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Miguel Hernández-Cerón, Juan Carlos Martínez-Lazcano, Carmen Rubio, Verónica Custodio, Edith González-Guevara, Carlos Castillo-Pérez, Carlos Paz
Lesions of the cerebellar dentate nucleus (DN) reduce the after-discharge duration induced by repetitive kindling stimulation and decrease seizures to a lower rank according to Racine's scale. The DN sends cholinergic and glutamatergic fibers to the red nucleus (RN), which is composed of glutamatergic and GABAergic cells. To test the participation of these neurotransmitters in seizures, we compared the levels of glutamate and gamma-aminobutyric acid (GABA) at the RN in a control condition, a kindled stage, and a kindled stage followed by DN lesions...
October 18, 2016: Journal of Neuroscience Research
Takuya Toyonaga, Shigeru Yamaguchi, Kenji Hirata, Kentaro Kobayashi, Osamu Manabe, Shiro Watanabe, Shunsuke Terasaka, Hiroyuki Kobayashi, Naoya Hattori, Tohru Shiga, Yuji Kuge, Shinya Tanaka, Yoichi M Ito, Nagara Tamaki
PURPOSE: Metabolic activity and hypoxia are both important factors characterizing tumor aggressiveness. Here, we used F-18 fluoromisonidazole (FMISO) and F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) to define metabolically active hypoxic volume, and investigate its clinical significance in relation to progression free survival (PFS) and overall survival (OS) in glioblastoma patients. EXPERIMENTAL DESIGN: Glioblastoma patients (n = 32) underwent FMISO PET, FDG PET, and magnetic resonance imaging (MRI) before surgical intervention...
October 18, 2016: European Journal of Nuclear Medicine and Molecular Imaging
Max O Krucoff, Steven Cook, Owoicho Adogwa, Jessica Moreno, Siyun Yang, Jichun Xie, Alexander O Firempong, Nandan Lad, Carlos A Bagley
OBJECT: To examine the role of race, gender, and socioeconomics on presentations and outcomes of adult Chiari 1 malformations. METHODS: The charts of 638 adult patients with Chiari 1 malformations were reviewed, and 287 patients were included. Race, gender, insurance status, symptoms, depth of cerebellar tonsillar herniation, and presence of syringomyelia were examined as covariates in multivariate logistic regression models to identify independent predictors of presentation and outcome...
October 14, 2016: World Neurosurgery
Yanling Wei, Jun Yang, Jun Wang, Yang Yang, Juan Huang, Hao Gong, Hongli Cui, Dongfeng Chen
BACKGROUND: The dysbiosis of intestinal microbiota plays an important role in the development of gut-derived infections, making it a potential therapeutic target against multiple organ dysfunction syndrome (MODS) after sepsis. However, the effectiveness of fecal microbiota transplantation (FMT) in treating this disease has been rarely investigated. METHODS: Two male patients, a 65-year-old and an 84-year-old, were initially diagnosed with cerebellar hemorrhage and cerebral infarction, respectively, after admission...
October 18, 2016: Critical Care: the Official Journal of the Critical Care Forum
Alexander Tucker, Kritsanapol Boon-Unge, Nancy McLaughlin, Hassana Ibrahim, Nagesh Rao, Neil Martin, Richard Everson, Négar Khanlou
No abstract text is available yet for this article.
October 16, 2016: Journal of Pathology and Translational Medicine
Kyu-Man Han, Daseul Kim, Youngbo Sim, June Kang, Aram Kim, Eunsoo Won, Woo-Suk Tae, Byung-Joo Ham
BACKGROUND: Morphologic changes of the brainstem in major depressive disorder (MDD) have rarely been reported in neuroimaging studies, even though, monoaminergic neurotransmitters are synthesized in several brainstem regions. We aimed to investigate volume changes in each region of the brainstem and their association with antidepressant use or the remission status of MDD. METHODS: A total of 126 patients with MDD and 101 healthy controls underwent T1-weighted structural magnetic resonance imaging...
October 11, 2016: Journal of Affective Disorders
K M Naga Srinivas Nadella, Hana Roš, Chiara Baragli, Victoria A Griffiths, George Konstantinou, Theo Koimtzis, Geoffrey J Evans, Paul A Kirkby, R Angus Silver
Understanding how neural circuits process information requires rapid measurements of activity from identified neurons distributed in 3D space. Here we describe an acousto-optic lens two-photon microscope that performs high-speed focusing and line scanning within a volume spanning hundreds of micrometers. We demonstrate its random-access functionality by selectively imaging cerebellar interneurons sparsely distributed in 3D space and by simultaneously recording from the soma, proximal and distal dendrites of neocortical pyramidal cells in awake behaving mice...
