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https://www.readbyqxmd.com/read/28110411/development-of-the-siope-dipg-network-registry-and-imaging-repository-a-collaborative-effort-to-optimize-research-into-a-rare-and-lethal-disease
#1
Sophie E M Veldhuijzen van Zanten, Joshua Baugh, Brooklyn Chaney, Dennis De Jongh, Esther Sanchez Aliaga, Frederik Barkhof, Johan Noltes, Ruben De Wolf, Jet Van Dijk, Antonio Cannarozzo, Carin M Damen-Korbijn, Jan A Lieverst, Niclas Colditz, Marion Hoffmann, Monika Warmuth-Metz, Brigitte Bison, David T W Jones, Dominik Sturm, Gerrit H Gielen, Chris Jones, Esther Hulleman, Raphael Calmon, David Castel, Pascale Varlet, Géraldine Giraud, Irene Slavc, Stefaan Van Gool, Sandra Jacobs, Filip Jadrijevic-Cvrlje, David Sumerauer, Karsten Nysom, Virve Pentikainen, Sanna-Maria Kivivuori, Pierre Leblond, Natasha Entz-Werle, Andre O von Bueren, Antonis Kattamis, Darren R Hargrave, Péter Hauser, Miklos Garami, Halldora K Thorarinsdottir, Jane Pears, Lorenza Gandola, Giedre Rutkauskiene, Geert O Janssens, Ingrid K Torsvik, Marta Perek-Polnik, Maria J Gil-da-Costa, Olga Zheludkova, Liudmila Shats, Ladislav Deak, Lidija Kitanovski, Ofelia Cruz, Andres Morales La Madrid, Stefan Holm, Nicolas Gerber, Rejin Kebudi, Richard Grundy, Enrique Lopez-Aguilar, Marta Zapata-Tarres, John Emmerik, Tim Hayden, Simon Bailey, Veronica Biassoni, Maura Massimino, Jacques Grill, William P Vandertop, Gertjan J L Kaspers, Maryam Fouladi, Christof M Kramm, Dannis G van Vuurden
Diffuse intrinsic pontine glioma (DIPG) is a rare and deadly childhood malignancy. After 40 years of mostly single-center, often non-randomized trials with variable patient inclusions, there has been no improvement in survival. It is therefore time for international collaboration in DIPG research, to provide new hope for children, parents and medical professionals fighting DIPG. In a first step towards collaboration, in 2011, a network of biologists and clinicians working in the field of DIPG was established within the European Society for Paediatric Oncology (SIOPE) Brain Tumour Group: the SIOPE DIPG Network...
January 21, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28108856/diagnosis-and-management-for-trigeminal-neuralgia-caused-solely-by-venous-compression
#2
Takuro Inoue, Hisao Hirai, Ayako Shima, Fumio Suzuki, Takanori Fukushima, Masayuki Matsuda
BACKGROUND: Trigeminal neuralgia (TN) caused solely by venous compression is uncommon. An appropriate vein management protocol for microvascular decompression (MVD) has not been established. We evaluated the management of veins for TN solely due to venous involvement. METHOD: We analyzed 31 patients with TN in whom only veins were manipulated during MVD. The culprit veins smaller in diameter than the vein of the cerebellopontine fissure (VCPF) were coagulated and divided in 20 patients (divided group), while transposition was performed for larger veins in the remaining 11 patients (non-divided group)...
January 20, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28108511/biomarker-based-pet-imaging-of-diffuse-intrinsic-pontine-glioma-in-mouse-models
#3
Susanne Kossatz, Brandon Carney, Melanie E Schweitzer, Giuseppe Carlucci, Vesselin Miloushev, Uday B Maachani, Prajwal Rajappa, Kayvan Keshari, David J Pisapia, Wolfgang A Weber, Mark M Souweidane, Thomas Reiner
Diffuse intrinsic pontine glioma (DIPG) is a childhood brainstem tumor with a universally poor prognosis. Here, we characterize on a positron emission tomography (PET) probe for imaging DIPG in vivo. In human histological tissues, the probes target, poly(ADP)ribose polymerase 1 (PARP1), was highly expressed in DIPG compared to normal brain. PET imaging allowed for the sensitive detection of DIPG in a genetically engineered mouse model (GEMM), and probe uptake correlated to histologically determined tumor infiltration...
