Subacute sclerosing panencephalitis (SSPE) is typically observed in school-going children, adolescents, and young adults. Herein, we report a 3-year-old boy of SSPE and systematically review all such reported cases (age ≤3 years). We searched PubMed database on June 16, 2020 with “English language & Human only” restrictions. Eligible publications were screened and retrieved by three authors (NK, SP, and RU). Variables like author, year of publication, country, age of onset, sex, duration of illness, immunization, history of measles, clinical presentation, neuroimaging, treatment, and outcome were extracted. Neurological outcome was assessed in terms of improvement, static course or death. The defined search criteria resulted in 791 manuscripts (1951 to June 16, 2020). Only 47 manuscripts were eligible for data extraction. Seventy cases were retrieved. The mean age of onset was 26.34 months. The M:F ratio was 3.2:1. Turkey (19 cases), India (10 cases), and Japan (10 cases) collectively comprised more than half cases of SSPE. Only six children were immunized, 17 have no measles immunization, and the rest 47 had insufficient immunization records. Preceding history of measles was present in 27 cases. Most patients presented in stage II/III with altered sensorium, myoclonic jerks, and seizures. The most common reported outcome was death (18/70) followed by vegetative state (10/70). The absence of measles vaccination is frequently associated with childhood SSPE. Early measles vaccination may prevent SSPE in toddlers. Perinatal measles infection results in short onset latency and fulminant course. Outcome was not good in reviewed age group of ≤3 years.

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Acute disseminated encephalomyelitis (ADEM) occurs in children commonly following infections and vaccination. Central demyelinating disorder such as ADEM following snake bite is rarely reported. We present a case of an 11-year-old boy who developed altered sensorium and quadriparesis suggestive of ADEM following a cobra bite. The role of early diagnosis with a high index of clinical suspicion and optimum neuroimaging modality in recovery is highlighted.

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Dandy–Walker malformation (DWM) is a common congenital posterior fossa anomaly, however, its association with concomitant bilateral inner ear anomalies has been rarely reported so far. We present such a case that showed characteristic imaging features of DWM along with uncommon vestibulocochlear anomalies like cochlear hypoplasia and Internal Auditory Canal atresia.

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Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are postinfectious, inflammatory, and neurological disorders involving the peripheral and central nervous system, respectively. Although the clinical features are distinct, they share a common pathogenesis. Both the disorders are not uncommon in children. Concurrent GBS and ADEM occurring simultaneously is a rare entity. We are reporting a case of a 5-year-old boy presenting with acute-onset ascending paralysis with irritability, hyperreflexia, and positive Babinski sign. This patient was diagnosed as having both GBS and ADEM. The CSF albumin-cytological disassociation, electrophysiological diagnosis, and MRI of brain supported the concurrent present of the two disorders.

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Objective: To describe the long-term outcome in children with acute ischemic stroke (AIS) from a tertiary-care center. Materials and Methods: Prospective, observational study of children diagnosed with AIS between the ages of six months and 12 years and who completed two to five years of follow-up. Results: Forty-nine children (35 boys, 14 girls) were included. The mean age at onset of stroke was 35.6 ± 31.5 months (6–108 months). A majority of children had presented with hemiparesis (93.8%). Risk factors were identified in 65.4% of cases. Moyamoya vasculopathy (28%), iron-deficiency anemia (24.4%), and trauma (12.2%) were the most common risk factors. The majority of infarcts were cortical (32.6%), followed by combined cortical and subcortical (30.6%), and isolated subcortical (26.5%). At follow-up, recurrent stroke (24.4%), residual epilepsy (24.4%), and motor disability requiring support for ambulation (6%) were noted. The mean general developmental score (GDS) was 71.2 ± 18.7. Global developmental delay in 46.9% and delayed social intelligence in 22.4% was noted. On subset analysis, children had physical (42.9%), cognitive (34.7%), communication (30.6%), adaptive-behavior (26.5%), and social–emotional (22.4%) delay. Predictors of good cognitive outcome were younger age at onset (OR 0.964, P = 0.006), isolated subcortical infarcts (OR 26.386, P = 0.028), and absence of seizures at presentation (OR 0.197, P = 0.044). Predictors of poor social quotient were seizures at onset (OR 0.049, P = 0.012) and recurrent stroke (OR 0.055, P = 0.012). Conclusion: Neurodevelopmental problems and epilepsy occur in a significant proportion of children with AIS in the long term. Good outcomes are predicted by the younger age of onset, subcortical infarcts, absence of seizures, and absence of recurrence of stroke.

