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Journal of Pediatric Neurosciences

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https://www.readbyqxmd.com/read/27857814/a-rare-case-of-an-8-month-old-child-with-dengue-fever-complicated-by-acute-diffuse-transverse-myelitis
#1
Elisheba Patras, Pavan Kumar Polagani, Anuradha Sural, Nittin R Parkhe
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857813/anesthetic-management-of-a-child-with-lennox-gastaut-syndrome-with-intractable-epilepsy-posted-for-intracranial-surgery
#2
Indu Kapoor, Girija Prasad Rath
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857812/mitochondrial-membrane-protein-associated-neurodegeneration-in-a-turkish-patient
#3
Faruk Incecik, Ozlem M Hergüner, Seyda Besen, Serdar Ceylaner
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857811/primary-choroid-plexus-papilloma-of-cerebellopontine-angle-an-unusual-entity-in-infancy
#4
Ankur Kapoor, Ashish Aggarwal, Chirag K Ahuja, Pravin Salunke
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857810/cutaneous-and-brain-malformations-of-epidermal-nevus-syndrome-a-classical-image
#5
Anil Israni, Rachana Dubey, Biswaroop Chakrabarty, Atin Kumar, Sheffali Gulati
No abstract text is available yet for this article.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857809/delayed-speech-hyperactivity-and-coarse-facies-does-sanfilippo-syndrome-come-to-mind
#6
Ayşe Kartal
Mucopolysaccharidosis Type IIIA (MPS IIIA) or Sanfilippo-A syndrome is caused by a deficiency in lysosomal a-heparan N-sulfatase. Its clinical manifestations include progressive dementia, hyperactivity, and aggressive behavior. Unlike other mucopolysaccharide disorders, the diagnosis of MPS IIIA is challenging in both adults and children. This diagnostic challenge has been associated with the high incidence of false negative results encountered on urinary screening tests. We herein describe Sanfilippo-A syndrome in a pediatric patient who presented with progressive hyperactivity, delayed language, and developmental delay and a negative urine screening test...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857808/nervous-system-involvement-in-clinical-peripheral-inflammation-a-description-of-three-pediatric-cases
#7
Giovanna Vitaliti, Omidreza Tabatabaie, Nassim Matin, Giovanni Roberto Giugno, Piero Pavone, Riccardo Lubrano, Raffaele Falsaperla
Latest research data have emphasized the interaction between the nervous and the immune systems. In this regard, it has been demonstrated that the disruption of the blood-brain barrier (BBB) secondary to peripheral inflammation may play a key role in this relationship. This assumption is linked to recent findings according to which units that constitute the BBB are not only simply neurologic but have also been reconsidered as "neurovascular" elements, through which immune system molecules are vehiculated within the central nervous system (CNS)...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857807/transanal-protrusion-of-ventriculoperitoneal-shunt-via-appendicular-perforation-a-rare-case-report
#8
Shaam Bodeliwala, Atul Agrawal, Amit Mittal, Daljit Singh, B G Vageesh, Hukum Singh
Perforation of abdominal viscera and protrusion of the distal end of ventriculoperitoneal shunt (VPS) through natural orifice is well known but rare complication. We report a case of a transanal protrusion of distal end of VPS through appendix perforation without any symptomatology of prior appendicitis. To the best of our knowledge, no case of such kind has been reported in literature yet. The management plan of these patients should be looked in a different way because they may have underlying inflammation of the appendix and distal end of shunt removal should be done by proper surgical exploration followed by added appendicectomy...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857806/newborn-with-congenital-facial-palsy-and-bilateral-anotia-atresia-of-external-auditory-canal-rare-occurrence
#9
Rohan R Mahale, Anish Mehta, Aju Abraham John, Kiran Buddaraju, Abhinandan K Shankar, Srinivasa Rangasetty