January 20, 2017: Cancer Research
https://www.readbyqxmd.com/read/28107439/development-of-a-dipg-orthotopic-model-in-mice-using-an-implantable-guide-screw-system
#4
Miguel Marigil, Naiara Martinez-Velez, Pablo D Domínguez, Miguel Angel Idoate, Enric Xipell, Ana Patiño-García, Marisol Gonzalez-Huarriz, Marc García-Moure, Marie-Pierre Junier, Hervé Chneiweiss, Elías El-Habr, Ricardo Diez-Valle, Sonia Tejada-Solís, Marta M Alonso
OBJECTIVE: In this work we set to develop and to validate a new in vivo frameless orthotopic Diffuse Intrinsic Pontine Glioma (DIPG) model based in the implantation of a guide-screw system. METHODS: It consisted of a guide-screw also called bolt, a Hamilton syringe with a 26-gauge needle and an insulin-like 15-gauge needle. The guide screw is 2.6 mm in length and harbors a 0.5 mm central hole which accepts the needle of the Hamilton syringe avoiding a theoretical displacement during insertion...
2017: PloS One
https://www.readbyqxmd.com/read/28107254/selective-unidirectional-horizontal-saccadic-paralysis-from-acute-ipsilateral-pontine-stroke-response
#5
Joshua M Kruger, Juan M Sanchez, Ruth Eliahou, Marc Gotkine
No abstract text is available yet for this article.
January 19, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28104360/brown-colored-buccal-mucosa-associated-with-methemoglobinemia-cyanosis-appears-different-in-varying-ethnic-groups
#6
Rafiq Kanji, Hank Joseph Schneider, Mark Pontin
No abstract text is available yet for this article.
January 16, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28104248/continuous-hemifacial-myokymia-as-the-revealing-symptom-of-demyelinating-disease-of-the-cns
#7
Frédéric London, Nawal Hadhoum, Hélène Zéphir, Patrick Vermersch, Olivier Outteryck
Facial myokymia (FM) is an uncommon involuntary movement, disorder of the musculature supplied by the facial nerve and, characterized by spontaneous undulating, vermicular movements beneath the, skin. It has rarely been described as a form of presentation of multiple, sclerosis. We describe a 31-year-old man presenting with continuous, unilateral facial myokymia as the revealing symptom of a demyelinating, disorder of central nervous system. Brain magnetic resonance imaging, showed an ipsilateral pontine T2/FLAIR hyperintensity close to the, postgenu course of facial nerve, suggestive of a segmental demyelination, of facial nerve causing facial nuclear hyperactivity and resulting in FM...
January 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28099181/adverse-events-and-risk-factors-during-emergency-intubation-in-a-tertiary-paediatric-emergency-department
#8
Ruth M C Löllgen, Jennifer Pontin, Matthew Gow, Mary E McCaskill
BACKGROUND: Rapid sequence intubation and emergency intubation in the emergency department (ED) can be life-saving procedures, but require the appropriate skills, experience and preparation to avoid complications ranging from simple trauma to life-threatening desaturation. Only scarce data exist in the published literature on complications following emergency intubation in children and most guidelines are extrapolated from the adult population. PATIENTS AND METHODS: We reviewed all emergency intubations of patients in our tertiary paediatric ED within a 2-year period to estimate the incidence of complications and to analyse the risk factors associated with this procedure...
January 17, 2017: European Journal of Emergency Medicine: Official Journal of the European Society for Emergency Medicine
https://www.readbyqxmd.com/read/28093680/the-international-diffuse-intrinsic-pontine-glioma-registry-an-infrastructure-to-accelerate-collaborative-research-for-an-orphan-disease
#9
Joshua Baugh, Ute Bartels, James Leach, Blaise Jones, Brooklyn Chaney, Katherine E Warren, Jenavieve Kirkendall, Renee Doughman, Cynthia Hawkins, Lili Miles, Christine Fuller, Tim Hassall, Eric Bouffet, Adam Lane, Darren Hargrave, Jacques Grill, Lindsey M Hoffman, Chris Jones, Alex Towbin, Sharon A Savage, Michelle Monje, Xiao-Nan Li, David S Ziegler, Sophie Veldhuijzen van Zanten, Christof M Kramm, Dannis G van Vuurden, Maryam Fouladi
Diffuse intrinsic pontine glioma (DIPG), a rare, often fatal childhood brain tumor, remains a major therapeutic challenge. In 2012, investigators, funded by the DIPG Collaborative (a philanthropic partnership among 29 private foundations), launched the International DIPG Registry (IDIPGR) to advance understanding of DIPG. Comprised of comprehensive deidentified but linked clinical, imaging, histopathological, and genomic repositories, the IDIPGR uses standardized case report forms for uniform data collection; serial imaging and histopathology are centrally reviewed by IDIPGR neuro-radiologists and neuro-pathologists, respectively...
