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Journal of Pediatric Neurosciences

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https://www.readbyqxmd.com/read/30271479/urethral-protrusion-of-the-distal-end-of-shunt
#1
Shailendra D Anjankar
No abstract text is available yet for this article.
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271478/primary-pediatric-intracranial-neuroblastoma-a-report-of-two-cases
#2
Ajit Mishra, Manish Beniwal, Bevinahalli N Nandeesh, Dwarakanath Srinivas, Sampath Somanna
Neuroblastoma is the most common pediatric extracranial solid malignancy. It has a high propensity for spread, especially to the bones and lymph nodes. The involvement of central nervous system is uncommon and most of the cases are restricted to the spine. Primary intracranial neuroblastoma is extremely rare and very few cases have been described in the available literature. We report two cases of primary intracranial neuroblastoma in pediatric age group.
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271477/lipid-storage-myopathy-with-ketonuria-a-case-of-fatty-acid-oxidation-related-myopathy-and-encephalopathy-due-to-multiple-acyl-coa-dehydrogenase-deficiency
#3
Sadanandavalli R Chandra, Rita Christopher, Gayathri Narayanappa, Nitin C Ramanujam, Pavan Katragadda, Akshata Huddar, Shreyashi Jha
Encephalopathy and Myopathy in children of varying ages can be due to variety of causes including Mitochondrial diseases, metabolic diseases like renal tubular acidosis, storage diseases as well as fatty acid oxidation (FAO) disorders. FAO related disorders have variable clinical presentation and manifest in different ages. They may present with hypoglycemia, effort intolerance, multi organ involvement with or without ketonuria. High degree of suspicion and appropriate investigations are mandatory for diagnosis...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271476/rare-and-treatable-cause-of-early-onset-refractory-absence-seizures
#4
Gajanan A Panandikar, Sangeeta H Ravat, Rahil R Ansari, Karan M Desai
Glut-1 transporter deficiency syndrome (GLUT1-DS) is a rare disorder caused by the mutation in SLC2A1 gene, which results in impaired glucose transport into the brain. It has a broad spectrum of phenotypic presentation ranging from cognitive decline, microcephaly, and refractory seizures to complex movement disorder. Recognition of this disorder is necessary as it is refractory to antiepileptic drugs (AEDs) and responds significantly to ketogenic diet. We report a case of 7-year-old girl who presented with paroxysmal eye movements in infancy with early-onset absence epilepsy (EOAE), which worsened in early morning and on fasting and was found to be refractory to four AEDs...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271475/autosomal-recessive-spastic-ataxia-of-charlevoix-saguenay-a-turkish-child
#5
Faruk Incecik, Ozlem M Hergüner, Atil Bisgin
Autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is caused by mutations of the SACS gene, characterized by late-infantile-onset spastic ataxia and other neurological features. ARSACS has a high prevalence in northeastern Quebec, Canada. Recently, several ARSACS cases have been reported from outside Canada. We report typical clinical and neuroimaging features in a Turkish child, which confirmed genetic diagnosis of ARSACS.
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271474/spontaneous-asymptomatic-migration-of-ventriculoperitoneal-shunt-into-thoracic-cavity-a-case-report-with-review-of-literature
#6
Hinglaj Saha, Shib Shankar Burman, Kaushik Saha, Dipak Ghosh
Intrathoracic migration of ventriculoperitoneal shunt is a rare complication and usually complicated with pneumonia or cerebrospinal fluid hydrothorax, which leads to diagnosis and early interventions. Very rarely patient remains asymptomatic. Here is a case report of a 6-year-old boy with a rare asymptomatic intrathoracic migration of ventriculoperitoneal shunt.
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271473/glutaric-aciduria-type-1-with-microcephaly-masquerading-as-spastic-cerebral-palsy
#7
Indar K Sharawat, Lesa Dawman
Glutaric aciduria type 1 (GA-1) is an autosomal-recessive disorder caused by the deficiency of the mitochondrial enzyme glutaryl-CoA dehydrogenase. A 13-month-old boy presented with microcephaly, developmental delay, and progressive spasticity and was being treated as spastic cerebral palsy, later on had loss of developmental milestones after acute episode of illness at 12 months of age. The magnetic resonance imaging of brain revealed widened Sylvian fissure, hyperintensities in bilateral globus pallidus, and bilateral frontoparietal atrophy along with white matter loss...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271472/extensive-mongolian-spots-in-a-hypotonic-infant-with-gm1-gangliosidosis
#8
Suprabhat Ranjan, Pratap K Patra
Mongolian spots (MS), also known as dermal melanocytosis, are the most frequently seen birthmarks and generally present as large, blue or blue-black macules or patches, mostly limited to the lumbosacral region. They are considered as a benign manifestation; however, extensive MS can be a manifestation of certain underlying inborn errors of metabolism including GM1 gangliosidosis.