October 17, 2016: Nature Methods
Egesta Lopci, Laura Olivari, Lorenzo Bello, Pierina Navarria, Arturo Chiti
We report the case of a 55-year-old woman with cerebral proliferative angiopathy (CPA). Her medical history included brain surgery for small vascular lesions and suspicion of cerebral malignancy. C methionine PET (C-METH PET) demonstrated a diffusely increased uptake on the right hemisphere. Contrast-enhanced MRI documented a massive lesion with a diffuse "nidus" appearance, involving the right cerebral hemisphere (sparing the inferior frontal gyrus and the anterior frontal lobe), the brainstem, and the middle cerebellar peduncle...
October 5, 2016: Clinical Nuclear Medicine
Anna Maria Pinto, Valentina Imperatore, Laura Bianciardi, Margherita Baldassarri, Paolo Galluzzi, Simone Furini, Giovanni Centini, Alessandra Renieri, Francesca Mari
Orofacial clefts are the most common congenital craniofacial anomalies and can occur as an isolated defect or be associated with other anomalies such as posterior fossa anomalies as a part of several genetic syndromes. We report two consecutive voluntary pregnancy interruptions in a nonconsanguineous couple following the fetal ultrasound finding of cleft lip and palate and posterior fossa anomalies confirmed by means of post-termination examination on the second fetus. The quantitative fluorescent PCR, the karyotype, and the comparative genomic hybridization-array analysis after amniocentesis were normal...
October 4, 2016: Clinical Dysmorphology
Youn Hee Jee, Nadine Sowada, Thomas C Markello, Iraj Rezvani, Guntram Borck, Jeffrey Baron
Linear growth failure can be caused by many different genetic abnormalities. In many cases, the genetic defect affects not only the growth plate, causing short stature, but also other organs/tissues causing additional clinical abnormalities. The proband was evaluated at 10 years of age for impaired postnatal linear growth (height 113.3 cm, -4.6 SDS), a bone age that was delayed by 5 years, dysmorphic facies, cognitive impairment, and central nervous system anomalies. His younger brother, presented only with growth failure at 10 months of age...
October 17, 2016: Clinical Genetics
Rachael L Cohen, Russell L Margolis
PURPOSE OF REVIEW: Spinocerebellar ataxia type 12 (SCA12) is a rare autosomal dominant neurodegenerative disease characterized by tremor, gait abnormalities, and neuropsychiatric syndromes. The location of the causative CAG/CTG expansion mutation in PPP2R2B, a gene encoding regulatory units of the protein phosphatase 2A, may provide unique insights into the pathogenesis of neurodegeneration. RECENT FINDINGS: The first neuropathological examination of a brain from an SCA12 patient revealed both cerebellar and cerebral cortical atrophy, with a noted loss of Purkinje cells and no evidence of polyglutamine aggregates...
September 29, 2016: Current Opinion in Neurology
Renee E Shimizu, Allan D Wu, Barbara J Knowlton
Effective learning results not only in improved performance on a practiced task, but also in the ability to transfer the acquired knowledge to novel, similar tasks. Using a modified serial reaction time (RT) task, the authors examined the ability to transfer to novel sequences after practicing sequences in a repetitive order versus a nonrepeating interleaved order. Interleaved practice resulted in better performance on new sequences than repetitive practice. In a second study, participants practiced interleaved sequences in a functional MRI (fMRI) scanner and received a transfer test of novel sequences...
October 17, 2016: Behavioral Neuroscience
Y Lee, D K Lee, J M Lee, S J Chung, J J Lee, Y H Sohn, P H Lee
BACKGROUND AND PURPOSE: Although early cerebellar symptoms are one of the exclusive criteria in the diagnosis of progressive supranuclear palsy (PSP), cerebellar involvement in PSP is evident both clinically and pathologically. However, structural analysis focusing on the cerebellum has not been previously studied in patients with PSP. We aimed to evaluate cerebellar involvement in PSP using a magnetic resonance imaging-based segmental volumetric analysis. METHODS: We retrospectively enrolled 48 patients with PSP composed of 25 patients with PSP-Richardson's syndrome (RS) and 23 patients with pure akinesia with gait freezing, 39 patients with Parkinson's disease (PD) and 34 healthy controls...
October 16, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Robert C A M van Waardenburg
Tyrosyl-DNA phosphodiesterase I (TDP1), like most DNA repair associated proteins, is not essential for cell viability. However, dysfunctioning TDP1 or ATM (ataxia telangiectasia mutated) results in autosomal recessive neuropathology with similar phenotypes, including cerebellar atrophy. Dual inactivation of TDP1 and ATM causes synthetic lethality. A TDP1H(493)R catalytic mutant is associated with spinocerebellar ataxia with axonal neuropathy (SCAN1), and stabilizes the TDP1 catalytic obligatory enzyme-DNA covalent complex...
2016: Journal of Neurology & Neuromedicine
Navid Elmi Sadr, Bijan Samavat, Payam Mehrian, Alireza Hedayatfar
Introduction. Terson syndrome is described as intraocular hemorrhage in association with any type of intracranial hemorrhage and is associated with higher mortality rate and vision loss. Intraocular hemorrhage in Terson syndrome may be diagnosed using computed tomography but there are false positive results. Silicone oil which is widely used for internal tamponade of complicated retinal detachments has high attenuation on computed tomography and hyperintensity on T1-weighted magnetic resonance imaging that can mimic intraocular hemorrhage...