January 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28093369/rehabilitation-of-the-pontine-ataxia-dysmetria-syndrome-a-case-presentation
#10
Stephanie Li, Kasondra Hartman, Krishna Surapaneni, Eric L Altschuler
We present a case of a patient with significant ataxia and dysmetria following a lacunar pontine infarction and review the literature on this uncommon syndrome. The patient had an excellent clinical course with near resolution of symptoms and signs in less than three weeks. We characterize and illustrate with videos of the ataxia and dysmetria. Interestingly, the characteristics of the dysmetria appear to be different than that seen in patients with dysmetria arising from a cerebellar or thalamic lesion. We discuss the likely neurophysiologic mechanisms responsible for the condition and recovery...
January 13, 2017: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/28089558/startle-suppression-after-mild-traumatic-brain-injury-is-associated-with-an-increase-in-pro-inflammatory-cytokines-reactive-gliosis-and-neuronal-loss-in-the-caudal-pontine-reticular-nucleus
#11
Swamini P Sinha, Pelin Avcu, Kevin M Spiegler, Sreeya Komaravolu, Kevin Kim, Tara Cominski, Richard J Servatius, Kevin C H Pang
Mild traumatic brain injury (mTBI) can produce somatic symptoms such as headache, dizziness, fatigue, sleep disturbances and sensorimotor dysfunction. Sensorimotor function can be measured by tests such as the acoustic startle reflex (ASR), an evolutionarily conserved defensive response to a brief yet sharp acoustic stimulus. mTBI produces a long-lasting suppression of ASR in rodents and humans; however, the mechanism of this suppression is unknown. The present study examined whether inflammatory processes in the brainstem (particularly the caudal pontine reticular nucleus, PnC) could account for the suppression of ASR after mTBI, because the PnC is an essential nucleus of the ASR circuit...
January 9, 2017: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/28087826/comparison-of-brain-mri-findings-with-language-and-motor-function-in-the-dystroglycanopathies
#12
Brianna N Brun, Shelley R H Mockler, Katie M Laubscher, Carrie M Stephan, Anne M Wallace, Julia A Collison, M Bridget Zimmerman, William B Dobyns, Katherine D Mathews
OBJECTIVE: To describe the spectrum of brain MRI findings in a cohort of individuals with dystroglycanopathies (DGs) and relate MRI results to function. METHODS: All available brain MRIs done for clinical indications on individuals enrolled in a DG natural history study (NCT00313677) were reviewed. Reports were reviewed when MRI was not available. MRIs were categorized as follows: (1) cortical, brainstem, and cerebellar malformations; (2) cortical and cerebellar malformations; or (3) normal...
January 13, 2017: Neurology
https://www.readbyqxmd.com/read/28077515/medial-prefrontal-cortex-pontine-nuclei-projections-modulate-suboptimal-cue-induced-associative-motor-learning
#13
Guang-Yan Wu, Shu-Lei Liu, Juan Yao, Lin Sun, Bing Wu, Yi Yang, Xuan Li, Qian-Quan Sun, Hua Feng, Jian-Feng Sui
Diverse and powerful mechanisms have evolved to enable organisms to modulate learning and memory under a variety of survival conditions. Cumulative evidence has shown that the prefrontal cortex (PFC) is closely involved in many higher-order cognitive functions. However, when and how the medial PFC (mPFC) modulates associative motor learning remains largely unknown. Here, we show that delay eyeblink conditioning (DEC) with the weak conditioned stimulus (wCS) but not the strong CS (sCS) elicited a significant increase in the levels of c-Fos expression in caudal mPFC...
January 10, 2017: Cerebral Cortex
https://www.readbyqxmd.com/read/28072702/hypersomnia-due-to-injury-of-the-ventral-ascending-reticular-activating-system-following-cerebellar-herniation-a-case-report
#14
Sung Ho Jang, Chul Hoon Chang, Young Jin Jung, Hyeok Gyu Kwon
RATIONALE: We report on a patient with hypersomnia who showed injury of the lower ascending reticular activating system (ARAS) following cerebellar herniation due to a cerebellar infarct, detected on diffusion tensor tractography (DTT). PATIENT CONCERNS: A 53-year-old male patient was diagnosed as a left cerebellar infarct, and underwent decompressive suboccipital craniectomy due to brain edema at 2 days after the onset of a cerebellar infarct. Three weeks after onset when the patient started rehabilitation, he showed hypersomnia without impairment of consciousness; he fell asleep most of daytime without external stimulation and showed an abnormal score on the Epworth Sleepiness Scale: 15 (full score: 24, cut off for hypersomnia: 10)...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28068949/plaque-distribution-of-low-grade-basilar-artery-atherosclerosis-and-its-clinical-relevance
#15
Jin Yu, Ming-Li Li, Yu-Yuan Xu, Shi-Wen Wu, Min Lou, Xue-Tao Mu, Feng Feng, Shan Gao, Wei-Hai Xu
BACKGROUND: The underlying pathophysiology of BA distribution is unclear and intriguing. Using high-resolution magnetic resonance imaging (HR-MRI), we sought to explore the plaque distribution of low-grade basilar artery (BA) atherosclerosis and its clinical relevance. METHODS: We retrospectively analyzed the imaging and clinical data of 61 patients with low-grade atherosclerotic BA stenosis (<50%). On HR-MRI, the plaques were categorized based on the involvement of the ventral, dorsal, or lateral sides of BA wall...