Congenital facial palsy (CFP) is clinically defined as facial palsy of the seventh cranial nerve which is present at birth or shortly thereafter. It is generally considered to be either developmental or acquired in origin. Facial palsy of developmental origin is associated with other anomalies including those of pinna and external auditory canal, which range from mild defects to severe microtia and atresia. We report a 2-day-old male newborn that had right CFP with bilateral anotia and atresia of external auditory canals which is rare...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857805/neuropsychiatric-phenotype-in-a-child-with-pseudohypoparathyroidism
#10
Paola Visconti, Annio Posar, Maria Cristina Scaduto, Angelo Russo, Federica Tamburrino, Laura Mazzanti
Pseudohypoparathyroidism (PHP) is a rare heterogeneous genetic disease characterized by end-organ resistance to parathyroid hormone. In adulthood, heterogeneous neurological and psychiatric disorders have been reported which are associated with hypoparathyroidism in general and with PHP in particular, while for childhood, data are scanty. We report a case of a boy with PHP type 1b, in whom neurological signs at the onset prevailed, characterized by tic-like dyskinesias associated with a series of heterogeneous not well-defined neurological and behavioral features, describing the diagnostic work-up performed and the follow-up...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857804/spontaneous-thrombosis-of-vein-of-galen-malformation
#11
Kalpana Devi Kariyappa, Murali Krishnaswami, Francis Gnanaprakasam, Madan Ramachandran, Visvanathan Krishnaswamy
Vein of Galen malformation (VOGM) is a rare intracranial vascular malformation. Before the advent and advancement of various endovascular techniques, there was dismal prognosis. Rarely, this condition may spontaneously thrombose without the need for surgical or endovascular treatment with good prognosis. We report a case of an 8-month-old infant who had serial imaging, suggestive of VOGM and presented to us for further management, wherein the imaging revealed spontaneous thrombosis.
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857803/sympatholysis-subsequent-to-intraoperative-amygdalohippocampal-stimulation-a-report-of-three-pediatric-neurosurgical-cases-with-literature-review
#12
Ankur Khandelwal, Rudrashish Haldar, Arun Srivastava, Prabhat K Singh
The existence of neural connection between the limbic system (hypothalamus, hippocampus, amygdala, and other adjacent areas) and the autonomic nervous system has been postulated to trigger severe hemodynamic responses. The hemodynamic consequences of stimulation of amygdala or hippocampus have been sporadically reported in animal studies and adult patients. However, the effect of this stimulation in pediatric patients is scarce. We present our experience of three cases of sympatholysis during intraoperative manipulation of amygdalohippocampus and review the pertinent literature...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857802/giant-melanocytic-nevi-with-neurocutaneous-melanosis-masquerading-as-neurofibromas
#13
Vykuntaraju K Gowda, Anita Basude, Sahana M Srinivas, Maya Bhat
Neurocutaneous melanosis is congenital melanocytic nevus with neurological manifestations. We report a 4-year-old female child presenting with hyperpigmented and nodular skin lesion associated with developmental delay and convulsions. The child had multiple brownish-black nevi on the face and chest and giant melanocytic nevi on thoraco-abdomen, back, and gluteal region. Computed tomography scan of the brain showed calcification in the pons, right cerebellar hemisphere, and left medial temporal lobe. Skin biopsy done from nodular hyperpigmented site was suggestive of melanocytic nevi...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857801/schwartz-jampel-syndrome-with-gastroduodenal-bleeding
#14
İpek Polat, Pakize Karaoğlu, Uluç Yiş, Semra Hız Kurul
Schwartz-Jampel syndrome is a rare autosomal recessive disorder with joint contractures, generalized myotonia, skeletal anomalies, and facial dysmorphism. The patients with Schwartz-Jampel syndrome have muscle stiffness and electromyography reveals complex, repetitive discharges as myotonic discharges. It is unusual for a Schwartz-Jampel syndrome case to have recurrent gastrointestinal bleeding episodes. The stable endothelial barrier is provided by perlecan which is an important component of vascular structures...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857800/dyke-davidoff-masson-syndrome-a-rare-cause-of-cerebral-hemiatrophy-in-children