2016: Case Reports in Ophthalmological Medicine
Martin A Nzegwu, Samuel Ohegbulam, Chika Ndubuisi, Okwuoma Okwunodulu, Emeka Okorie, Sunday Nkwerem, Onyiye Okonkwo, Onyeka Aniume, Sunday Nnamani, Onyekachi Nwokoro, Anthony Eni, Isaiah Nwideyi, Victor Nzegwu
Liponeurocytoma is a newly defined clinical entity predominantly seen in the cerebellum as a slow-growing tumor. In this report, we present the case of a 6-year-old Nigerian girl with a liponeurocytoma, and review of literature.
September 5, 2016: Rare Tumors
Jan-Folkard Willms, Gerasimos Baltsavias, Jan-Karl Burkhardt, Silvia Ernst, Alexander A Tarnutzer
We discuss a case with combined vestibulocochlear and facial neuropathy mimicking a less urgent peripheral vestibular pattern of acute vestibular syndrome (AVS). With initial magnetic resonance imaging read as normal, the patient was treated for vestibular neuropathy until headaches worsened and a diagnosis of subarachnoid hemorrhage was made. On conventional angiography, a ruptured distal right-sided aneurysm of the anterior inferior cerebellar artery was diagnosed and coiled. Whereas acute vestibular loss usually points to a benign peripheral cause of AVS, combined neuropathy of the vestibulocochlear and the facial nerve requires immediate neuroimaging focusing on the cerebellopontine angle...
October 13, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Yu Kobayashi, Jun Tohyama, Tomoyuki Akiyama, Shinichi Magara, Hideshi Kawashima, Noriyuki Akasaka, Mitsuko Nakashima, Hirotomo Saitsu, Naomichi Matsumoto
Cerebral folate deficiency due to folate receptor 1 gene (FOLR1) mutations is an autosomal recessive disorder resulting from a brain-specific folate transport defect. It is characterized by late infantile onset, severe psychomotor regression, epilepsy, and leukodystrophy. We describe a consanguineous girl exhibiting severe developmental regression, intractable epilepsy, polyneuropathy, and profound hypomyelination with cortical involvement. Magnetic resonance imaging showed cortical disturbances in addition to profound hypomyelination and cerebellar atrophy...
October 12, 2016: Brain & Development
Tomokazu Kimizu, Yukitoshi Takahashi, Taikan Oboshi, Asako Horino, Takayoshi Koike, Shinsaku Yoshitomi, Tatsuo Mori, Tokito Yamaguchi, Hiroko Ikeda, Nobuhiko Okamoto, Mitsuko Nakashima, Hirotomo Saitsu, Mitsuhiro Kato, Naomichi Matsumoto, Katsumi Imai
INTRODUCTION: Mutations of SLC35A2 that encodes Golgi-localized Uridine diphosphate (UDP)-galactose transporter at Xp11.23 lead to congenital disorders of glycosylation (CDG). Although patients with CDG generally have diverse systemic symptoms, patients with a SLC35A2 mutation manifest predominantly disorders of the central nervous system (CNS). CASE REPORT: A female infant aged 12months was referred to our center because of intractable seizures. The patient was born with birth weight of 3228g after 40weeks of unremarkable gestation...
October 12, 2016: Brain & Development
Marios K Georgakis, Maria A Karalexi, Eleni I Kalogirou, Anton Ryzhov, Anna Zborovskaya, Nadya Dimitrova, Sultan Eser, Luis Antunes, Mario Sekerija, Tina Zagar, Joana Bastos, Domenic Agius, Margareta Florea, Daniela Coza, Evdoxia Bouka, Charis Bourgioti, Helen Dana, Emmanuel Hatzipantelis, Maria Moschovi, Savvas Papadopoulos, Georgios Sfakianos, Evgenia Papakonstantinou, Sophia Polychronopoulou, Spyros Sgouros, Kalliopi Stefanaki, Eftichia Stiakaki, Katerina Strantzia, Basilios Zountsas, Apostolos Pourtsidis, Eustratios Patsouris, Eleni Th Petridou
Pilocytic astrocytomas (PA) comprise the most common childhood central nervous system (CNS) tumor. Exploiting registry-based data from Southern and Eastern Europe (SEE) and SEER, US, we opted to examine incidence, time trends, survival and tentative outcome disparities of childhood PA by sociodemographic and clinical features. Childhood PA were retrieved from 12 SEE registries (N = 552; 1983-2014) and SEER (N = 2723; 1973-2012). Age-standardized incidence rates (ASR) were estimated and survival was examined via Kaplan-Meier and Cox regression analysis...
October 14, 2016: Journal of Neuro-oncology
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