January 10, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28068928/newly-recognized-cerebral-infarctions-on-postmortem-imaging-a-report-of-three-cases-with-systemic-infectious-disease
#16
Sakon Noriki, Kazuyuki Kinoshita, Kunihiro Inai, Toyohiko Sakai, Hirohiko Kimura, Takahiro Yamauchi, Masayuki Iwano, Hironobu Naiki
BACKGROUND: Postmortem imaging (PMI) refers to the imaging of cadavers by computed tomography (CT) and/or magnetic resonance imaging (MRI). Three cases of cerebral infarctions that were not found during life but were newly recognized on PMI and were associated with severe systemic infections are presented. CASE PRESENTATIONS: An 81-year-old woman with a pacemaker and slightly impaired liver function presented with fever. Imaging suggested interstitial pneumonia and an iliopsoas abscess, and blood tests showed liver dysfunction and disseminated intravascular coagulation (DIC)...
January 10, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28061497/cerebellar-hemorrhage-due-to-a-direct-carotid-cavernous-fistula-after-surgery-for-maxillary-cancer
#17
Yoshinobu Kamio, Hisaya Hiramatsu, Mika Kamiya, Shuhei Yamashita, Hiroki Namba
Infratentorial cerebral hemorrhage due to a direct carotid-cavernous fistula (CCF) is very rare. To our knowledge, only four such cases have been reported. Cerebellar hemorrhage due to a direct CCF has not been reported. We describe a 63-year-old female who presented with reduced consciousness 3 days after undergoing a maxillectomy for maxillary cancer. Computed tomography showed a cerebellar hemorrhage. Magnetic resonance angiography showed a left-sided direct CCF draining into the left petrosal and cerebellar veins through the left superior petrosal sinus (SPS)...
January 1, 2017: Journal of Korean Neurosurgical Society
https://www.readbyqxmd.com/read/28052119/pre-clinical-study-of-panobinostat-in-xenograft-and-genetically-engineered-murine-diffuse-intrinsic-pontine-glioma-models
#18
Tammy Hennika, Guo Hu, Nagore G Olaciregui, Kelly L Barton, Anahid Ehteda, Arjanna Chitranjan, Cecilia Chang, Andrew J Gifford, Maria Tsoli, David S Ziegler, Angel M Carcaboso, Oren J Becher
BACKGROUND: Diffuse intrinsic pontine glioma (DIPG), or high-grade brainstem glioma (BSG), is one of the major causes of brain tumor-related deaths in children. Its prognosis has remained poor despite numerous efforts to improve survival. Panobinostat, a histone deacetylase inhibitor, is a targeted agent that has recently shown pre-clinical efficacy and entered a phase I clinical trial for the treatment of children with recurrent or progressive DIPG. METHODS: A collaborative pre-clinical study was conducted using both a genetic BSG mouse model driven by PDGF-B signaling, p53 loss, and ectopic H3...
2017: PloS One
https://www.readbyqxmd.com/read/28051280/tongue-somatosensory-evoked-potentials-reflect-midbrain-involvement-in-patients-with-clinically-isolated-syndrome
#19
Magdalena Krbot Skorić, Ivan Adamec, Luka Crnošija, Tereza Gabelić, Barbara Barun, Ivana Zadro, Silva Butković Soldo, Mario Habek
AIM: To test the hypothesis that tSSEP findings reflect clinical and MRI MS lesions, the aim of this study was to investigate tSSEP changes in patients with clinically isolated syndrome (CIS) in relation to clinical and brainstem MRI findings. The second aim was to investigate whether the interpretation of the tSSEP results in the form of the tSSEP score enables better evaluation of the afferent trigeminal pathway involvement than analyzing each tSSEP parameter separately. METHODS: 115 consecutive CIS patients were enrolled from August 1, 2014 until March 1, 2016...
December 31, 2016: Croatian Medical Journal
https://www.readbyqxmd.com/read/28043883/trigeminal-ganglioneuroma-a-rare-cause-of-trigeminal-neuralgia-caused-by-cerebello-pontine-angle-tumor
#20
Nadeem Khan, Alex Michael, Ali Choucair, Esther Bit-Ivan
Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in literature so far. Out of those reported, all have been recognized in Eastern Asia. We report a rare case of trigeminal ganglioneuroma in a 52-year-old Caucasian male presenting with right trigeminal neuralgia.
December 30, 2016: World Neurosurgery
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