in whom PNES could not be reproduced during STVEEG were later found to have an organic cause. Only the presence of psychiatric comorbidity independently predicted successful event reproduction during STVEEG, with statistical significance even after adjusting for other variables (p = 0.03). CONCLUSION: The efficacy of verbal suggestion alone in inducing paroxysmal nonepileptic seizures is on par with using a tuning fork or cotton swab in conjunction with verbal suggestion during STVEEG.

The spectrum of neurological manifestations and genotype-phenotype correlation in Indian children with Gaucher disease.

Gaucher disease (GD), one of the most frequent autosomal recessive lysosomal storage disorders, occurs due to bi-allelic pathogenic variants in the GBA1. Worldwide, the c.1448T>C (L483P) homozygous pathogenic variant is reported to be associated with neurological GD phenotype. Clinical distinction between GD1 and GD3 may be challenging due to subtle neurological features. Objective methods to evaluate neurological signs and saccades may help in early diagnosis. This study was conducted to assess the neurological phenotype, and its severity using a modified severity scoring tool (mSST), and the genotype-phenotype correlation. A total of 45 children aged 2 years 6 months to 15 years with a confirmed enzymatic and molecular diagnosis of GD with or without therapy were recruited. mSST tool was used to assess the severity of the neurological phenotype. A digital eye movement tracker (View Point Tracker) was used to assess eye movements. Clinical and genetic findings were analyzed. Out of 45 patients, 39 (86.7%) had at least one neurological phenotype detected using the mSST tool, with impairment of cognitive function (68.8%, 31/45) being the commonest feature. Thirty-two of 45 (71%) were assessed for saccadic eye movements using the eye tracker. Of these, 62.5% (20/32) had absent saccades. Four children (8.9%, 4/32) without clinical oculomotor apraxia had absent saccades on the viewpoint eye tracker. Overall, 77.7% (35/45), had homozygosity for c.1448T>C in GBA1 of which 91.4% (32/35) had neurological manifestations. Other alleles associated with neurological phenotype included c.1603C>T(p.R535C), c.1184C>T (p.S395F), c.115+1G>A (g.4234G>A), c.260G>A (p.R87Q) and c.1352A>G (p.Y451C). To conclude, in India, the c.1448T>C pathogenic variant in GBA1 is the commonest  and is associated with neurological phenotype of GD. Therefore, every patient of GD should be assessed using the mSST scoring tool for an early pick up of neurological features. The routine use of a viewpoint eye tracker in children with GD would be useful for early recognition of saccadic abnormalities.

The Childhood and Adolescent Sleep Evaluation Questionnaire (CASEQ): Development and validation of an ICSD-3-based screening instrument, a community and hospital-based study.

Sleep disorders are common in children and adolescents. Polysomnography is the gold-standard diagnostic method; however, it is a tedious procedure. The objective of the study was to develop a screening questionnaire for sleep problems based on International Classification of Sleep Disorders-3 in children and adolescents, and validate it with clinical evaluation and polysomnography. A questionnaire was developed in English and Hindi with content validation by eight multidisciplinary experts. Respondents were parents of children and adolescents, aged 2-18 years, recruited from a public school and a tertiary care teaching hospital in north India. A subset of these children and adolescents underwent overnight polysomnography and detailed clinical evaluation within 4 weeks of applying the questionnaire. The questionnaire, named Childhood and Adolescent Sleep Evaluation Questionnaire, contains primary questions covering all subgroups of disorders under International Classification of Sleep Disorders-3, and secondary questions on sleep hygiene and comorbidities. The questionnaire was filled by 750 respondents, out of which 100 cases underwent polysomnography and clinical evaluation. The internal consistency in the form of Cronbach's α was 0.8 for the questionnaire. The sensitivity, specificity, positive and negative predictive values for the questionnaire in identifying those with sleep problems compared with detailed clinical and polysomnographic evaluations were 85%, 100%, 100% and 62.5%, respectively. For individual subgroups of disorders, the sensitivity, specificity, positive and negative predictive values varied between 72.7% and 100%, 88.9% and 100%, 62.5% and 100% and 81.6% and 100%, respectively. The Childhood and Adolescent Sleep Evaluation Questionnaire has good psychometric properties, moreover, its simplicity and translatability make it ideal for use at the community and hospital settings.

Neurobehavioral deterioration associated with sleep-augmented epileptiform abnormalities: A steroid responsive state in children.

