Giant terminal myelocystocele: A case report
Gaurav Varshney, Deepak K GuptaJournal of Pediatric Neurosciences 2020 15(3):286-289Myelocystoceles, also known as syringoceles, syringomyeloceles, and lipomeningomyelocystoceles, represent 5% of all lumbosacral skin-covered masses. Terminal myelocystocele (TMC) is a rare variant of spinal dysraphism. At times, patients with TMCs can present with a huge lumbosacral mass (giant TMC). A 14-month-old female child presented with progressive increasing swelling in the lumbosacral region (35 cm × 35 cm × 30 cm) since birth with inability to move both lower lim...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Gaurav Varshney Deepak K Gupta Source Type: research
A rare case of ataxia-telangiectasia-like disorder with MRE11 mutation
We report a boy who presented with mild cerebellar ataxia and dystonia with cerebellar atrophy on brain imaging. Clinical exome sequencing showed compound heterozygous variants in MRE11 gene. He was diagnosed as ATLD, which has not been reported in Indian subcontinent so far. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Rohan R Mahale Nishanth Reddy Pavagada Mathuranth Pooja Mailankody Hansashree Padmanabha Chandra Sadnavalli Retnaswami Source Type: research
Macrophagic myofasciitis: A report of two south Indian infants
Vykuntaraju K Gowda, Varunvenkat M Srinivasan, Yasha Muthane, Gayathri NarayanappaJournal of Pediatric Neurosciences 2020 15(3):279-282Macrophagic myofasciitis is a rare inflammatory myopathy characterized by peri-fascicular macrophage infiltration without muscle necrosis. Here we report two children presented in the early infancy. Case 1: a 5-month-old girl presented with lack of neck control and floppiness. On examination, generalized hypotonia, absent deep tendon reflexes, and motor power of 2/5 (Medical Research Council grade) were observed. Case 2: a 17-day-old boy presented with poor feeding, tachypnea, and floppines...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Vykuntaraju K Gowda Varunvenkat M Srinivasan Yasha Muthane Gayathri Narayanappa Source Type: research
Hemiconvulsion-hemiplegia-epilepsy syndrome: A case series
We present four cases with age between 6 months and 3 years diagnosed with HHE syndrome. Two patients were lost to follow-up; other two cases had severe developmental delay and refractory epilepsy. An early diagnosis, a good seizure control, and a better understanding of the underlying mechanisms of HHE are needed to improve the outcome of this condition. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Harshit Bhargava Deepak Dwivedi Source Type: research
Hypomyelination and congenital cataract: Three siblings presentation
We present the findings of three siblings who applied to us with the same clinical features. These patients were referred to our clinic due to the presence of bilateral congenital cataract and progressive neurological impairment with peripheral neuropathy. Brain magnetic resonance imaging (MRI) showed diffuse hypomyelination, whereas neurophysiological studies showed sensorimotor peripheral polyneuropathy. Cases with hypomyelination in MRI represent the largest group of undiagnosed diseases among patients with leukoencephalopathies. To diagnose cases with peripheral neuropathy, their clinical and neuroradiological findings...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Zeynep Selen Karalok Esra Gurkasb Kursad Aydinc Serdar Ceylaner Source Type: research
Intracranial tubercular subdural empyema: A case report and review of literature
Vinod K S Gautam, Sambhav UpadhyayJournal of Pediatric Neurosciences 2020 15(3):266-269Tuberculosis (TB) is a major health problem in developing countries. Approximately 1 million children became ill with TB in year 2016. Neurotuberculosis is associated with high mortality and morbidity. Intracranial tubercular subdural empyema is extremely rare in pediatric population. Authors report a case of 13-year-old male child who had presented with recurrent seizures and features of raised intracranial pressure. Neuroradiology of skull revealed intracranial subdural empyema. He was managed effectively with burr hole evacuation of t...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Vinod K S Gautam Sambhav Upadhyay Source Type: research
Arrested hydrocephalus complicated by growing skull fracture - A case report with review of literature
Ashok Kumar, Gaurav Jaiswal, Vivek K Kankane, Pavan K Kumar, Tarun K GuptaJournal of Pediatric Neurosciences 2020 15(3):261-265Growing skull fracture (GSF) is an extremely uncommon entity and accounts for less than 1% of the skull fractures. This type of fracture is commonly seen in children of less than 3 year of age and two third of them occur in less than one year of age. Occurrence of GSF is higher in infancy and early childhood because of rapid growth of brain and skull take place in initial two year of the life. Dural tear is most common etiological factor that leads to growing skull fracture. Growing skull fr...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Ashok Kumar Gaurav Jaiswal Vivek K Kankane Pavan K Kumar Tarun K Gupta Source Type: research
Giant anterior fontanel encephalocoele: A rare case report and review of literature
Bikash R Behera, Sanjib Mishra, Manmath K Dhir, Jyotirmayee Biswal, Siba N BhujabalJournal of Pediatric Neurosciences 2020 15(3):257-260An encephalocoele is a spectrum of cranial dysraphism caused due to defective closure of neural tube during early embryonic life, leading to formation of a cerebrospinal fluid (CSF)–filled sac containing brain tissue and meninges, all herniated through a bony defect of the skull. Anterior encephalocoeles, though rare in the Western world, are relatively common in southeast Asia, including some parts of India. Among anterior encephalocoeles, fronto-ethmoidal type is the most co...
