Purpose: Chiari malformation type III (CM III) is the rarest type compared to other types of CMs. CM III usually reported as sporadic case reports which reflect the rarity of this anomaly. We report two cases of operated CM III at our institute with a reasonable outcome and reviewed the literature to illustrate the variability of prognosis and related hydrocephalus. Materials and Methods: We operated two cases of CM III in our hospital followed by ventriculoperitoneal shunt (VPS) placement with an accepted neurological outcome at 10 and 6 months follow-up. We reviewed the literature for other cases of CM III with focusing on prognosis to illustrate the real image of reported prognosis and related hydrocephalus. Results: After follow-up for 10 and 6 months, respectively, both cases had mild developmental delays. In this review, we report 51 cases of CM III over the last 30 years since 1989, there was slight male predilection, hydrocephalus was evident in 27 cases which was almost managed with VPSs and was evident in seven deaths. Conclusion: CM III is a rare anomaly which usually carries a bad prognosis, but death is not ultimate, and there may be a minority who carry good prognosis. This bad prognosis pushes some parents to refuse surgery otherwise repair should be done. With good pre- and postoperative care, physical therapy, and follow-up, the outcome is reasonable.

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The corpus callosum is the principal supratentorial cerebral commissure, which connects the two cerebral hemispheres in the midline. It is divided into rostrum, genu, body, and splenium. Affected patients may develop mental retardation, dysmorphic features, spasticity, ataxia, or epilepsy. Corpus callosal abnormalities may be isolated or be associated with other anomalies such as sulcal abnormality, ventriculomegaly, cerebellar hypoplasia or cerebellar vermian hypoplasia. Magnetic resonance imaging (MRI) plays a major role in the diagnosis of fetal corpus callosal developmental abnormalities when they are suspected on sonography. This pictorial essay shows the MRI findings in fetal corpus callosal developmental abnormalities in a very systematic manner.

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Background: Vitamin B12 deficiency occurs primarily as a result of insufficient dietary intake in children in developing countries. Vitamin B12 deficiency produces a cluster of neurological symptoms in children. Aim: The aim of this study was to describe the vitamin B12 status of patients who were admitted with neurological symptoms and to evaluate the clinical response to vitamin B12 treatment. Materials and Methods: This study was conducted on children who had vitamin B12 deficiency presented with neurological findings from January 2014 to October 2016. Patients with serum vitamin B12 levels lower than 300 pg/mL received intramuscular or oral vitamin B12 treatment. Results: Three hundred and fifty-one patients presenting with neurologic symptoms and who had low serum vitamin B12 deficiency were analyzed. Our study population was composed mainly of adolescent age. The most common symptom with respect to age was headache. In infant patients, most common symptoms were seizure and developmental delay. Conclusion: Early diagnosis and vitamin B12 treatment are advocated to avoid long-term injury. Our study shows that patients with serum vitamin B12 levels lower than 300 pg/mL showed clinical improvement of neurological symptoms after receiving vitamin B12 treatment.

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Split cord malformation is well documented and reported in various case series and reports in the literature. The excision of bony spur in type 1 split cord malformation is challenging due to the intricate pathologic anatomy. The standard method advocated is to excise the bony spur with the help of a high-speed microdrill and a forward cutting punch. We describe a novel method of using fine-tipped slender microrongeur, which can negotiate the narrow confines harboring the bony spur and protect the adjacent hemicords. The surgical nuances are detailed and discussed.

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Background: Attention-deficit hyperactivity disorder (ADHD) is a common disorder in children, but its etiology and pathogenesis are still unclear. Aims: The aims of this study were to measure the level of serum interleukin-6 (IL-6) and tumor necrosis factor alpha (TNF-α) as markers of immune system involvement in children with ADHD, and to study their correlation with symptoms severity of ADHD. Materials and Methods: The study was conducted on 80 children diagnosed as ADHD based on the criteria adapted from the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition. Eighty healthy children of matched age and sex served as a control group. All children enrolled in the study were subjected to history taking, clinical examination, and psychometric tests. Assay for serum IL-6 and TNF-α for all patients and controls was performed using enzyme-linked immunosorbent assay. Results: The mean serum level of IL-6 was 26.11 ± 11.14 and 6.23 ± 2.52 in children with ADHD and controls, respectively. Children with ADHD showed significantly higher serum IL-6 levels than the control group (P = 0.001). Serum IL-6 showed no significant correlation with the intelligence quotient (IQ) or the Abbreviated Conners’ Rating Scale scores for parents. However, TNF-α showed no significant differences between the two groups and no significant correlation with the IQ or the Abbreviated Conners’ Rating Scale scores for parents. Conclusion: Serum IL-6 levels were significantly higher in children with ADHD compared to controls; however, the IL-6 levels did not correlate with ADHD symptoms severity. Increased IL-6 levels may contribute to the etiology of ADHD.