OBJECTIVE: Retrospective case record analysis of children with Neurobehavioral Deterioration associated with Sleep-augmented Epileptiform abnormalities (NDSE). METHODS: Hospital records of children with NDSE (July, 2015 through December, 2016) were analyzed. Children were categorized as: Encephalopathy with electrical status epilepticus in sleep (ESES) if sleep EEG Spike-wave-Index (SWI) was ≥50% and sleep-induced epileptiform activity (SIEA)-related cognitive dysfunction if SWI ≥25% but <50%. Demography, neurobehavior profile (IQ/SQ and behavior using validated psychometric tools), etiology, investigations and treatment details were documented. Outcome assessment was based on three-month follow-up records. RESULTS: Eighteen children with NDSE {12 boys; median age at diagnosis: 7.5 years (IQR: 6-10 years); SIEA (7); ESES (11)} were included. Etiology was structural (23%) and presumed genetic (77%). All children received intravenous-methylprednisolone pulse followed by oral steroids for eight weeks. Electroencephalography of children with SIEA was partly organized with median SWI of 40% (IQR 35, 42), with anterior-predominant epileptiform abnormalities and less apparent secondary synchronization. Children with ESES had a disorganized EEG background with median SWI of 80% (IQR 66, 95). Both SIEA and ESES groups had a similar neurobehavior profile. Behavior scores improved in 6/8 children with ESES and 5/7 in SIEA post steroids. In both the groups, median SWI improved (to <5% in SIEA, 45% in ESES). Mild improvement in IQ/SQ was also noted {SIEA [Median (IQR): 3 (1.6, 4.3)]; ESES [Median (IQR): 3.8 (2.8, 7)]}. CONCLUSION: The study supports the fact that SWI >50% in the nap EEG is not mandatory for the diagnosis of ESES, thus it should not be a constraint for steroid treatment.

Tumefactive demyelination in pediatrics: An unusual late neurological complication of hematopoietic stem cell transplant.

Immune-mediated demyelination is a rare posttransplant complication. Here, we report an 8.5-year-old boy who developed left hemiparesis, 18 months post matched sibling donor hematopoietic stem cell transplant (HSCT) for relapsed acute myeloid leukemia and was diagnosed to have tumefactive demyelination. The diagnosis was established based on clinical and radiological features. The complete resolution of the lesions with steroids further established the immune-mediated pathophysiology.

Development of a novel outcome prediction score (PEDSS) for pediatric convulsive status epilepticus: A longitudinal observational study.

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.

Prevalence of smooth muscle dysfunction among children with Duchenne muscular dystrophy.

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.

Saposin B-Deficient Metachromatic Leukodystrophy Mimicking Acute Flaccid Paralysis.

Metachromatic leukodystrophy (MLD) is a rare sphingolipid storage disorder caused by arylsulfatase A (ARSA) deficiency, resulting in central and peripheral demyelination. However, an uncommon form of MLD caused by saposin B deficiency is also described (around 10 mutations reported till date). MLD is a systemic disorder affecting the central and peripheral nervous system, gall bladder, and kidneys. Acute flaccid paralysis as the initial clinical presentation is previously known in ARSA-deficient MLD. Hereby, we report a child with acute flaccid paralysis with brain magnetic resonance imaging showing nonspecific periventricular leukodystrophy. He had progressive cognitive decline with gall bladder polyposis. ARSA levels were within normal limits. Leukodystrophy gene panel revealed a homozygous pathogenic deletion (Lys227del variant) in prosaposin (PSAP) gene. Hence, a final diagnosis of saposin B-deficient MLD was established. The index case highlights the importance of clinical and electrophysiological clues in the diagnosis of such atypical presentations of MLD.

Polyneuropathy in Critically Ill Mechanically Ventilated Children: Experience From a Tertiary Care Hospital in North India.

