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cystic cns

D Diaz-Aguilar, T Niu, S Terterov, R Scharnweber, A Tucker, J Woodard, H Brara, C Merna, H Shah, S Wang, S Rahman
Background: Neurenteric cysts (NECs) are rare developmental malformations of the central nervous system (CNS) which originate as benign congenital lesions. They originate from developmental foregut precursors, and are presumed to be the result of abnormal partitioning of the embryonic notochord plate. Such NECs predominantly arise in the cervical region in patients around 6 years of age or in their twenties or thirties. Notably, NECs of the conus medullaris are exceedingly rare, especially in patients of advanced age...
2018: Surgical Neurology International
Federico Bianchi, Gianpiero Tamburrini, Marco Gessi, Paolo Frassanito, Luca Massimi, Massimo Caldarelli
BACKGROUND: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs. CASE DESCRIPTION: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus...
March 8, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Jinxiu Cai, Wanlan Li, Jiang Du, Nini Xu, Peiyi Gao, Jian Zhou, Xiaofeng Li
RATIONALE: Cerebellar liponeurocytoma is a rare tumor of the central nervous system (CNS) characterized by low proliferation but high likelihood of recurrence. Because of its rarity and the paucity of systematic follow-up, the biological behaviors and clinical features of this tumor are still poorly understood. We herein reported a case of cerebellar liponeurocytoma originating in the cerebral hemisphere. PATIENT CONCERNS: A 11-year-old male with intermittent headache, nausea, and vomiting...
January 2018: Medicine (Baltimore)
Houda Nasser Al Yaqoubi, Nishat Fatema
Meckel-Gruber syndrome (MGS) is a rare and lethal ciliopathic disorder, with the incidence ranging between 1 in 13 000-400 000 live births. MGS is characterized by multisystem developmental malformations with the classical features of renal cystic dysplasia, occipital encephalocele and post-axial polydactyly. Except for occipital encephalocele, the CNS abnormalities associated with MGS that are less frequently reported include hydrocephaly, anencephaly or malformation of cerebellum. Our presented case of MGS is associated with anencephaly and other facial abnormalities...
February 2018: Oxford Medical Case Reports
Kris Ann P Schultz, Gretchen M Williams, Junne Kamihara, Douglas R Stewart, Anne K Harris, Andrew J Bauer, Joyce Turner, Rachana Shah, Katherine Schneider, Kami Wolfe Schneider, Ann Garrity Carr, Laura A Harney, Shari Baldinger, A Lindsay Frazier, Daniel Orbach, Dominik T Schneider, David Malkin, Louis P Dehner, Yoav H Messinger, Ashley Hill
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma and brain tumors including pineoblastoma and pituitary blastoma...
January 17, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Gökhan Çavuş, Vedat Açik, Yeliz Çavuş, Emre Bilgin, Yurdal Gezercan, Ali Ihsan Ökten
PURPOSE: A hydatid cyst is a parasitic illness that is caused by the larvae of Echinococcus granulosus. Hydatid cysts occur in the liver in 75% and in the lungs in 15% of cases. Central nervous system involvement is rare (perhaps as low as 1%), and the majority of such cases are observed in children. Headache and vomiting are the most frequently observed symptoms. In patients diagnosed with a hydatid cyst, imaging methods and serologic tests are very important for identifying cranial involvement...
January 5, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Maria Claudia Jurcă, Kinga Kozma, CodruŢa Diana Petcheşi, Marius Bembea, Ovidiu Laurean Pop, Gabriela MuŢiu, Mihaela Cristiana Coroi, Alexandru Daniel Jurcă, Luciana Dobjanschi
Dandy-Walker complex (DWC) is a malformative association of the central nervous system. DWC includes four different types: Dandy-Walker malformation (vermis agenesis or hypoplasia, cystic dilatation of the fourth ventricle and a large posterior fossa); Dandy-Walker variant (vermis hypoplasia, cystic dilatation of the fourth ventricle, normal posterior fossa); mega cysterna magna (large posterior fossa, normal vermis and fourth ventricle) and posterior fossa arachnoid cyst. We present and discuss four cases with different morphological and clinical forms of the Dandy-Walker complex...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Lindsay Edwards, Lisa Hui
Fetal structural anomalies are found in up to 3% of all pregnancies and ultrasound-based screening has been an integral part of routine prenatal care for decades. The prenatal detection of fetal anomalies allows for optimal perinatal management, providing expectant parents with opportunities for additional imaging, genetic testing, and the provision of information regarding prognosis and management options. Approximately one-half of all major structural anomalies can now be detected in the first trimester, including acrania/anencephaly, abdominal wall defects, holoprosencephaly and cystic hygromata...