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Expanded dengue syndrome includes unusual or atypical manifestations of dengue fever by involving various organ systems. There have been increasing reports of dengue fever with unusual manifestations. Even though dengue virus is considered as a non-neurotropic virus, central nervous system complications have been reported. We are reporting a 4-year-old child who presented with acute dengue hemorrhagic encephalitis along with classical features of dengue infection and magnetic resonance imaging findings, suggestive of hemorrhage in the thalamus and cerebellum.

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Background: Head injury in infancy and childhood has been documented as the single most common cause of death. In India, children aged <15 years constitute 35% of the total population and contribute to 20–30% of all head injuries. In this study, we attempted to analyze the epidemiological factors, management, and outcome of traumatic brain injury (TBI). The objective of this study were to find the causes of head injury in children and its pattern of distribution in this population and to analyze the efforts required to prevent the injury and management focusing on limiting the progression of primary brain injury and minimizing secondary brain insult. Results: A total of 2714 patients with head injury were admitted at our hospital during the study period and, out of them, 508 (18.17%) were pediatric patients with age less than 18 years. Of the 508 patients, only 497 patients were included in this study. In the present study, 357 (71.83%) were males and 140 (28.16%) were females. In total, 351 cases were managed conservatively whereas surgical intervention was conducted in 146 cases (P < 0.001). In this study, the most common mode of injury was a road traffic accident (RTA) (46.88%; n=233), followed by fall from height (34.8%; n=173) (P < 0.001). It was also seen that epidural hematoma and fracture hematoma were the most common computed tomography findings in pediatric patients with head injury followed by parenchymal contusion or contusion with or without fracture followed by diffuse axonal injury. A total of 344 cases out of 497 cases were discharged with Glasgow outcome score (GOS)-5 whereas nine cases remained in a persistent vegetative state (GOS-2). Conclusion: Early intervention aimed at the primary lesion in TBI in children generally carries a good outcome, and limits as much as possible the ongoing biomechanical, physiological, and pathological sequelae post-TBI. In teenagers, the importance of proper self-care along with adequate safety gears while doing any TBI-prone activity should be emphasized.

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Multiloculated hydrocephalus (MLH) is a disease in which no single treatment has shown to be superior to others. The authors report a pediatric case of postmeningitic MLH diagnosed at the age of 3 months. It was treated with antibiotics and right ventriculoperitoneal (VP) shunt. At 10 months of age, the patient again presented with gross MLH and non-functioning shunt tube. Patient underwent endoscopic fenestration of the multiple cysts along with endoscopic-guided left VP shunt. At 1 year of age, the patient again presented with MLH with large right-sided cyst. Patient again underwent right VP shunt. The child improved in postoperative period and hence discharged. Pediatric MLH is a neurosurgical challenge. The prognosis is guarded and it is not possible to assure the parents regarding the cure of the disease. Decision of treatment options is difficult since there is no end to the treatment of many such cases. Neuroendoscopy has a definite role in MLH.