OBJECTIVES: To determine the prevalence of critical illness polyneuropathy and its risk factors in critically ill children mechanically ventilated for 7 days or more. DESIGN: Observational cohort study. SETTING: PICU of a tertiary care hospital from North India. PATIENTS: Children 1-15 years old admitted in PICU from June 2016 to September 2017, mechanically ventilated for 7 days or more, excluding those with diagnosed neuromuscular disease, stroke, or spinal pathology. INTERVENTION: Demographic details, diagnosis, treatment details, and anthropometry at admission and enrolment were recorded. Nerve conduction studies were performed after enrolment and repeated a week later, if the child was still in PICU. Medical Research Council scoring for muscle strength was performed in survivors. Risk factors including Pediatric Index of Mortality-2 score, sepsis, multiple organ dysfunction, hypoalbuminemia, use of steroids, neuromuscular-blocking agents, and vasopressors were recorded. Samples for the level of micronutrients (copper, zinc, folate, and vitamin B12) were collected at the time of enrolling the child and at the time of discharge. MEASUREMENTS AND MAIN RESULTS: Thirty-two children were enrolled, of whom 29 had features of critical illness polyneuropathy on evaluation at day 8 of mechanical ventilation (prevalence, 90.6% [95% CI, 80.5-100%]). The polyneuropathy was axonal in 26 (81.2%), mixed in one patient (3.1%), and uncharacterized in two (6.2%). Sepsis and multiple organ dysfunction were present in 31 subjects (96.9%). No risk factors for critical illness polyneuropathy could be identified although the study was not sufficiently powered to do so. The difference between serum micronutrient levels (copper, zinc, folate, and vitamin B12) between patients who developed polyneuropathy, and those who did not, was statistically insignificant. CONCLUSIONS: We observed a high prevalence of critical illness polyneuropathy in children in PICU, mechanically ventilated for 7 days or more; almost all of whom had underlying sepsis.

Childhood macrophagic myofasciitis: A series from the Indian subcontinent.

INTRODUCTION: Macrophagic myofasciitis (MMF) is a rare disorder, reported mainly in European adults, with occasional childhood cases. We report a series of 6 patients with pediatric MMF from the Indian subcontinent. METHODS: Clinical details, creatine kinase levels, and results of electromyography are described for patients diagnosed with MMF. Fresh-frozen and formalin-fixed muscle biopsies were evaluated by hematoxylin-eosin staining, histochemistry, immunohistochemistry, and electron microscopy. RESULTS: Six of 2,218 muscle biopsies were diagnosed as MMF; patient charts were reviewed. The 6 patients were all children; all presented with hypotonia and/or motor delay. Mean age at diagnosis was 16.2 months. There were 4 boys and 2 girls. All had a history of hepatitis B vaccination. Histopathology revealed infiltration by sheets of large periodic acid-Schiff stain-positive histiocytes. Ultrastructural examination demonstrated needle-shaped crystals within histiocytes. One patient had a co-existent neuromuscular disorder, merosin-deficient congenital muscular dystrophy. CONCLUSIONS: MMF is a rare inflammatory myopathy that should be considered in the differential diagnosis of congenital myopathies in children. Muscle Nerve 56: 71-77, 2017.

A Combination of Moyamoya Pattern and Cerebral Venous Sinus Thrombosis: A Case of Tubercular Vasculopathy.

BACKGROUND: Moyamoya vasculopathy, arising secondary to tubercular meningitis (TBM) is unusual. There have also been a few reports of cerebral venous sinus thrombosis (CVST) in TBM. A case of TBM, complicated simultaneously by Moyamoya syndrome and CVST, is being presented here. CASE: A 1-year-old girl presented with febrile encephalopathy, vomiting, seizures and left hemiparesis. Cerebrospinal fluid analysis was suggestive of TBM. Extensive infarcts were noted in the magnetic resonance imaging, involving right middle cerebral artery (MCA), anterior cerebral artery and the left MCA. Magnetic resonance venogram revealed left transverse venous sinus thrombosis and magnetic resonance angiography showed bilateral moyamoya pattern of arteriopathy. Patient was started on antitubercular therapy and low molecular weight heparin. CONCLUSIONS: Early vascular involvement affecting both arterial and venous structures has not hitherto been reported in CNS tuberculosis. Early recognition of secondary complications of CNS tuberculosis is crucial to prevent the morbidity and mortality associated with TBM.

Tolvaptan for Treatment of Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) in a Child with Corpus Callosum Agenesis.

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is one of the common causes of euvolemic hyponatremia (serum Na+ < 135 mEq/L) in hospitalized children. It is characterized by increased serum ADH, leading to water retention via its action on V2 receptors in the distal renal tubules. Various conditions such as pain, the postoperative state, drugs, central nervous system infections, tumors, malformations, and pneumonia can predispose a person to SIADH. The conventional treatment of SIADH includes fluid restriction and salt supplementation. Occasionally, this may fail to control hyponatremia, mandating pharmacological therapy. V2-receptor antagonists are an FDA-approved therapy for adults with euvolemic and hypervolemic hyponatremia. However, there is limited experience with their use in the pediatric population. Here, the authors present a girl with corpus callosum agenesis with severe symptomatic hyponatremia due to SIADH who was successfully managed with the V2-receptor antagonist tolvaptan.