#15
Pareshkumar A Thakkar, Reema Haresh Dave
Dyke-Davidoff-Masson syndrome (DDMS) is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with positive radiological findings. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Here, we report a case of a 10-year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857799/a-rare-case-of-infantile-cerebellar-pilocytic-astrocytoma-and-thrombocytopenia-presenting-with-intratumoral-hemorrhage
#16
Shashank R Ramdurg, Jaybrata Maitra
Incidence of gliomas presenting with hemorrhage is around 3.7-7.2%. Low-grade gliomas account for <1% tumor with hemorrhage. Infants presenting with cerebellar pilocytic astrocytomas (PAs) and hemorrhage with thrombocytopenia have not been reported. We report an interesting case of a 9-month-old infant who presented to the emergency department in a drowsy state with recurrent vomiting. Laboratory investigations showed anemia, thrombocytopenia, and coagulopathy. Radiological evaluation showed a large PA with bleed...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857798/not-all-cases-of-nyctalopia-are-benign-unusual-and-serendipitous-presentation-of-arnold-chiari-type-1-malformation-at-a-pediatric-tertiary-care-center
#17
Kailash Chandra Patra, Abhijeet Prakash Kirtane
The Arnold-Chiari Type 1 malformation (CM1) is a rare congenital abnormality characterized by ectopia or caudal herniation of the cerebellar tonsils through the foramen magnum into the cervical spine, resulting in crowding at the craniocervical junction. It seldom presents in childhood with symptoms and a normal neurological examination. More typically, CM1 presents in young adult women with neurological symptoms including a headache, cervical pain, cranial nerve palsies, neurosensory deficit, and ataxia. Ocular manifestations associated with Chiari I include third and sixth cranial nerve paresis and convergence/divergence abnormalities...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857797/recurrent-acute-disseminated-encephalomyelitis-a-favorable-outcome-among-recurrent-brain-diseases-in-pediatric-patient
#18
Anirban Chatterjee, Supratim Datta
Acute disseminated encephalomyelitis (ADEM) is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) and considered mostly a monophasic course. Recurrence of ADEM is rare entity, posing diagnostic dilemma with multiple sclerosis (MS). There were no definite diagnostic criteria or established treatment for ADEM. International Pediatric MS Study Group laid down first consensus definition. We report a boy presented with recurrent episodes of fever, paraparesis, seizure, and unconsciousness...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857796/dwarf-with-dual-spinal-kyphotic-deformity-at-the-cervical-and-dorsal-spine-unassociated-with-odontoid-hypoplasia-surgical-management
#19
Guru Dutta Satyarthee, Dipanker Singh Mankotia
Morquio's syndrome is associated with systemic skeletal hypoplasia leading to generalized skeletal deformation. The hypoplasia of odontoid process is frequent association, which is responsible for atlantoaxial dislocation causing compressive myelopathy. However, development of sub-axial cervical kyphotic deformity unassociated with odontoid hypoplasia is extremely rare, and coexistence of dorsal kyphotic deformity is not reported in the western literature till date and represents first case. Current case is 16-year-old boy, who presented with severe kyphotic deformity of cervical spine with spastic quadriparesis...
July 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27857795/delayed-intramedullary-abscess-in-operated-case-of-spinal-lipoma
#20
Kamlesh Singh Bhaisora, Chaitanya Godbole, Kuntal Kanti Das, Anant Mehrotra, Shardhara Jayesh, Rabi Narayan Sahu, Sanjay Behari, Arun Kumar Srivastava, Awadhesh Kumar Jaiswal
Intramedullary abscess is a rare condition with high rate of mortality and morbidity. We are presenting a case of delayed intramedullary abscess in an operated case of spinal lipoma, after 2 years of primary surgery. To best of our knowledge this only second case of intramedullary abscess in a case of spinal lipoma without dermal sinus.
July 2016: Journal of Pediatric Neurosciences
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