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Frontonasal encephalocele is a rare anatomical variant of the meningoencephalocele. Peri-operative anesthesia concerns involve anticipated difficult mask ventilation as well as surgical complications such as compression and rupture of meningoencephalocele. The compression of encephalocele is associated with raised intracranial pressure (ICP) and even rupture of overlying skin leading to cerebrospinal fluid leak, hemorrhage, exposure of underlying frontal lobe, meningitis, seizures, and even death. We report a case of 6-year-old female presenting with rare variant of frontonasal encephalocele for bifrontal craniotomy, excision of encephalocele, and repair. Difficult mask ventilation was anticipated, and patient airway was managed with use of size 4 anatomical mask.

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Introduction: The position of the tonsillar tip in relation to the foramen magnum varies from individual to individual, even after excluding all predisposing factors that might alter the tonsillar tip position; the mere presence of the tonsillar ectopia should not be misinterpreted as Chiari malformation. Aim: Our aim is to establish the normal range of the tonsillar tip position and the incidence of tonsillar ectopia and its relation to age and sex in asymptomatic Indian children with structurally normal MRI brain imaging. Materials and Methods: We have conducted a retrospective data collection comprising 301 structurally normal MRI brain images done for children younger than 14 years, in which we measured the level of the tonsillar tip from the foramen magnum by taking the Mc Rae line as constant. Measurements were taken in the right and left parasagittal sections of T1-weighted MRI brain images by using pre-existing software. Statistical Analysis Used: Data analysis was done by SPSS v 25.0; all P values <0.05 were considered statistically significant. Results and Conclusion: The incidence of tonsillar ectopia in Indian children was about 13.79%, and the median tonsillar position was 3.2 mm above the foramen magnum with a median range of 5.2 mm above to the level of the foramen magnum (0).

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An eight-year-old girl presented to the outpatient department with a history of decreased hearing from the right ear, bloody ear discharge, and facial deviation to the left side. On clinical examination, she was afebrile with conductive right-sided hearing loss. With a primary diagnosis of unsafe type of chronic suppurative otitis media in mind, computed tomography scan of the temporal bone was performed which showed an aggressive destructive lesion in the petrous temporal bone. Magnetic resonance imaging was then performed to look for soft tissue extension and vascular involvement. Based on the clinical and imaging findings, she was diagnosed as a high-grade neoplasm of the right temporal bone, such as rhabdomyosarcoma. Histopathology finally confirmed the diagnosis of an embryonal type of rhabdomyosarcoma of the middle ear cleft.

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Introduction: Worster Drought syndrome (WDS) is a type of cerebral palsy that affects the muscles around the mouth and throat. This causes problems with swallowing, feeding, talking, and dribbling. Although the impairments are predominantly motor, a range of cognitive, behavioral, and seizures also exist. These features point towards the involvement of the perisylvian area of the cerebral cortex. Purpose of the Study: In WDS, the predominant impairment is a profound expressive communication problem and feeding difficulties; hence, it is under-recognized and even under-reported. Besides, these children have a specific phenotype which when recognized early can make a significant difference in management. This study aims to know the etiology and profile of WDS. Materials and Methods: This is a prospective cohort study in a tertiary care hospital from January 2018 to December 2019. All patients who met the absolute criterion for the diagnosis of WDS were included. A detailed history, clinical examination findings, relevant investigations like CT/MRI brain, and EEG were recorded in a predesigned proforma, and results were analyzed. Results: A total of 83 children (M:F = 52:31) presented within the age group of 2 years to 18 years. Seventy (84.33%) of them were delivered by a normal vaginal route. All children had global developmental delay, expressive speech delay, and spasticity. Other features were undernutrition 75(90%), drooling 66(79.51%), seizures 44(52.60%), and prolonged feeding 42(50.60%). Birth asphyxia was present in 72 (86.70%) cases, but neuroimaging was suggestive of perisylvian gliosis in 80 (96.38%) cases. Conclusions: WDS should be considered in children with cerebral palsy if mild motor delay with a predominant expressive language delay is present without hearing impairment and autistic features. In India, birth asphyxia is the most common cause.