A Relook at Cerebral Palsy Beyond Motor Pathology: A Cross-Sectional Study of Sensory Processing Abilities.

BACKGROUND: Sensory processing refers to receiving, organizing, and interpreting sensory stimuli from the sensory system. Unlike other neurodevelopmental disorders, knowledge about the sensory processing abilities of children with cerebral palsy (CP) is lacking. OBJECTIVE: To study the difference in sensory processing abilities of children with cerebral palsy in comparison to age matched typically developing children (TDC). METHODS AND MATERIAL: A cross-sectional analysis of sensory processing abilities of children with CP and TDC was performed from July 2018 through February 2020. The child sensory profile2 (CSP2) caregiver questionnaire was used to detect sensory processing differences (SPD) across nine sensory domains and four sensory processing patterns. A comparison was made between the two study groups as well as between the CP subtypes. RESULT: Around 226 children with CP and 58 TDC were screened. Finally, 150 children with CP and 50 TDC were enrolled. Probable SPD (>1SD) was observed in (121/150) 80.7% of children with CP compared to (13/50) 26% in TDC (p < 0.001). Definite SPD (>2SD) was seen in 40.7% (61/150) of children with CP vs. none in TDC (p < 0.001). The body position domain which tests the vestibular and proprioceptive processing was primarily affected in CP. Most children with CP fell under the "bystander" pattern suggesting poor registration of sensory stimuli. No significant difference in the pattern of sensory processing was observed between the CP subtypes. Prevalence of definite SPD positively correlated with the gross motor functional classification system level. CONCLUSION: Sensory processing abilities of children with CP differ significantly from TDC. Proprioceptive and vestibular sensory processing is primarily affected in CP.

Optimal duration for recording EEG in children and adolescents- a prospective interventional study.

PURPOSE: This study aimed to determine the proportion of EEG recordings yielding diagnostic findings leading to a change in diagnosis beyond a 20-minute recording window, striking a balance between diagnostic yield and clinical practicability. METHODS: At a tertiary care teaching hospital in North India, 225 subjects aged 1 month to 18 years undergoing outpatient EEG were enrolled. Patients with epileptic encephalopathies, nonepileptic phenomena, and breakthrough seizures in the last 24 hours were excluded. Two recording protocols were employed: Category A (n=163, awake recording with activation procedures for 15 minutes followed by an attempt at sleep for 60 minutes) and Category B (n=62, sleep recording for 55 minutes followed by 5 minutes of awake recording for younger children and those with impaired cognition). EEGs were prospectively reported at 20, 30, 40, 50, and 60-minute time points, with no retrospective changes allowed. RESULTS: Among abnormal EEGs, the final diagnosis was changed beyond 20 minutes in 38.9% and 20.4% in categories A and B, respectively. A significant change in the final diagnosis among abnormal EEGs beyond 20 minutes was seen in - those who achieved sleep compared to those who didn't (45% versus 19%, p=0.03) in category A, and - focal compared to generalised seizures (Category A: 26.1% versus 8.3%, p=0.01; Category B: 23.8% versus 0%, p=0.02). CONCLUSION: Forty minutes of awake EEGs with/without sleep and 30 minutes of sleep EEGs achieve a final diagnosis in nearly 90%. Prolonging awake records beyond 20 minutes, incorporating sleep, is particularly beneficial in focal epilepsies.

Child Neurology: Allgrove Syndrome: An Intriguing Etiology of Motor Neuron Disease in Children

Motor neuron diseases are a rare group of neurodegenerative disorders with considerable phenotypic heterogeneity and a multitude of etiologies in the pediatric population. In this study, we report 2 unrelated adolescents (a boy and a girl) who presented with 4-6 years of progressive difficulty in walking, thinning of limbs, and gradually progressive darkening of the skin. Examination revealed generalized hyperpigmentation of skin and features suggestive of motor neuron involvement such as tongue atrophy, wasting of distal extremities, and brisk deep tendon reflexes. On detailed exploration for systemic involvement, history of dysphagia, inability to produce tears, and Addisonian crises were evident. An etiologic diagnosis of Allgrove syndrome, which is characterized by a triad of achalasia, alacrimia, and adrenal insufficiency was considered. Next-generation sequencing revealed pathogenic variants in the AAAS gene, confirming the diagnosis. Steroid replacement therapy was initiated along with relevant multidisciplinary referrals. The disease stabilized in the boy and a significant improvement was noted in the girl. These cases highlight the value of non-neurologic cues in navigating the etiologic complexities of motor neuron diseases in children and adolescents. It is imperative for neurologists to develop awareness of the diverse neurologic manifestations associated with Allgrove syndrome because they are often the first to be approached. A multidisciplinary team of experts including neurologists, endocrinologists, gastroenterologists, ophthalmologists, and dermatologists is essential for planning comprehensive care for these patients.

