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Issue Highlights Guidelines for the diagnosis and treatment of acute encephalopathy in childhoodMasashi Mizuguchi, Takashi Ichiyama, George Imataka, Akihisa Okumura, Tomohide Goto, Hiroshi Sakuma, Jun-ichi Takanashi, Kei Murayama, Takanori Yamagata, Hideo Yamanouchi, Tokiko Fukuda, Yoshihiro MaegakiDOI: https://doi.org/10.1016/j.braindev.2020.08.001Brain and Development, Vol. 43, Issue 1PreviewFull-Text HTMLPDFxThe cardinal symptom of acute encephalopathy is impairment of consciousness of acute onset during the course of an infectious disease, with duration and severity meeting defined criteria. Acute encephalopathy consists of multiple syndromes such as acute necrotizing encephalopathy, acute encephalopathy with biphasic seizures and late reduced diffusion and clinically mild encephalitis/encephalopathy with reversible splenial lesion. Among these syndromes, there are both similarities and differences.Epilepsy in Angelman syndrome: A scoping reviewDebopam SamantaDOI: https://doi.org/10.1016/j.braindev.2020.08.014Brain and Development, Vol. 43, Issue 1PreviewFull-Text HTMLPDFxAngelman Syndrome (AS) is characterized by severe developmental delays including marked speech impairment, movement abnormalities(ataxia, tremor), and unique behaviors such as frequent laughter and is caused by dysfunctional maternal UBE3A gene (maternal 15q11-13 deletions, maternal specific UBE3A mutation, uniparental disomy, and imprinting defect). Intractable epileptic seizures since early childhood with characteristic EEG abnormalities are present in 80–90% patients with AS. Underlying pathophysiology may involve neocortical and thalamocortical hyperexcitability secondary to severe reduction of GABAergic input, as well as dysfunctional synaptic plasticity, deficient synaptogenesis, and neuronal morphological immaturity. Chaperone therapy for molecular pathology in lysosomal diseasesYoshiyuki SuzukiDOI: https://doi.org/10.1016/j.braindev.2020.06.015Brain and Development, Vol. 43, Issue 1PreviewFull-Text HTMLPDFxIn lysosomal diseases, enzyme deficiency is caused by misfolding of mutant enzyme protein with abnormal steric structure that is expressed by gene mutation. Chaperone therapy is a new molecular therapeutic approach primarily for lysosomal diseases. The misfolded mutant enzyme is digested rapidly or aggregated to induce endoplasmic reticulum stress. As a result, the catalytic activity is lost. The following sequence of events results in chaperone therapy to achieve correction of molecular pathology.  Predictors of ADHD persistence in elementary school children who were assessed in earlier grades: A prospective cohort study from Istanbul, TurkeySebla Gökçe, Yankı Yazgan, Herdem Aslan Genç, Gresa Çarkaxhiu Bulut, Esengül Kayan, Onur Tuğçe Poyraz Fındık, Ayşe Burcu Ayaz, Canan Yusufoğlu, Didem Ayyıldız, Burcu Budak, Ender Atabay, Sultan Seval Yılmaz, Umut Kaytanlı, Elif AkınDOI: https://doi.org/10.1016/j.braindev.2020.11.013Publication stage: In Press Corrected ProofPreviewFull-Text HTMLPDFxAttention-deficit hyperactivity disorder (ADHD) is the most common neurodevelopmental disorders among school-age children worldwide. In a more recent follow-up study, Biederman et al. found that 78% of children diagnosed with ADHD between the ages of 6–17 years continued to have a full (35%) or a partial persistence after eleven years.Refractory cerebral infarction in a child with an ACTA2 mutationKeita Kanamori, Yuri Sakaguchi, Kyoji Tsuda, Satoshi Ihara, Sahoko MiyamaDOI: https://doi.org/10.1016/j.braindev.2020.12.001Publication stage: In Press Corrected ProofPreviewFull-Text HTMLPDFxCerebral infarctions in patients with a specific ACTA2 mutation can occur even in early childhood, recur frequently, and cause severe motor and cognitive impairment. Physicians should be highly aware of this disease and be ready to provide the medical and surgical interventions necessary to minimize the disabling sequelae.Early non-convulsive seizures are associated with the development of acute encephalopathy with biphasic seizures and late reduced diffusionAzusa Maruyama, Shoichi Tokumoto, Hiroshi Yamaguchi, Yusuke Ishida, Tsukasa Tanaka, Kazumi Tomioka, Masahiro Nishiyama, Kyoko Fujita, Daisaku Toyoshima, Hiroaki NagaseDOI: https://doi.org/10.1016/j.braindev.2020.11.012Publication stage: In Press Corrected ProofPreviewFull-Text HTMLPDFxChildren with either febrile seizure or acute encephalopathy exhibit seizures and/or impaired consciousness accompanied by fever of unknown etiology (SICF). Among children with SICF, we previously reported those who have