Electrical status epilepticus during slow-wave sleep (ESES) is an epilepsy syndrome with sleep-induced epileptic discharges and acquired impairment of cognition or behavior. Since the disease’s original description in 1971, no clear consensus has emerged on diagnostic criteria or optimal treatment. The treatment of ESES can be challenging, often including numerous antiepileptic drugs, immunomodulatory agents, and even surgical interventions. There is little evidence to guide treatment because only retrospective studies and case reports on the efficacy of treatment of ESES are present in literature. In this paper, we aim to analyze the etiopathogenesis of ESES in the new genetic era and to evaluate the treatment modalities in accordance with the genetic data and electroclinic spectrum of ESES.

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Objective: Hydrocephalus is one of the most common complications of tuberculous meningitis. Various cerebrospinal fluid diversion procedures, endoscopic third ventriculostomy (ETV) and shunt surgery, are performed for the management of the hydrocephalus associated with tuberculous bacterial meningitis (TBM). There is decreased clarity on the type of procedure to be done. So, this study aims at generating knowledge to understand the conditions in which either of the two procedures, ETV and shunt surgery, is a better option and to develop good practice guidelines for the treatment of tubercular meningitis hydrocephalus (TBMH). Materials and Methods: A systematic search of literature was performed by using PubMed and Cochrane Central Register of Controlled Trials (CENTRAL) for articles published from January 2001 to April 2020. A total of 15 studies were included irrespective of their design and having diagnosis with TBMH treated with ventriculoperitoneal shunt surgery (VPS) or ETV. Then, a systemic review was done regarding outcomes, complications, and recommendations made in different studies. Results: The total number of patients diagnosed with TBMH was 603; among them, 348 patients had VPS and 255 patients had ETV. In the VPS group, the patient’s good outcome varied from 25% to 68% and complication rate ranged from 10% to 43.8%. The average percentage of good outcome in the VPS group was 51.8%. In the ETV group, the patient’s good outcome varied from 41% to 77% and complication rate ranged from 10% to 23.5%. The average percentage of good outcome in the ETV group was 68%. There was a significant difference in the average percentage of good outcome in the ETV versus the VPS shunt group (P-value was 0.008). Conclusion: There was a significant difference in the average percentage of good outcome after ETV than VPS, and complication rate was also slightly lower after ETV than VPS during the chronic phase of illness. So during the acute phase of illness VPS placement is preferred whereas in the chronic burnout phase ETV is preferred because poor anatomy leads to more complications with ETV during the acute phase of the disease.

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Background: Common first-line antiepileptic drugs (AEDs) used in children are valproate, phenytoin, and levetiracetam. Many side effects for these AEDs are reported including obesity, atherosclerosis, and metabolic syndrome. The aim of our study was to evaluate changes in carotid artery intima-media thickness (CIMT) in epileptic children and to correlate with lipid profile of those who are on long-term antiepileptic therapy. Materials and Methods: This case–control study was done over 18 months in department of pediatrics. Sample size was 84 with equal number of cases and controls. Epileptic children between 1 and 18 years of age receiving monotherapy with valproate, phenytoin, or levetiracetam for at least 6 months were included in the study. Measurement of CIMT was done by B mode ultrasonography. Lipid profile was analyzed. Statistical analysis was performed using one-way analysis of variance (ANOVA) test and Pearson correlation. Results: Among 42 cases of epilepsy, 30 were on valproate, 9 on phenytoin, and 3 on levetiracetam monotherapy. No significant difference was noted in body mass index (BMI) among children receiving AED compared with that of controls (P = 0.82). Mean value for CIMT was significantly higher among valproate (0.43 ± 0.04, P ≤ 0.001), phenytoin (0.44 ± 0.04, P ≤ 0.001), and levetiracetam group (0.43 ± 0.03, P = 0.01) compared to controls (0.39 ± 0.01). Significant correlation was noted between CIMT and total cholesterol (P = 0.034), triglyceride (P = 0.011), low-density lipoprotein (P = 0.008), and very low-density lipoprotein (P = 0.011). Conclusion: Children on long-term monotherapy with valproate, phenytoin, and levetiracetam have significantly abnormal CIMT. This might be associated with atherosclerotic changes, and these children may require close follow-up to prevent cardiovascular and cerebrovascular risks.

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MTHFR enzyme deficiency is an autosomal-recessive inborn error of folate metabolism. The deficiency cause defect in the remethylation of homocysteine to methionine leading to increased blood levels of homocysteine. Hyperhomocysteinemia in infants cause seizures, hypotonia, apnoea, microcephaly, progressing to coma and death if untreated whereas in childhood onset it causes developmental delay, seizures, psychiatric disturbances, spastic gait, and ataxia. We report a 10-year-old girl with rapidly progressive spastic paraplegia requiring wheelchair ambulation within 3 months of symptom onset with behavioral disturbances. Plasma homocysteine and plasma lactate were high with normal vitamin B12 levels. Clinical exome sequencing showed homozygous missense mutation in MTHFR gene which was likely pathogenic variant. Respiratory chain complex assay from muscle sample showed reduced complex 1 deficiency (<20%).