NDUFV1-Related Mitochondrial Complex-1 Disorders: A Retrospective Case Series and Literature Review.

BACKGROUND: Pathogenic variants in the NDUFV1 gene disrupt mitochondrial complex I, leading to neuroregression with leukoencephalopathy and basal ganglia involvement on neuroimaging. This study aims to provide a concise review on NDUFV1-related disorders while adding the largest cohort from a single center to the existing literature. METHODS: We retrospectively collected genetically proven cases of NDUFV1 pathogenic variants from our center over the last decade and explored reported instances in existing literature. Magnetic resonance imaging (MRI) patterns observed in these patients were split into three types-Leigh (putamen, basal ganglia, thalamus, and brainstem involvement), mitochondrial leukodystrophy (ML) (cerebral white matter involvement with cystic cavitations), and mixed (both). RESULTS: Analysis included 44 children (seven from our center and 37 from literature). The most prevalent comorbidities were hypertonia, ocular abnormalities, feeding issues, and hypotonia at onset. Children with the Leigh-type MRI pattern exhibited significantly higher rates of breathing difficulties, whereas those with a mixed phenotype had a higher prevalence of dystonia. The c.1156C>T variant in exon 8 of the NDUFV1 gene was the most common variant among individuals of Asian ethnicity and is predominantly associated with irritability and dystonia. Seizures and Leigh pattern of MRI of the brain was found to be less commonly associated with this variant. Higher rate of mortality was observed in children with Leigh-type pattern on brain MRI and those who did not receive mitochondrial cocktail. CONCLUSIONS: MRI phenotyping might help predict outcome. Appropriate and timely treatment with mitochondrial cocktail may reduce the probability of death and may positively impact the long-term outcomes, regardless of the genetic variant or age of onset.

Evaluation of Infantile Brachial Plexopathy Using 3T MRI and High-Resolution Ultrasound: Experience From a Tertiary Care Centre.

BACKGROUND: Currently, clinical assessment is the main tool for the evaluation of brachial plexus injury, complemented by electrophysiologic studies (EPS), and imaging studies whenever available. Imaging plays an important role as it enables the differentiation of pre-ganglionic and postganglionic injuries, and adds objectivity to presurgical evaluation. OBJECTIVES: The primary objective was to evaluate the utility of magnetic resonance imaging (MRI) and high-resolution ultrasonography (USG) in the localization and characterization of brachial plexus injury in infants. MATERIALS AND METHODS: In this prospective study, 34 infants with signs and symptoms of brachial plexus injury were evaluated by clinical examination, EPS, MRI, and USG. Imaging findings were correlated with intraoperative findings in infants who underwent surgical management. The association between EPS and MRI findings, and USG and MRI findings were assessed using Fisher's exact test. Semi-quantitative subjective analysis of various MRI sequences was done as well. RESULTS: The most common findings of preganglionic injury and postganglionic injury, in our study, were pseudomeningocele and nerve thickening, respectively. MRI detection of injuries had a significant association with EPS findings. All MRI-detected injuries had a muscle power of grade 3 or less. muscle. Three-dimensional (3D) short tau inversion recovery (STIR) sequence was found to be superior for detecting postganglionic injuries (P < 0.05). CONCLUSION: Imaging studies enable localization of the site of injury, determining the extent, and nature/morphology of injury. The gamut of findings obtained from MRI is far wider compared to that from USG. USG can be used as the first-line screening investigation.

Efficacy of daily versus intermittent low glycemic index therapy diet in children with drug-resistant epilepsy: A randomized controlled trial.