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Background: There are differences in injured mechanisms among pediatric traumatic brain injury (TBI) in developing countries. This study aimed to develop and validate clinical nomogram for predicting intracranial injury in pediatric TBI that will be implicated in balancing the unnecessary investigation in the general practice. Materials and Methods: The retrospective study was conducted in all patients who were younger than 15 years old and underwent computed tomography (CT) of the brain after TBI in southern Thailand. Injured mechanisms and clinical characteristics were identified and analyzed with binary logistic regression for predicting intracranial injury. Using random sampling without replacement, the total data was split into nomogram developing dataset (80%) and testing dataset (20%). Therefore, a nomogram was constructed and applied via the web-based application from the developing dataset. Using testing dataset, validation as binary classifiers was performed by various probabilities levels. Results: A total of 900 victims were enrolled. The mean age was 87.2 (standard deviation [SD] 57.4) months, and 65.3% of all patients injured were from road traffic accidents. The rate of positive findings in CT of the brain was 32.8%. A nomogram was developed from the significant variables, including age groups, road traffic accidents, loss of consciousness, scalp hematoma/laceration, motor weakness, signs of basilar skull fraction, low Glasgow Coma Scale score, and pupillary light reflex.Therefore, a nomogram was developed from 80% of data and was validated from 20% of data. The accuracy, sensitivity, specificity, positive, and negative predictive values of the nomogram were 0.83, 0.42, 1.00, 1.00, and 0.81 at a cutoff value of 0.5 probability. Conclusion: This study provides a clinical nomogram that will be applied to making decisions in general practice as a diagnostic tool from high specificity.

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Context: Cerebral venous sinus (sinovenous) thrombosis (CVST) in childhood is a rare, but under recognized, disorder, typically of multifactorial etiology, with neurologic sequelae apparent in up to 40% of survivors and mortality approaching 10%. Aim: The aim of this study was to enlist the patients diagnosed as CVST younger than 14 years of age and to diagnose the etiology along with radiological correlation. Settings and Design: This prospective clinical study was conducted for 2 years in the Department of Neurology, Srirama Chandra Bhanja Medical College & Hospital (SCBMCH), Cuttack, Odisha, India. Materials and Methods: All the patients were enlisted in a prestructured format with detailed clinico-radiological evaluation. Treatment was performed according to recent guidelines. Outcome after 3 months was analyzed. Ethical clearance was obtained from institutional ethics committee. Statistical Analysis: Data were statistically analyzed using IBM SPSS Statistics for Windows, version 20 (IBM Corp., Armonk, N.Y., USA). Results: The total number of patients included in the study was 30. Of them, six were neonates. The most common provocative factor was tuberculous meningitis. Phototherapy after neonatal hyperbilirubinemia was prominent cause in neonatal age group. Multiple sinus involvement was seen in most of the patients. Transverse sinus was the most common sinus to be involved. Conclusion: CVST is an underdiagnosed but important cause of stroke in childhood, occurring most often in the neonatal period. Mortality and morbidity are significant. Infections hyper coagulative disorders are the two primary associations. Magnetic resonance venography is the investigation of choice. Early diagnosis with management along with plan for secondary prevention can save from catastrophic consequences.

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Aim: Sodium tetradecyl sulfate (STS) sclerotherapy in pediatric patients is usually undertaken under sedation inside digital subtraction angiography (DSA) suite. These patients are day-care patients and need adequate sedation for small duration. We performed this study to compare propofol and dexmedetomidine as sedative agents in these patients. Materials and Methods: Seventy American Society of Anesthesiologists (ASA) physical status I patients scheduled to undergo sclerotherapy for low-flow venous malformations under sedation were randomized to be administered either dexmedetomidine (Group D) or propofol (Group P). In Group D, initially 2 µg/kg of dexmedetomidine was administered over 10min (or till attainment of a Ramsay sedation score [RSS] of 5), followed by an infusion at the rate of 0.3 µg/kg/h. In Group P, propofol 1mg/kg bolus followed by an infusion at 100 µg/kg/min was administered, titrated to an RSS of 5. We measured intraoperative heart rate, blood pressure, respiratory rate, duration of procedure, and incidence of arterial desaturation, bradycardia, and respiratory depression in the two groups. Results: All the patients in both groups completed the procedure. The mean anesthesia time was significantly longer in Group D. Intraoperative heart rates remained comparable in the two groups, whereas systolic and diastolic BP were significantly higher in Group D throughout the procedure. No patient in Group D experienced arterial desaturation, whereas five patients in Group P reported a SpO₂ of <90%. Conclusion: Both propofol and dexmedetomidine can be used for administering sedation in pediatric patients undergoing sclerotherapy for superficial venous malformations in DSA suite. Although propofol provides a rapid onset and reduced duration of action, dexmedetomidine provides reduced episodes of arterial desaturation and respiratory depression.