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Introduction: This study was conducted to provide detailed information about clinical characteristics and short-term treatment outcome of childhood headache. Materials and Methods: This prospective observational study was done over a period of 15 months (January 2013 to March 2014) at a rural tertiary care center in North India. Detailed history, clinical examination, specialty review, and follow-up details were maintained on pretested structured proforma. Final diagnosis of headache type was made as per International Classification of Headache Disorders, 2nd edition. Results: Out of 100 (45 boys) children aged 8–18 years, 52% were diagnosed with migraine, 23% with tension-type headache, and 25% with secondary headache. Diffuse headache was the commonest (41%), and photophobia, phonophobia, and dizziness were the commonest symptoms in all headache subtypes. Sixty-five percentage of migraine headache were triggered by exertion. Lack of sleep and anxiety were triggers in most (65%) of tension type headache. Of 52 migraine children, 21 were started on prophylaxis for migraine and 14 of them reported significant improvement. Secondary causes for headache were found in 25% of children and half of them were having refractive errors. Conclusions: Results of the study show migraine being the commonest type of headache in children followed by secondary headache. This study also highlights the need for long-term follow-up of childhood headache.

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Background: Ventriculoperitoneal shunt (VP shunt) surgery is one of the most common procedures being performed in the pediatric age group in neurosurgical centers worldwide. Although considered a simple surgery, the complications have made it one of the most difficult procedures to manage in the long term. In this study, we describe our experiences in managing the unusual complications of this common procedure in children. Materials and Methods: This retrospective study enrolled 13 pediatric cases having unusual complications related to VP shunt who presented to us from August 2013 to August 2019. All the cases were evaluated for their demographic profile, type of complications, possible pathophysiology, outcome, and management. We had used only spring-valve-based Chhabra VP shunt in all of our cases. Results: The age group ranged between 2 and 18 years, with a male to female ratio of 9:4. Anal extrusion of distal shunt tube was observed in four cases, and two cases each of urethral and oral extrusion were also seen. Two cases developed extrusion of shunt tube through the anterior abdominal wall and one case developed umbilical fistula. Multiple extradural hematoma and intraventricular bleed with partial shunt coil were also seen in each of the cases, respectively. All cases were managed as per need. Conclusion: Unusual complications of VP shunt surgery are increasingly being observed nowadays. Prompt diagnosis and treatment of these complications result in an excellent outcome.

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Objective: Stroke is relatively rare in children, but it can lead to significant morbidity and mortality. Understanding the risk factors related to stroke will optimize the outcomes in children. Published cohorts of children with stroke recurrence rates are variable. This study has been done to determine the risk factors, clinical features, and outcomes of recurrent pediatric stroke in a developing country. Materials and Methods: We enrolled 98 children with stroke: 16 children were excluded due to incomplete investigations and were lost to follow-up. This study was conducted in a tertiary care hospital in Dhaka, Bangladesh from January 2017 to December 2019. Detailed investigations regarding stroke, physical examination, and targeted investigations were done. Results: We studied 82 patients: Among them, 52 had their 1st attack, and 30 had recurrent attacks of stroke. The age range of onset of stroke was 4.78 ± 3.90 and 5.30 ± 4.25 in the first and recurrent stroke, respectively. In this study, 36.5% of the enrolled children presented with a recurrence of stroke, whereas the rest presented with the first episode of stroke. The most common cause of recurrent stroke was intracranial vasculopathy, with the most common being vascular narrowing and moya moya disease (MMD). Protein C deficiency was observed more frequently in recurrent stroke. No significant difference was found in the risk factors of the first and recurrent stroke. During follow-up, patients with recurrent stroke developed cognitive decline, epilepsy, and speech disorder more frequently. Conclusion: In children with recurrent stroke vasculopathy, particularly MMD and protein C deficiency were observed more frequently. Moreover, in this group, sequelae-like cognitive dysfunction, speech disorder, and epilepsy were observed more frequently.

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A meticulous clinical examination is indispensable in the diagnosis of neurometabolic disorders. We describe an 11-month-old girl with the classical “Hurler phenotype” who was referred to us with a diagnosis of mucopolysaccharidosis. But on “eyeing” the extensive Mongolian spots on the back and the cherry red spot in the eye, GM1 gangliosidosis were suspected and confirmed by enzyme assay. Examination of the fundus and the presence of extensive Mongolian spots can provide valuable clues to the diagnosis of metabolic disorders.

