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Brain & Development | Page 2

Josef Finsterer
No abstract text is available yet for this article.
April 2, 2019: Brain & Development
Akira Yasumura, Mikimasa Omori, Ayako Fukuda, Junichi Takahashi, Yukiko Yasumura, Eiji Nakagawa, Toshihide Koike, Yushiro Yamashita, Tasuku Miyajima, Tatsuya Koeda, Masao Aihara, Masumi Inagaki
BACKGROUND: The neural correlates of executive function disorders are thought to be predominantly localized within the prefrontal cortex (PFC). However, no study to date has investigated changes in this system across different age groups in children with attention deficit hyperactivity disorder (ADHD). Thus, this study aimed to explore changes in PFC function in children with ADHD. METHODS: Study participants included typically developing (TD) children (n = 140) and children with ADHD (n = 67) of primary school age...
April 2, 2019: Brain & Development
Tomoki Maeda
No abstract text is available yet for this article.
March 29, 2019: Brain & Development
Tsukasa Tanaka, Hiroaki Nagase, Hiroshi Yamaguchi, Yusuke Ishida, Kazumi Tomioka, Masahiro Nishiyama, Daisaku Toyoshima, Azusa Maruyama, Kyoko Fujita, Kandai Nozu, Noriyuki Nishimura, Hiroshi Kurosawa, Ryojiro Tanaka, Kazumoto Iijima
BACKGROUND: Seizures and/or impaired consciousness accompanied by fever without known etiology (SICF) is common in the pediatric emergency setting. No optimal strategy for the management of SICF in childhood currently exists. We previously demonstrated the effectiveness of targeted temperature management (TTM) against SICF with a high risk of morbidity; however, some patients with SICF develop neurological sequelae despite TTM, which necessitate additional neuroprotective treatment. The clinical characteristics of these severe cases have not been studied...
March 28, 2019: Brain & Development
Mahesh Kamate, Mayank Detroja, Virupaxi Hattiholi
Neuronal ceroid lipofuscinosis (NCL) is a group of progressive neurodegenerative disorders characterized by intracellular accumulation of ceroid lipopigments. Based on gene defect of NCL-associated proteins, 14 types of NCL have been described till date. NCL type 11 was first described in 2014 in two siblings as adult-onset NCL and was found to be due to a homozygous progranulin gene mutation. These siblings had progressive retinopathy, recurrent generalized seizures, moderate ataxia and subtle cognitive dysfunction...
March 25, 2019: Brain & Development
Alessandra Carvalho, Carlos Brites, Ganeshwaran Mochida, Paloma Ventura, Adriana Fernandes, Maria Lúcia Lage, Tânia Taguchi, Ivar Brandi, Alfredo Silva, Giulia Franceschi, Pedro Lucena, Rita Lucena
OBJECTIVE: To describe the neurological and neurodevelopmental features at 1 year of age in children with cerebral palsy (CP) related to probable congenital Zika (CZ), followed in a referral neurorehabilitation hospital. METHODS: Data on 82 children with CP associated with probable CZ, who consecutively attended the neurodevelopmental and neurological assessment around one year of age, were collected. For neurodevelopmental evaluation, Bayley-III Scales of Infant and Toddler Development was used...
March 23, 2019: Brain & Development
Aya Hashimoto, Ichiro Kuki, Masataka Fukuoka, Kiyohiro Kim, Takeshi Inoue, Megumi Nukui, Shin Okazaki, Hisashi Kawawaki, Yuji Nakamura, Shinji Saitoh
BACKGROUND: Cerebellar ataxia, Areflexia, Pes cavus, Optic atrophy and Sensorineural hearing loss (CAPOS) syndrome is a known ATP1A3-related disorder, but little has been elucidated regarding its pathophysiology. We now report two new patients, a Japanese boy and his mother with a pathogenic mutation (c.2452G>A) in ATP1A3, who were diagnosed with CAPOS syndrome. METHODS: After febrile illnesses at 7 months of age, and again at 22 months of age, the boy had a reduced level of consciousness, truncal ataxia and eye movement-disorders...
March 20, 2019: Brain & Development
Maoling Yang, Nong Xiao, Shuang Chen, Wei Jiang
BACKGROUND: This study aimed to analyze clinical and imaging features of children with severe Japanese encephalitis (JE), and to analyze causes and solutions for psychiatric symptoms of JE during the convalescent period. METHODS: We analyzed clinical information for 78 children with severe JE at the Department of Neurology, Department of Infection, and Department of Rehabilitation in our hospital during 2014-2016. Seventy-eight cases of severe JE were divided into patients with psychiatric symptoms and no psychiatric symptoms groups...
March 19, 2019: Brain & Development
Tomoko Mizuno, Ayako Kashimada, Toshihiro Nomura, Kengo Moriyama, Haruna Yokoyama, Setsuko Hasegawa, Masatoshi Takagi, Shuki Mizutani
BACKGROUND: Spinocerebellar ataxia type 5 (SCA5), a dominant spinocerebellar ataxia is caused by spectrin beta nonerythrocytic 2 gene (SPTBN2) mutation. It typically consists of a slow progressive cerebellar ataxia with an onset principally in adulthood. Here, we report on the first Japanese patient with infantile-onset SCA5 associated with a novel heterozygous SPTBN2 mutation. CASE REPORT: The patient, a 6-year-old girl, developed delayed motor development and unsteady arm movement during infancy...