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271471/pyogenic-meningitis-complicated-with-extensive-central-nervous-system-vasculitis-and-moyamoya-vasculopathy
#9
Sumeet R Dhawan, Jitendra K Sahu, Sameer Vyas, Sunit C Singhi, Pratibha D Singhi
Meningitis is an uncommon complications of head trauma. Vasculitis in bacterial meningitis is seen in 9%-25% of adults. Neurological deficits in bacterial meningitis are seen in about one-third of children. Isolated cranial nerve palsies are common, whereas major deficits such as hemiparesis and quadriparesis are rare. We describe a case of a 7-year-old boy who had post-traumatic meningitis complicated with quadriparesis and severe vasculitis of bilateral anterior and posterior circulation with moyamoya vasculopathy...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271470/a-rare-cause-of-refractory-epilepsy-posterior-periventricular-nodular-heterotopia
#10
Meltem C Direk, Mustafa Komur, Anıl Ozgur, Cetin Okuyaz
A 15-year-old male patient presented to our outpatient clinic with drug-resistant seizures. Magnetic resonance imaging of the brain showed bilateral posterior nodular heterotopia and left cerebellar dysgenesis. The patient was diagnosed with cortical developmental malformation and medically refractory epilepsy. The filamin A gene mutation was negative. Posterior periventricular nodular heterotopia is a rarer and a more different entity from classical periventricular nodular heterotopia with no gender difference and negative filamin A gene mutation...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271469/new-onset-persistent-refractory-hypertension-after-medulloblastoma-excision-in-children-an-indicator-of-poor-prognosis-a-case-series
#11
Barkha Bindu, Ranadhir Mitra, Gyaninder P Singh, Manoj Phalak
Hypertension in the clinical setting of posterior fossa tumors is a known entity and occurs due to medullary compression by the tumor. Such hypertension usually responds to tumor excision. Postoperative hypertension occurring after posterior fossa tumor excision has been attributed to brain stem edema in a single report earlier, which resolved without any intervention. Here, we report two pediatric patients who developed new onset refractory, persistent postoperative hypertension after medulloblastoma excision, and discuss possible causes and the prognostic significance of this condition...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271468/cornelia-de-lange-syndrome-a-case-series-from-a-resource-limited-country
#12
Indar K Sharawat, Lesa Dawman
Cornelia de Lange syndrome is a rare genetic condition with developmental disorder and malformation affecting multiple systems. To describe the clinical and laboratory details and outcome of the children diagnosed with Cornelia de Lange syndrome, we retrospectively studied six cases who presented to our hospital between the years 2013 and 2015. Almost all had developmental retardation, with recurrent respiratory tract infections, and feeding difficulties. Synophrys with long curly eyelashes with low anterior and posterior hairline was present in all the children...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271467/neurocutaneous-melanosis-with-bilateral-temporal-lobe-lesions-without-leptomeningeal-enhancement-a-distinct-entity-or-subtype
#13
Sanjay Kumar, Subir Dey, Yayati Pimpalwar, Akhilesh Rao
Neurocutaneous melanosis (NCM) is a rare congenital disorder. Most of the cases described in literature for this entity have involvement of the leptomeninges and other structures of brain such as brain stem, temporal lobes, and spinal meninges and no involvement of leptomeninges and presence of lesions in bilateral temporal lobes. NCM without the involvement of leptomeninges should be considered a distinct entity as the prognosis is favorable as compared to cases with leptomeningeal involvement who develop early hydrocephalus and multiple cranial nerve palsies...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271466/spontaneous-pneumocephalus-secondary-to-leaking-myelomeningocele
#14
Raja Krishnan Kutty, Sunilkumar B Sreemathyamma, Jyothish Laila Sivanandapanicker, Anilkumar Peethambaran
Myelomeningocele is one among the most common forms of spinal dysraphism. We report a newborn male child with leaking myelomeningocele who presented with apneic spells. He underwent a magnetic resonance imaging of the neuraxis, which revealed Chiari malformation and severe hydromyelia along with pneumocephalus. He was planned to undergo urgent repair of the neural tube defect but his parents were not willing for surgery. We report this case as spontaneous pneumocephalus secondary to open neural tube defect, which is very rare and sparsely reported...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271465/outcome-of-ventriculosubgaleal-shunt-in-the-management-of-infectious-and-non-infectious-hydrocephalus-in-pre-term-infants