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Background: Autism is a neurological disorder with either genetic or environmental component. Autism generally presents changes of intestinal permeability to produce alteration of metabolism in the gastrointestinal tract. The intestinal macrobiota produces metabolites, opioid-like peptides, that show properties experimentally associated with autism. The aim of this study is to understand the cause of intestinal permeability’s alteration. Materials and Methods: We determined intestinal MUC2 on stool samples of 12 patients and healthy controls with Fecal Mucin Assay and analyzed the results with Mann–Whitney U-test calculator. Results: The results of the dosage of MUC2’s concentration in autistic people decrease when compared with those of healthy control groups: this result is statistically significant: the p-value is 0.00124. Discussion: The results show an increase of MUC2, perhaps due to genetic origin. We hypnotize a probable over expression on highly hypoglycosylated MUC1. All this prevents a regular linkage of MUC2 to MUC1, so as to form many channels in mucosa. The free MUC2 relies on themselves and joins together with hydrogen bridge bonds, leaving the highly glycosylated end parts to become the starting point for abnormal growth of the bacteria, which in turn produce opioid such as peptides, that pass into the channels of the mucosa layer.

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Objectives: The towel test is considered as a reliable clinical examination finding to test the elbow flexion in Brachial plexus birth palsy. A mature central nervous system makes this test positive at 6 months’ time and those who failed the test require microsurgical exploration and nerve repair/transfers for upper limb functions. Not all infants pass in this test. Majority of the children develop a clear hand preference at 6 months of age. We hypothesize the validity of the towel test in brachial plexus birth palsy children between 6 and 9 months of age and the possibility of mother and infant handedness in the false-negative tests. Materials and Methods: A retrospective analysis conducted between 2015 and 2019 about the utility of “towel test” involving 12 boys and 11 girls was analyzed and interpreted. The side involved in these infants and the mother’s handedness was also noted. Based on the age, side, gender, and mother-handedness, the results of towel tests were statistically interpreted. Results: The mean age of the infants involved was 6.6 months (range 6–9 months). Twenty children (87%) had positive tests. Three (13%) infants (male = 2; female = 1) had false-negative test. There was no action on the normal side to remove the towel but weak movements in the affected upper limb persisted to try and remove the towel. The left-hand mother’s infant had false-negative towel test, which was found statistically significant (P < 0.01). Conclusion: Clinicians should know that false negativity may coexist while using the towel test and handedness could be a possibility in them. In such conditions, additional tests can be used to assess the ongoing motor recovery in brachial plexus birth palsy children between 6 and 9 months of age.

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Background: Alobar holoprosencephaly is a rare disorder of brain development. Most of the cases have facial abnormalities and the survival of such children is usually not prolonged. Case Description: We are reporting a 2-month-old female child presented with seizures and delayed developmental milestones. Interestingly, she has no facial abnormality which is quite unusual. The management of such children is difficult and individualized. Conclusion: The counseling of the parents should be done regarding the severity and outcome of such congenital disease and also the importance of the antenatal check-up during a future pregnancy.

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A 2-year-old girl with recent chickenpox infection was noted to have a fixed and dilated right pupil. Diluted 0.1% pilocarpine eyedrop test was successful in constructing the dilated right pupil. Cranial nerve examination and computer tomography brain scan were otherwise normal. We report this case as internal ophthalmoplegia, or post-viral Adie’s pupil, which appears to be extremely rare.

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Caniopharyngiomas pose significant surgical challenge because of their location and close proximity to major neurovascular structures. Early and delayed vascular complications associated with Craniopharyngioma and its surgery have been documented. Various mechanisms have been postulated for the origin of aneurysms in Craniopharyngioma. Though the surgery and minor injuries to the vessel wall might predispose the aneurysm formation, exact mechanism and relation is lacking. We report a case of a psedoaneurysm of the distal ACA AT A_2`-A₃ junction in a case of cystic Craniopharyngioma who presented with rupture, 2 weeks after primary surgery. Insight into the probable causes of aneurysm formation and its subsequent rupture and management have been described.

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Immunotherapy is an important treatment modality for several pediatric neurology conditions. The immunosuppressive therapies may predispose to a heightened risk of infection and fatal consequences. The paper aims to examine the immunological effects of various commonly used immunomodulators and their associated risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection. Immunotherapies may exhibit different effects at the different stages of coronavirus disease 19 (COVID-19) pathogenesis. Conventional immunosuppressive therapies appear to produce less favorable results or may aggravate the risk of infection, especially during the primary response phase. Whereas immunomodulators such as inhibitors of pro-inflammatory cytokines, antagonists of complement activity, and those drugs which decrease viral-neutralizing antibodies levels seem to have beneficial effects during the secondary hyper inflammation phase of infection. However, adequate clinical data is yet to be available for most of the immunotherapies. Based on the emerging evidence, the authors attempt to draw some conclusions of practical importance on the applications of different immunomodulators for children with pediatric neurological disorders in the COVID-19 pandemic.

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