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Leptomeningeal disease

Chenhui Mao, Jing Gao, Liri Jin, Bin Peng, Yupu Guo
Neurosyphilis occurs in the late stage of systemic syphilis infection; early diagnosis and treatment are crucial to the prognosis. We review 3 autopsy cases with different subtypes of neurosyphilis, that is cases with meningovascular, general paresis, and a combination of the 2, respectively. We investigated the gross morphology and leptomeninges, vessels, cerebral cortex, white matter, brainstem, cerebellum, olfactory bulb and spinal cord microscopically. We found that meningovascular inflammation exists in both early and late phases of neurosyphilis, not only in the meningovascular subtype...
March 12, 2018: Journal of Neuropathology and Experimental Neurology
A Sekhar, B Corbo, K Das, S Biswas
Leptomeningeal carcinomatosis is a devastating condition with very poor prognosis. With advances in cancer treatment leading to improved survival in solid tumours, the incidence of ante-mortem diagnosis of leptomeningeal carcinomatosis is likely to rise. Without a high index of suspicion and appropriate imaging the diagnosis can be missed. We report two cases of leptomeningeal carcinomatosis secondary to metastatic breast disease with varied clinical presentation where the diagnosis was initially missed.
December 2017: Journal of the Royal College of Physicians of Edinburgh
Alessia Pellerino, Luca Bertero, Roberta Rudà, Riccardo Soffietti
Neoplastic meningitis (NM) is a devastating complication of solid tumors with poor outcome. Some randomized clinical trials have been conducted with heterogeneous inclusion criteria, diagnostic parameters, response evaluation and primary endpoints. Recently, the Leptomeningeal Assessment in Neuro-Oncology (LANO) Group and the European Society for Medical Oncology/European Association for Neuro-Oncology have proposed some recommendations in order to provide diagnostic criteria and response evaluation scores for NM...
2018: Therapeutic Advances in Neurological Disorders
Peter Y M Woo, James T F Zhuang, Jason M K Ho, Andrew Seto, Hoi-Tung Wong, Kwong-Yau Chan
Leptomeningeal spread and hydrocephalus are increasingly recognized as late disease complications of glioblastoma with almost a quarter of patients requiring early cerebrospinal fluid shunting. The neurosurgeon is challenged with maintaining shunt patency when tumor disease progression is rapid and adjuvant oncologic therapy has yet to be initiated. We describe our experience in treating a young female with diffuse glioblastoma leptomeningeal spread and communicating hydrocephalus who had several episodes of shunt obstruction due to intraluminal tumor cell-fibrin deposits...
March 13, 2018: Acta Neurochirurgica
Ana Luiza Miranda Silva Dias, Fabiana Higashi, Ana Lúcia M Peres, Pricilla Cury, Edvan de Queiroz Crusoé, Vânia Tietsche de Moraes Hungria
INTRODUCTION: The estimated involvement of the central nervous system in patients with multiple myeloma is rare at about 1%. The infiltration can be identified at the time multiple myeloma is diagnosed or during its progression. However, it is more common in refractory disease or during relapse. METHODS: This retrospective cohort study reviewed data from medical records of patients followed up at the Gammopathy Outpatient Clinic of Santa Casa de Misericórdia de São Paulo from January 2008 to December 2016...
January 2018: Revista Brasileira de Hematologia e Hemoterapia
Rola A Mahmoud, Charles K Abrams
Peripheral neurological complications of lymphomas are rare and much less frequent than central complications. Nonetheless, on occasion, systemic non-Hodgkin's lymphoma may directly infiltrate the peripheral nervous system at various levels. This report describes a man with non-Hodgkin's lymphoma and leptomeningeal disease who developed progressive areflexic quadraparesis. Initial electromyography (EMG) was consistent with a polyradiculopathy and a repeat EMG performed 1 month later for worsening symptoms showed evidence of demyelination...