March 18, 2019: Brain & Development
Lokesh Saini, Priyanka Madaan, M R Rudresh Naik
No abstract text is available yet for this article.
March 12, 2019: Brain & Development
Akihisa Okumura, Harushi Mori
No abstract text is available yet for this article.
March 11, 2019: Brain & Development
Kana Okazaki Fukui, Masaya Kubota, Hiroshi Terashima, Akira Ishiguro, Hirofumi Kashii
BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most prevalent encephalopathy in Japanese children. AESD is characterized by a prolonged febrile seizure on day 1 followed by secondary seizures and MRI abnormality on days 4-6, resulting in high incidence of neurological sequelae. We aimed to clarify whether early administration of vitamins (vitamin B1, vitamin B6, and l-carnitine) would improve the clinical course of AESD. METHODS: We retrospectively reviewed 34 patients with acute encephalopathy who were admitted to our hospital between January 2009 and August 2016...
March 9, 2019: Brain & Development
Toru Imagi, Tomonaga Matsushita, Miyuki Matsushita, Yukako Yae, Takaoki Yokochi, Go Kawano, Yukihiro Akita, Keizo Ohbu, Toyojiro Matsuishi
Infarct locations in children with arterial ischemic stroke have primarily been reported to be lobar or in the basal ganglia, and those in patients with Down syndrome (DS) and antiphospholipid syndrome (APS) are typically wide and multiple. No solitary brain stem infarctions have ever been reported in children with DS until now. Here, we report a case of brain stem infarction in a 6-year-old boy with DS who had no cardiac, renal, or intestinal complications. He exhibited ataxic gait and medial longitudinal fasciculus (MLF) symptoms at first presentation...
March 8, 2019: Brain & Development
Kyoko Hazama, Takashi Shiihara, Hiroyuki Tsukagoshi, Takeshi Matsushige, Yuri Dowa, Mio Watanabe
BACKGROUND: Rhinovirus is a common respiratory pathogen for children throughout the year; nevertheless, its central nervous system involvement is extremely rare, and only two cases have been reported to date: meningitis and sepsis-like illness. PATIENT: A previously healthy 2-year-old Japanese boy developed fever, followed by seizures and lethargy. His cerebrospinal fluid cell count and protein level were slightly increased; brain magnetic resonance imaging showed abnormal intensities in the bilateral cerebellar dentate nuclei, which were prominent in diffusion-weighted images...
March 5, 2019: Brain & Development
Chihiro Matsumoto, Madoka Mori-Yoshimura, Satoru Noguchi, Yukari Endo, Yasushi Oya, Miho Murata, Ichizo Nishino, Yuji Takahashi
We report a 38-year-old woman who presented with mild proximal dominant muscle weakness and fatigability that fluctuated during menstruation and treatment with ephedrine-containing medication. The patient had been diagnosed with "congenital myopathy with tubular aggregates" by muscle biopsy at age 19. Her revised diagnosis was congenital myasthenic syndrome (CMS) caused by a mutation in GFPT1 (2p13.3 [MIM 610542], c.722_723insG homozygote, CMS-GFPT1) based on a screening gene analysis. Muscle CT revealed diffuse atrophy of proximal and axial muscles focused on the vastus lateralis, hamstrings, medial gastrocnemius and soleus muscles...
March 4, 2019: Brain & Development
Yosuke Kita, Fumiko Ashizawa, Masumi Inagaki
PURPOSE: Motor skill screening tools are essential for the early detection of developmental coordination disorder (DCD). The present study aimed to examine any cultural and rater effects on these tools. This then enabled us to judge the validity of the original cut-off values for identifying diagnosable children. METHODS: A community sample survey was performed in Japan; 3852 children aged 6-9 years were recruited. Both parents and teachers evaluated the motor skills of their children using the Movement Assessment Battery for Children - Second Edition Checklist...
March 2, 2019: Brain & Development
Masahiro Nishiyama, Hiroaki Nagase, Kazumi Tomioka, Tsukasa Tanaka, Hiroshi Yamaguchi, Yusuke Ishida, Daisaku Toyoshima, Kyoko Fujita, Azusa Maruyama, Kaori Sasaki, Yoshinobu Oyazato, Taku Nakagawa, Yuichi Takami, Kandai Nozu, Noriyuki Nishimura, Ichiro Nakashima, Kazumoto Iijima
The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5 ± 3...
March 1, 2019: Brain & Development
Akihisa Okumura, Hirokazu Kurahashi, Hideyuki Iwayama, Shingo Numoto
OBJECTIVE: This study measured the serum carnitine levels in patients with epilepsy and determined the factors contributing to low carnitine levels. METHODS: We measured the serum carnitine levels in 94 consecutive patients with epilepsy, including the free carnitine (FC) and acylcarnitine fractions, using an enzyme cycling method. We defined a low FC as a serum FC level < 36 μmol/L. Age, body mass index (BMI), standard deviation score of BMI (BMI-SDS), use of valproate, cognitive disorder, and feeding problems differed between patients with low and normal FC...
February 28, 2019: Brain & Development
Christopher Medina-Kirchner, Ciara A Torres
No abstract text is available yet for this article.
February 26, 2019: Brain & Development
Klára Brožová, Jan Brož
No abstract text is available yet for this article.
February 22, 2019: Brain & Development
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