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Bikash R Behera Sanjib Mishra Manmath K Dhir Jyotirmayee Biswal Siba N Bhujabal Source Type: research
Levetiracetam versus fosphenytoin in pediatric convulsive status epilepticus: A randomized controlled trial
Conclusion: Levetiracetam is as efficacious as fosphenytoin in control of pediatric status epilepticus and is associated with lesser side effects. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Anusha Handral Basavraja G Veerappa Vykuntaraju K Gowda Sanjay K Shivappa Naveen Benakappa Asha Benakappa Source Type: research
Comparing sedative effect of dexmedetomidine versus midazolam for sedation of children while undergoing computerized tomography imaging
Conclusion: Using 3 μg/kg intranasal DEX for sedation of 1–6-year-old children was a suitable method to undergo noninvasive studies such as CT imaging. Intranasal DEX is superior to MID due to higher sedation satisfactory, faster starting effect of sedation, and lower side effects and complications. Nevertheless, in children with hemodynamic instability DEX is not an appropriate choice. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Reza Azizkhani Farhad Heydari Mohammadreza Ghazavi Maryam Riahinezhad Mohammadreza Habibzadeh Ali Bigdeli Keihan Golshani Saeid Majidinejad Abolfazl Mohammadbeigi Source Type: research
A treatise on pediatric meningiomas: Single-center retrospective cohort experience and review of literature
Conclusion: Although rare, pediatric meningiomas are biologically different from their adult counterparts. They have a male predominance, common in intraventricular region and cystic in nature. Radical excision is associated with good prognosis. Although benign, they are aggressive in nature and have a tendency to recur. The response to adjuvant therapy is modest. Further molecular research and genetic studies are necessary to understand the biology of pediatric meningiomas, which will help in the identification of targeted molecular therapy. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Sonal Jain Dattatraya Muzumdar Abhidha Shah Atul Goel Source Type: research
Comparison the oral premedication of midazolam, dexmedetomidine, and melatonin for children & #8217;s sedation and ease of separation from parents before anesthesia
Conclusion: Dexmedetomidine is more effective in acceptance of anesthesia induction. Furthermore, the midazolam and dexmedetomidine groups indicated better ease of separation and sedation scores than melatonin. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Bijan Yazdi Mahsa Mombeini Hesameddin Modir Alireza Kamali Source Type: research
Clinical profile, yield of cartridge-based nucleic acid amplification test (GeneXpert), and outcome in children with tubercular meningitis
Conclusion: GeneXpert has high diagnostic specificity, but negative results do not rule out TBM. CSF GeneXpert provided quick results. Placement of Ommaya reservoir in TBM stage II and III with hydrocephalus was not successful. Hydrocephalus was managed conservatively with success (53%). (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Santosh K Soma Lokesh Lingappa Subodh Raju Ramesh Konanki Amit K Gaur Ashwini Mohan Smilu Mohanlal Source Type: research
Urodynamic study findings prior to myelomeningocele repair in neonates
Conclusion: Detailed analysis of urodynamic study findings in larger patient groups may be important to understand the physiopathology of prenatal damage in patients with MMC. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Ibrahim Alatas Kerem Ozel Nursu Kara Huseyin Canaz Source Type: research
Dynamic surface exercise training in improving trunk control and gross motor functions among children with quadriplegic cerebral palsy: A single Center, randomized controlled trial
Conclusion: Six-week dynamic surface exercise therapy along with standard physiotherapy was effective in improving trunk control and gross motor function performance among children with spastic quadriplegic CP aged 6–12 years. (Source: Journal of Pediatric Neurosciences)
Source: Journal of Pediatric Neurosciences - November 6, 2020 Category: Neuroscience Authors: Sravan Reddy Gandhi Karunanithi Balaji Source Type: research