refractory status epilepticus or prolonged neurological abnormalities with normal AST levels are at a high risk for the development of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), considered to be caused by excitotoxicity. Non-convulsive seizures (NCS) are common in critically ill children and cause excitotoxic neuronal injury.Reply to the letter: “Perampanel may be beneficial in leigh syndrome by its anti-oxidative but not anti-epileptic effect”Shuhei Kimura, Hideaki Shiraishi, Kiyoshi Egawa, Masaya Uchida, Michihiko UenoDOI: https://doi.org/10.1016/j.braindev.2020.10.008Publication stage: In Press Corrected ProofPreviewFull-Text HTMLPDFSupplemental MaterialsxWe appreciate the comments of Josef Finsterer on our article. We agree that some aspects of the recovery of activities of daily living (ADLs) with perampanel (PER) in this case cannot be explained by antiepileptic effects alone. A decrease in ADLs when energy demand is increased in Leigh syndrome (LS) is well known and would be expected to exacerbate the pathogenesis of LS itself, triggered by infection. There is a good possibility that the dystonia and tonicity of his extremities that appeared during this period were caused by involuntary movements of the limbs.International classification of functioning, disability and health framework (ICF) based adaptive functioning outcomes of children with organic acidemias from a middle-income countryEzgi Özalp Akin, Bahar Bingoler Pekcici, Fatma Tuba EminogluDOI: https://doi.org/10.1016/j.braindev.2020.11.011Publication stage: In Press Corrected ProofPreviewFull-Text HTMLPDFxThe World Health Organization International Classification of Functioning, Disability and Health Framework (ICF) states that a child’s health conditions, functions, activities, participation in life and contextual factors shape disability. Research on the development of children with organic acidemias (OA) mostly focused on cognitive and medical outcomes. This study aimed to examine adaptive functioning of children with OAs based on ICF. About Brain and Development Published by Elsevier since 1993, this is the official English language journal of the Japanese Society of Child Neurology (JSCN). Since 2006 (volume 28), the journal increased its publication to 10 issues from 8 issues per year, and the journal is a well recognised vehicle for pediatric neurologists to publish their research. The Japanese Society of Child Neurology (JSCN) was founded in 1961. Publication of its official journal in Japanese, "No To Hattatsu", began in 1969, and its official journal in English, "Brain Development", was first published in 1979. Since 2007 the society has been reorganized as a limited liability nonprofits mutual benefit corporation for further development.JSCN is among the largest affiliate societies of the Japan Pediatric Association. The current number of full members is around 3500. These are grouped into nine local chapters (Kyushu, Chugoku-Shikoku, Kinki, Tokai, Hokuriku, Koshin-etsu, Kanto, Tohoku and Hokkaido), each of which holds its own annual meeting once or twice a year. The General Assembly of JSCN is held once a year, usually in May or June. The average number of participants has been around 1500, with approximately 400 free papers, plenary lectures, and invited lectures. The subjects of these papers and lectures encompass almost every aspect of child neurology, neurosurgery, psychiatry, rehabilitation medicine, and related basic sciences such as molecular biology, neurophysiology, neuropathology, and neuropharmacology.Since 1971, JSCN has also sponsored the Graduate Seminar of Child Neurology, an annual teaching conference for young pediatric neurologists. Every year, between 100 and 120 young pediatric neurologists attend lectures, case conferences, and symposia that help upgrade their knowledge on pediatric neurology and related fields. Access this journal on ScienceDirect Visit ScienceDirect to see if you have access via your institution. JSCN Best Paper Awards Annually, the Japanese Society of Child Neurology (JSCN) selects and recognizes the best papers for the year. Brain & Development will announce Best Paper Awards for 2019 at the 62nd Annual Meeting of JSCN, which will be held online in August 2020.Yoshihiko SaitoAssociation of inattention with slow-spindle density in sleep EEG of children with attention deficit-hyperactivity disorderAkiko ShibataThermolabile polymorphism of carnitine palmitoyltransferase 2: A genetic risk factor of overall acute encephalopathyRecipients of the award from 1983 to present

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