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β-Mannosidosis is a rare lysosomal storage disorder that is caused by a deficiency of β-mannosidase activity, which is due to mutations of the MANBA gene. Two Indian siblings born out of a third-degree consanguineous marriage presented during late infancy with global developmental delay. On examination, both the siblings had hypotonia; hepatosplenomegaly was present in the first sibling whereas it was absent in the second sibling. Fundus evaluation, hearing assessment, and skeletal survey were normal in both siblings. Enzyme assay showed the absence of the β-mannosidase enzyme. Next-generation sequencing showed a homozygous variation of c.1317 + 1G>A in intron 10 of the MANBA (–) gene in the elder sibling. Sanger sequencing confirmed the same mutation in the homozygous state in both siblings and in the heterozygous state in both parents.

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Aneurysm in the extracranial segment of the internal carotid artery is extremely rare in children. Traumatic aneurysms of the internal carotid artery are generally “false aneurysms” (pseudoaneurysms) with at least part of the aneurysm wall composed only of the adventitial layer, or even just by the hematoma. There are few reports in the world medical literature about its etiology, presenting features, and catastrophic consequences. The natural history of the disease has not been well-defined yet. However, the potential risk of embolism originating from the aneurysm or even its rupture indicates a need for early diagnosis and intervention. We present a 12-year-old previously healthy child with recurrent oropharyngeal hemorrhage leading to massive hematemesis and hypovolemic shock due to right-sided internal carotid artery pseudoaneurysm with hemorrhage and right middle cerebral artery territory infarct. The child required urgent endovascular stenting with decompressive craniectomy for life-threatening intracranial bleeding with herniation with near-complete recovery.

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Background: Hydrocephalus is a complex and multifactorial neurological disorders. Childhood hydrocephalus like other chronic childhood illness is a major contributor to poor quality of life and huge financial burden to the affected family and nation. Epidemiological factors tend to vary with geographical location. Unlike in developed countries, most data from developing countries showed infection as the most common etiology. This study was conducted to analyze our epidemiological features of childhood hydrocephalus in Sokoto with a review of the literature. Aims: The aims of this study were to determine the frequency of causes of hydrocephalus in Sokoto and to determine the short-term outcomes of treatment and compare results with the literatures. Materials and Methods: This was a retrospective study with patients’ data from the theatre records, patients’ case notes, and radiological records.Outcomes were based on complications and changes in the occipitofrontal circumferences. Criteria for statistical significant was P < 0.05. Results: One hundred and thirty-eight patients satisfied inclusion criteria. No sex preponderance was found with a mean age of 16.41 months. Maternal illiteracy rate was 60% with 49.3% of the parents at lower socioeconomic class. Infection was the most frequent etiology (45.7%). Myelomeningocele associated hydrocephalus was also common (16.7%). However, post-hemorrhagic hydrocephalus was rare (2.9%). Mean preoperative and postoperative occipitofrontal circumferences were 54.22 cm and 47.92 cm, respectively, with P = 0.001. Conclusion: Poverty and illiteracy were strongly associated with childhood hydrocephalus in our patients. Infection was predominantly associated with morbidity with large number of patients being lost to follow-up.

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Background: Despite the advent of modern neurosurgical techniques, new antibiotics, and modern imaging techniques, infection after ventriculoperitoneal (VP) shunt insertion and/or ventriculostomy is still a serious issue. Aim: The aim of this work was to study bacteriological profile and antimicrobial susceptibility pattern of cerebrospinal fluid shunt infections in infants and children. Materials and Methods: A total of 90 patients under the age of 12 years undergoing cerebrospinal fluid shunt procedures were included. The CSF shunt fluid, external ventricular drain (EVD), shunt tube tip specimens were processed as per standard microbiological techniques. The organisms isolated were subjected to antimicrobial susceptibility using the Kirby–Bauer disk diffusion method. Results: Of 20 infected patients 10 (50%) were culture positive. Most common organisms isolated were Acinetobacter baumannii 03 (30%) followed by Enterococcus faecalis 2 (20%). Two isolates of A. baumannii and one isolate of Klebsiella pneumoniae showed carbapenem resistance, which were sensitive to colistin. All Gram-positive isolates were sensitive to vancomycin and linezolid. Reinfection was found only in one (8.33%) patient. In 12 (60%) infected cases with shunt failure, revision of shunt was done. The shunt related mortality in this study was 1.11%. Conclusion: Most common organisms isolated were A. baumannii followed by E. faecalis. Carbapenem resistance was noted in these isolates which were sensitive to colistin. All Gram-positive isolates were sensitive to vancomycin and linezolid.