#15
Raja K Kutty, Sunilkumar B Sreemathyamma, Paresh Korde, Rajmohan B Prabhakar, Anilkumar Peethambaran, Gnanaseelan K Libu
Background: Hydrocephalus in premature infants is an onerous disease. In such situations, choosing the best option for cerebrospinal fluid (CSF) diversion is difficult. Ventriculosubgaleal shunt is an effective method of temporary CSF diversion in such situations. In this retrospective study, we compare the outcome of ventriculosubgaleal shunt in premature infants with hydrocephalus of infectious and noninfectious etiology. Materials and Methods: All premature children with hydrocephalus secondary to various etiologies who underwent ventriculosubgaleal shunt were studied...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271464/abnormal-migration-and-extrusion-of-abdominal-end-of-ventriculoperitoneal-shunt-an-experience-of-eight-cases
#16
Ashish Chugh, Sarang Gotecha, Gaurav Amle, Anil Patil, Prashant Punia, Megha Kotecha
Background: Ventriculoperitoneal (VP) shunt is commonly used in the treatment of hydrocephalus. Migration and extrusion of the distal end of the VP shunt are relatively rarely occurring complications. Aim: To retrospectively analyze patients with extrusion of the abdominal end of ventriculoperitoneal shunts and evaluate the possible etiology and outcome. Settings and Design: All patients presenting with extrusion of lower end of the shunt were included...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271463/prospective-study-of-diphtheria-for-neurological-complications
#17
Prem L Prasad, Preeti L Rai
Aim: To study the spectrum of neurological complications of diphtheria, timing of onset with respect to respiratory disease, and pattern of recovery. Settings and Design: Prospective, observational, hospital-based study conducted in tertiary care hospital. Materials and Methods: Twenty-eight cases of diphtheria with neurological complications were admitted during the period of study. Demographic profile, age, gender, status of immunization, past history stressing on the severity of the respiratory disease, and complaints regarding diverse complications of diphtheria were recorded...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271462/prevalence-of-unrecognized-autism-spectrum-disorders-in-epilepsy-a-clinic-based-study
#18
Monica Juneja, Suchit Gupta, Abhinav Thakral
Objective: To assess prevalence of unrecognized autism spectrum disorders (ASDs) in children with epilepsy using Diagnostic and Statistical Manual IV (DSM-IV) criteria and to evaluate factors affecting it in this population. It was a cross-sectional study conducted at a teaching hospital. It included randomly selected 106 children in the age 4-12 years with epilepsy, and without any structural anomaly identifiable on computed tomography/magnetic resonance imaging. Children already diagnosed with ASD were excluded...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271461/electroclinical-pattern-and-epilepsy-evolution-in-an-infant-with-miller-dieker-syndrome
#19
Raffaele Falsaperla, Simona Domenica Marino, Silvia Marino, Piero Pavone
Aim of the study: To evaluate the electroclinical course and the correlation Electroencephalographic (EEG) pattern and epileptic seizures in an infant with Miller Dieker Syndrome (MDS) during the first year of life. Materials and Methods: MDS was diagnosed in the infant soon after birth and followed up from six months of life to one year, at the Department of Pediatrics, General Pediatric Operative Unit, Policlinico Vittorio Emanuele, University Hospital, XCatania, Italy, with clinical and serial EEG recording...
July 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/30271460/intraspinal-anomalies-in-early-onset-scoliosis-current-concepts
#20
REVIEW
Manoj Singrakhia, Nikhil Malewar, Ajit Jangle
Early onset scoliosis (EOS) is deformity of the spine below the age of 5 years. Children with EOS are at risk of impaired thoracic cage development and pulmonary maturation. Initial evaluation consists of determining the etiological cause for EOS, i.e., congenital, neurogenic, idiopathic, or syndromic. The advent of magnetic resonance imaging in recent times has lead to increased awareness of neurogenic causes leading to EOS. Evaluation of spinal cord anomalies in EOS is very important as early diagnosis and treatment can help in deformity stabilization and regression...
July 2018: Journal of Pediatric Neurosciences
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