December 9, 2017: Seminars in Fetal & Neonatal Medicine
Ruqaiah Altassan, Peter Witters, Zubaida Saifudeen, Dulce Quelhas, Jaak Jaeken, Elena Levtchenko, David Cassiman, Eva Morava
Phosphomannomutase 2 deficiency (PMM2-CDG) is the most common N-linked glycosylation disorder. The majority of patients present with a multisystem phenotype, including central nervous system involvement, hepatopathy, gastrointestinal and cardiac symptoms, endocrine dysfunction and abnormal coagulation. Renal abnormalities including congenital malformations and altered renal function are part of the multisystem manifestations of congenital disorders of glycosylation. We reviewed the literature on 933 patients with molecularly and/or enzymatically confirmed PMM2 deficiency to evaluate the incidence of renal involvement in PMM2-CDG...
March 2018: Molecular Genetics and Metabolism
Chris Gale, Yevgeniy Statnikov, Sena Jawad, Sabita N Uthaya, Neena Modi
OBJECTIVE: In 2015, the Department of Health in England announced an ambition to reduce 'brain injuries occurring during or soon after birth'. We describe the development of a pragmatic case definition and present annual incidence rates. DESIGN: Retrospective cohort study using data held in the National Neonatal Research Database (NNRD) extracted from neonatal electronic patient records from all National Health Service (NHS) neonatal units in England, Wales and Scotland...
October 22, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
F Ahlhelm, K Shariat, S Götschi, S Ulmer
CLINICAL PROBLEM: Intracerebral cysts are common findings in imaging of the neurocranium and are not always clinically significant. The pathological spectrum of intracerebral cysts is, however, very broad and in addition to incidental findings includes developmental disorders, malformation tumors, primary and secondary neoplasms and infectious etiologies, such as cerebral abscess formation, cysticercosis or residuals after congenital cytomegalovirus infections. Intracerebral cystic defects may be caused by inflammatory central nervous system (CNS) diseases, such as multiple sclerosis as well as by mitochondriopathies, leukodystrophy, electrolyte disturbances or osmotic demyelination syndrome or brain infarctions, e...
February 2018: Der Radiologe
Amber Marie Vasquez, D Zavasky, N A Chow, L Gade, E Zlatanic, S Elkind, A P Litvintseva, Peter G Pappas, J R Perfect, S G Revankar, S R Lockhart, T M Chiller, J Ackelsberg, S Vallabhaneni
Exophiala (Wangiella) dermatitidis is a dematiaceous fungus that can grow in yeast or mold forms and is typically found in decaying organic matter. It can cause central nervous system disease, particularly in immunocompromised patients, and has been implicated as a respiratory pathogen in cystic fibrosis patients [1,2]. It has also been identified as a colonizer in the gastrointestinal tract [3]. However, bloodstream infections with this organism are exceedingly rare.
November 7, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
Chris Gale, Yevgeniy Statnikov, Sena Jawad, Sabita N Uthaya, Neena Modi
OBJECTIVE: In 2015, the Department of Health in England announced an ambition to reduce 'brain injuries occurring during or soon after birth'. We describe the development of a pragmatic case definition and present annual incidence rates. DESIGN: Retrospective cohort study using data held in the National Neonatal Research Database (NNRD) extracted from neonatal electronic patient records from all National Health Service (NHS) neonatal units in England, Wales and Scotland...
October 22, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
Yosef Laviv, Joshua L Wang, Matthew P Anderson, Ekkehard M Kasper
Hemangioblastomas (HBs) are benign, highly vascular tumors, often characterized by loss of function of the von Hippel-Lindau (vHL) gene. They are the most common central nervous system tumor observed in vHL syndrome. Loss of function of the vHL gene creates a "pseudo-hypoxic" state, causing overactivation of hypoxia-inducible factor (HIF) and vascular endothelial growth factor (VEGF)-related pathways. In some cases, HBs can rapidly increase in size during pregnancy to then present acutely, which most frequently occurs after the 20th gestational week...