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Background: The current study was conducted to describe the findings of brain perfusion, hippocampal volumetric, and diffusion-weighted imaging (DWI) in children aged six months to seven years with febrile status epilepticus (FSE) and focal febrile seizures (FFS) when compared with age and gender-matched controls. Materials and Methods: This cross-sectional study was conducted among children aged six months to seven years presenting with FSE or FFS within 72 h of the seizure. Cases were subjected to magnetic resonance imaging (MRI) brain and sleep-deprived electroencephalography. Age and gender-matched children who were subjected to MRI brain for nonepileptic indications served as their control. Hippocampal volumes, T2 values, cerebral blood flow, and diffusion characteristics were compared between the cases and controls and also between those with FSE and FFS. Results: A total of 31 cases (FFS = 20, FSE = 11) and 30 controls were enrolled. There was no significant difference between right and left hippocampal volumes and T2 relaxometry values between cases and controls and also between children with FSE and FFS. Similarly, the cerebral blood flow was also comparable in cases and controls. There was a single case of FSE with hippocampal malrotation; one child showed diffusion restriction in the hippocampus after prolonged (>60 min) FSE. Conclusion: Children with FSE and FFS had comparable hippocampal volume and brain perfusion to healthy children. However, one child with FSE had hippocampal malrotation and another had diffusion restriction. The study findings need to be interpreted in the context of small sample size, and lack of follow-up neuroimaging.

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A bone marrow transplant recipient on cyclosporine initiated on ciprofloxacin for a renal abscess presented with encephalopathy, right hemiparesis, and multiorgan dysfunction. Imaging revealed white matter signal changes characteristic of cyclosporine leukoencephalopathy. This case illustrates the potential drug interaction of cyclosporine with ciprofloxacin and the need to exercise caution while prescribing antibiotics with cyclosporine.

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Background: Meningioma constitutes only 0.4%–4.1% of all the pediatric tumors. This article aims to find the impact of the pediatric meningioma surgery on the developmental and scholastic performance among these children over long-term follow-up. Materials and Methods: This study is a retrospective analysis of all the histopathological proven pediatric meningioma and a cross-sectional analysis to study the functional outcome, using Malin’s Intelligence Scale for Indian children (MISIC); scholastic performance was assessed from behavioral checklist for screening the learning disabled (BCSLD) and subjective self-filled questionnaire to know parent satisfaction. Results: Twenty-eight patients (mean age 14.52 ± 0.722 years) (M:F = 16:12) were analyzed (6 [21.4%] were grade-schooler and 22 [78.5%] were teenage), with most common symptom being headache (n = 20, 71.4%) and mean duration of symptoms was 11.19 ± 16.25 days. The mean intelligent quotient (IQ) of grade-schooler was 83.4 ± 9.072 compared to 75.69 ± 9.903 among teenage group. The BCSLD analyses showed that the postoperative score was poorer. Similarly, an average change in BCSLD was observed among the patients with complications (10) compared to the patients without complication (8). Conclusion: The discontinuation in schooling or lack of alternative education may lead to poor MISIC scores, wrongly categorizing the children in poor IQ group. In pediatric benign disease such as meningioma, the holistic approach should be opted from the time of first visit to neurological care team.

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Background: Head injury is one of the leading causes of mortality and morbidity in the developing as well as developed countries. Extradural hematoma is seen in 1–2% of cases of head injury patients. Extradural hematoma is infrequent in the pediatric age group (less than 18 years) as duramater is tightly adherent to the inner table of the skull and is present in about 1–3% of all pediatric head injuries. Materials and Methods: The study was a prospective study conducted in the Department of Neurosurgery at PGIMER, Dr. RML Hospital, New Delhi, over a period of 19 months with a follow-up of 6 months. In total, 38 patients were enrolled in the study. All traumatic extradural hematoma below 18 years of age of both sexes were included. Follow-up of all patients was done at 2 weeks, 1, 2, and 6 months. Noncontrast computed tomography (NCCT) head was done in all patients at the time of admission and whenever their neurological status deteriorated. Routine postoperative NCCT head was done. Outcome assessment was done by Glasgow outcome scale. Results: Of the 38 children, 21 were males and 17 were females. The maximum number of patients was between the ages of 1–5 years (55.26%). Fall from height was the most common cause. Loss of consciousness was the most common presenting symptom (78.94%). Thirty-three patients had GCS of 14–15 at the time of presentation. The parietal region was the most common location of EDH in our study (44.74%). Thirty-five patients had supratentorial and three patients had infratentorial EDH. Eighteen patients had associated brain injury, commonest being brain edema. Six patients were operated. There was no mortality in our study. Two patients had limb paresis. Conclusion: Pediatric EDH is a rare entity but a potentially life-threatening condition. Prompt diagnosis and timely intervention decrease morbidity and mortality.

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We report a rare cause of paraparesis in a 2-year-old girl. Along with lower limb weakness, she presented with a neurogenic bladder and lower limb deformities. Her magnetic resonance imaging showed multiple dorsolumbar segmentation defects and a dysgenetic spinal cord segment between T10 and S1. A thin septum connected the lower end of the normal cord to a bulky conus at S1-2. These features were suggestive of type 1 segmental spinal dysgenesis. The findings were confirmed at surgery, and the child was referred for supportive care.

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