INTRODUCTION: The predominant reason for the discontinuation of low glycemic index therapy (LGIT) in children with epilepsy is the dietary restrictions imposed therein. This trial intended to compare the efficacy of daily and intermittent LGIT in children with drug-resistant epilepsy (DRE). METHODS: This study was performed between February 2018 and January 2019 to compare the efficacy of daily and intermittent LGIT in children aged 1-15 years with DRE following 24 weeks of dietary therapy. Compliance, the difficulty faced by caregivers, adverse effects, impact on behaviour, and social quotient in both arms were compared. Children in the intermittent LGIT arm received a liberalized diet for two days every week (Saturday and Sunday), which also allowed medium glycemic index foods. Carbohydrate calories were allowed up to 20% of the total caloric requirement in the liberalized diet, as compared to only 10% in standard LGIT. RESULTS: Out of 132 children randomized (66 in each group), 122 completed 24 weeks follow up. Mean weekly seizure frequency reduction at 24 weeks in the intermittent LGIT group was comparable with that of the daily LGIT group in both intention-to-treat (ITT) and per-protocol analysis (-50.95%± 22.34% vs -47.16%± 23.41%, p=0.36 in ITT and -53.88%±20.54% vs -49.20%±21.87%, p=0.23) in per-protocol analysis for intermittent and daily LGIT group respectively). The proportion with ≥50% reduction in seizure frequency was also comparable between both groups (p=0.73 and 0.56 in ITT and per protocol analysis respectively). The proportion of patients with adverse events and satisfactory compliance rate also had a trend towards favoring intermittent LGIT (p=0.06 and 0.51, respectively), while caregiver difficulty was lower with intermittent LGIT (p=0.001). CONCLUSIONS: Intermittent LGIT is comparable to daily LGIT in terms of seizure frequency reduction after 24 weeks of dietary therapy. TRIAL REGISTRATION: ClinicalTrials.gov (Registration number- NCT03464487, https://clinicaltrials.gov/ct2/show/NCT03464487).

Disseminated phaeohyphomycosis due to Cladophialophora bantiana in an immunocompetent child.

A 3-year-old boy presented with acute headache, vomiting and right focal clonic seizures without history of fever, joint pain or altered sensorium. Neuroimaging showed multifocal contrast enhancing lesions with significant perilesional edema. CECT chest and abdomen showed multiple variable sized nodules in the lungs and hypodense lesion in liver with mesenteric lymphadenopathy. There was persistent eosinophilia with maximum upto 35 %. Liver biopsy and brain biopsy revealed Cladophialophora bantiana. He was treated with IV liposomal amphotericin and voriconazole for 6 weeks with repeat neuroimaging showing more than 50 % resolution of the intracranial lesions. He was transitioned to oral combination of flucytosine and voriconazole. At 14 months follow-up, he remained symptom free with complete radiological resolution of the lesions and no eosinophilia. High suspicion, an aggressive approach in obtaining microbiological diagnosis and timely combination antifungal therapy may give satisfactory outcome without surgery.

Incident Breakthrough Seizures, Serum Matrix Metalloproteinase-9 and Perfusion Magnetic Resonance Imaging Parameters in a Cohort of Children and Adolescents With Neurocysticercosis: A Longitudinal Observational Study.

BACKGROUND: The current study estimated incident breakthrough seizures, serum matrix metalloproteinase-9 (MMP-9), and perfusion magnetic resonance imaging (MRI) parameters in five- to 18-year-olds with neurocysticercosis (NCC) from colloidal or vesicular through calcified stages over at least 24 months' follow-up. METHODS: Single, colloidal, or vesicular parenchymal NCC cases were treated with albendazole and steroids and followed at a tertiary care north Indian hospital. Serum MMP-9 was estimated in colloidal or vesicular treatment-naive state and in a subset of calcified cases at six-month follow-up. The same subset of calcified cases also underwent perfusion MRI of the brain at six-month follow-up. RESULTS: Among 70 cases, 70% calcified at six-month follow-up. Over a median follow-up of 30 months, the incidence of breakthrough seizures was 48.6% (61.2% in calcified and 19.2% in resolved, P = 0.001; 32.9% early [within six months] and 15.7% late [beyond six months], P = 0.02). Serum MMP-9 levels were higher in colloidal and vesicular compared with calcified stage (242.5 vs 159.8 ng/mL, P = 0.007); however, there was no significant association with breakthrough seizures and/or calcification in follow-up. In a subgroup of calcified cases (n = 31), the median relative cerebral blood volume on perfusion MRI in and around the lesion was lower in those with seizures (n = 12) than in those without (n = 19) (10.7 vs 25.2 mL/100 g, P = 0.05). CONCLUSIONS: In post-treatment colloidal or vesicular NCC, incident breakthrough seizures decrease beyond six months. In calcified NCC with remote breakthrough seizures, significant perilesional hypoperfusion is seen compared with those without seizures.