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Dopa-responsive dystonia (DRD) encompasses a group of clinically and genetically heterogeneous disorders that typically manifest as limb-onset, diurnally fluctuating dystonia presenting in early life and exhibits a robust and sustained response to levodopa treatment. DRD is one of the treatable dystonia syndromes of childhood. It starts with the involvement of lower limb and associated with characteristic diurnal variation. Many times it is misdiagnosed as cerebral palsy due to selective lower limb preference. We report a series of three cases of DRD which were previously misdiagnosed. The first case presented as myelopathy and other two were diagnosed as cerebral palsy. It is a treatable condition with very good response to drugs. Early diagnosis and adequate therapy can prevent from catastrophic complications.

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Background: Critically ill individuals have an increased risk of acute symptomatic seizures secondary to systemic illnesses; unrecognized or untreated seizures can quickly convert into status epilepticus, which is associated with high morbidity and mortality. Objective: The aim of this study was to determine frequency, etiology, and outcome of seizures in critical ill children admitted in intensive care unit of a tertiary care hospital. Materials and Methods: Retrospective review of medical records of all children admitted in pediatric intensive care unit (PICU) of the Aga Khan University from January 2016 to December 2018 and who had a new-onset seizure irrespective of underlying diagnosis was carried out after ethical review committee approval. Data were collected on a structured proforma; it included demographic information as well as relevant clinical and outcome information. The data were analyzed on Statistical Package for the Social Sciences (SPSS) software program, version 19.0. The descriptive statistics frequency and percentage was computed for qualitative variable. Mean and standard deviation were computed for quantitative variable, and univariate analysis was performed. Results: During the study period, a total 2053 patients were admitted in the PICU. One hundred six (5%) had seizure. Sixty-three (59.5%) were males. Meningitis 21 (20%), sepsis 21 (20%), complicated pneumonia 18 (17%) were the major primary diagnosis in these children. Mean age of the study population was 75 months (standard deviation [SD] ± 54.4) and 72 (68%) were <5 years of age, whereas 63 (59.5%) were males. The seizures lasted >10min in 10 (10%) and were associated with high had neurological deficit (P = 0.001). We did not observe any correlation with electrolyte imbalance, renal failure, need of ventilator support with duration of seizure, and type of seizure (P > 0.005). Conclusion: Infection was the most common etiology associated with a new-onset seizure in children admitted in our PICU. Seizures lasting for >10min were observed with high neurological deficit. We did not find any association of mortality with seizure duration.

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Objective: Myelomeningocele is the most severe and the most frequent form of spina bifida. Most of the myelomeningocele patients undergo operations in new-born age. In terms of life quality and rehabilitation, follow-up’s of these patients in the growth and development period after the operation is critical. In our study, our aim is to emphasize the correlation of SEP results with MRI results and clinical features of the myelomeningocele patients. Materials and Methods: In our study, we included 36 patients who had undergone myelomeningocele operation and have been followed-up in Istanbul Bilim University Florence Nightingale Hospital, Spina Bifida Research and Treatment Centre. Posterior tibial nerve SEP was performed on each patient and neurological examinations were done in the same session. Results were compared with clinical functional lesion levels, levels of fusion defect and ambulation levels. In order to evaluate SEP results, we used age-related reference values from Boor et al.’s study in 2008. Patients were grouped as normal, unilaterally prolonged, bilaterally prolonged, unilaterally lost, and bilaterally lost. Results: The correlations of posterior tibial nerve SEP results were significant with ambulation levels (r = 0.428, P < 0.01), clinical functional lesion levels (r = 0.477, P < 0.01) and fusion defect levels (r = −0.528 P < 0.05). The lumbar SEP results were only significantly correlated with functional lesion levels (r = 0.443 P < 0.05). Conclusions: Radiological studies are insufficient when evaluating the functionality of the central nervous system. To fully evaluate the functionality and watch the neurological development with accuracy, especially in operated patients, electrophysiological studies should be an indispensable part of myelomeningocele follow-ups.

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