October 13, 2017: Neurosurgical Review
Martín Brízuela, Claudia Sarkis, Roberto González, Patricia Paulin, Fabiana Lubieniecki, Griselda Berberian
Hydatid disease is a parasitic infection whose etiologic agent is Echinococcus granulosus. Human is an accidental intermediate host and the most common site is the liver. The brain involvement is unusual and up to 75% of cases are described in the pediatric population. We present six children with cerebral hydatid disease admitted to the Pediatric Hospital J.P. Garrahan. All had neurological involvement on admission. The images showed single cystic lesion in the brain. They did not present involvement in other organs...
June 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
Takanori Hirose, Sumihito Nobusawa, Kazuhiko Sugiyama, Vishwa J Amatya, Naomi Fujimoto, Atsushi Sasaki, Yoshiki Mikami, Akiyoshi Kakita, Shinya Tanaka, Hideaki Yokoo
Astroblastoma is a rare, enigmatic tumor of the central nervous system (CNS) which shares some clinicopathologic aspects with other CNS tumors, especially ependymoma. To further clarify the nature of astroblastoma, we performed clinicopathologic and molecular genetic studies on eight cases of astroblastoma. The median age of the patients was 14.5 years, ranging from 5 to 60 years, and seven of the patients were female. All tumors arose in the cerebral hemisphere and radiologically appeared to be well-bordered, nodular tumors often associated with cystic areas and contrast-enhancement...
October 9, 2017: Brain Pathology
Matthew P Walker, Matt Cowlen, Dale Christensen, Mutsumi Miyamoto, Phillip Barley, Timothy Crowder
BACKGROUND: ENaC inhibition has long been an attractive therapeutic target for the treatment of cystic fibrosis. However, previous attempts at developing ENaC inhibitors have been unsuccessful due to complications arising from systemic circulation of the compounds. Here, we describe the preclinical toxicology assessment of a new inhaled peptide promoter of ENaC internalization delivered as a nebulized aerosol. METHODS: Preclinical assessment of SPX-101 safety was determined using an in vitro hERG assay, bolus injection of SPX-101 in a canine cardiovascular and respiratory safety pharmacology model and 28-day inhalation toxicology studies of nebulized drug in rats and dogs...
October 6, 2017: Inhalation Toxicology
Hirokazu Takami, Christopher S Graffeo, Avital Perry, Aditya Raghunathan, Robert B Jenkins, Caterina Giannini, Terry C Burns
BACKGROUND: Giant cell ependymoma (GCE) is a rare primary central nervous system neoplasm. We report a case of GCE arising in the lateral ventricle. CASE DESCRIPTION: A 22-year-old female presented with generalized seizures. Magnetic resonance imaging demonstrated a diffuse, nonenhancing, multicystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE...
December 2017: World Neurosurgery
Le Thi Anh Hong, Young-Min Kim, Hee Hwan Park, Dong Hoon Hwang, Yuexian Cui, Eun Mi Lee, Stephanie Yahn, Jae K Lee, Soo-Chang Song, Byung Gon Kim
The cystic cavity that develops following injuries to brain or spinal cord is a major obstacle for tissue repair in central nervous system (CNS). Here we report that injection of imidazole-poly(organophosphazenes) (I-5), a hydrogel with thermosensitive sol-gel transition behavior, almost completely eliminates cystic cavities in a clinically relevant rat spinal cord injury model. Cystic cavities are bridged by fibronectin-rich extracellular matrix. The fibrotic extracellular matrix remodeling is mediated by matrix metalloproteinase-9 expressed in macrophages within the fibrotic extracellular matrix...
September 14, 2017: Nature Communications
Ryouhei Matsuoka, Jun Muneuchi, Yusaku Nagatomo, Daisuke Shimizu, Seigo Okada, Chiaki Iida, Hiromitsu Shirouzu, Mamie Watanabe, Yasuhiko Takahashi, Haruhiko Maruyama
An 11-year-old boy collapsed during morning assembly at his junior high school. The automated external defibrillator detected ventricular fibrillation and provided shock delivery. He was successfully resuscitated and reverted to sinus rhythm. Electrocardiography showed ST-T elevation in the precordial leads. Echocardiography and angiography demonstrated akinesia of the apex and mid-wall of the left ventricle with preserved contraction of the basal segments, which suggested Takotsubo cardiomyopathy. The patient and his family had often eaten uncooked crab, and his father had a past history of infection with Paragonimiasis westermani...
September 8, 2017: Paediatrics and International Child Health
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