March 5, 2018: BMJ Case Reports
Manuel Valiente, Manmeet S Ahluwalia, Adrienne Boire, Priscilla K Brastianos, Sarah B Goldberg, Eudocia Q Lee, Emilie Le Rhun, Matthias Preusser, Frank Winkler, Riccardo Soffietti
Metastasis, involving the spread of systemic cancer to the brain, results in neurologic disability and death. Current treatments are largely palliative in nature; improved therapeutic approaches represent an unmet clinical need. However, recent experimental and clinical advances challenge the bleak long-term outcome of this disease. Encompassing key recent findings in epidemiology, genetics, microenvironment, leptomeningeal disease, neurocognition, targeted therapy, immunotherapy, and prophylaxis, we review preclinical and clinical studies to provide a comprehensive picture of contemporary research and the management of secondary brain tumors...
March 2018: Trends in Cancer
Elena Vetlova, Denis A Golbin, Andrey V Golanov, Alexander A Potapov, Sergey M Banov, Natalia Antipina, Valery V Kostjuchenko, Dmitry Y Usachev, Artem Y Belyaev, Sergey Goryaynov
INTRODUCTION:  Preoperative stereotactic radiosurgery (pre-SRS) is a recent advancement in the strategy for brain metastasis (BM) management, and available data demonstrate the advantages of pre-SRS before postoperative radiation treatment, including lower rates of local toxicity, leptomeningeal progression, and a high percentage of local control. The authors presented the results of pre-SRS in patients with BM. MATERIALS AND METHODS: Nineteen patients with BM (11 female and eight male) have been treated at N...
December 26, 2017: Curēus
Emmet J Jordan, Maeve A Lowery, Olca Basturk, Peter J Allen, Kenneth H Yu, Viviane Tabar, Kathryn Beal, Diane L Reidy, Yoshiya Yamada, Yelena Janjigian, Ghassan K Abou-Alfa, Eileen M O'Reilly
PURPOSE: To assess clinical characteristics of patients with metastatic pancreas ductal adenocarcinoma (PDAC) and brain metastases (BM), and to assess somatic and germ-line molecular profiles where performed. PATIENTS AND METHODS: Patients with PDAC and BM between January 1990 and January 2016 were identified. Molecular characteristics of somatic and germ-line testing where performed in the subset of patients who had provided informed consent. Somatic alterations were assessed by either MSK-IMPACT testing (>340 key cancer genes) or Sequenom testing (8-gene panel)...
February 7, 2018: Clinical Colorectal Cancer
Lauren R Schaff, Christian Grommes
PURPOSE OF REVIEW: Primary central nervous system lymphoma (PCNSL) is an aggressive malignancy confined to the brain, spinal cord, leptomeninges, and eyes. Due to its rarity, there is a paucity of randomized trials and a varied approach to its management in the oncologic community. This review summarizes recent literature guiding current clinical practice. RECENT FINDINGS: The presentation, work up, and management of PCNSL are discussed. Induction therapy incorporates a methotrexate-based chemotherapy regimen and is generally followed by a consolidation regimen including high dose chemotherapy (with or without autologous stem cell rescue)...
February 28, 2018: Current Oncology Reports
Austin T Kerns, Kelsey A Brakel, Christopher Premanandan, Ashlie Saffire, Sarah A Moore
Case summary: A 9-year-old spayed female domestic shorthair cat with clinical signs suggestive of chronic recurrent otitis media and recent seizures was presented with multifocal nervous system disease, including bilateral central and/or peripheral vestibular, cerebellar and forebrain deficits. Prior to presentation, there was inadequate improvement after 6 weeks of treatment for bilateral middle ear effusion from which a highly susceptible Staphylococcus species was cultured. This was followed by the development of seizures...
January 2018: JFMS Open Reports
Ethan B Ludmir, Arnold C Paulino, David R Grosshans, Mary Frances McAleer, Susan L McGovern, Winston W Huh, M Fatih Okcu, Leslie M Harrell, Anita Mahajan
PURPOSE: To assess clinical outcomes and patterns of failure, particularly regional nodal control, for pediatric patients treated with proton beam therapy (PBT) for head and neck alveolar rhabdomyosarcoma (HN-ARMS). MATERIALS AND METHODS: Between 2006 and 2015, 14 patients with HN-ARMS were enrolled in a prospective registry protocol and treated with PBT at a single institution. Of the patients, 8 (57%) presented with localized disease and 6 (43%) with regional nodal metastases...
January 31, 2018: International Journal of Radiation Oncology, Biology, Physics
Ai Peng Tan, Wui Khean Chong
BACKGROUND: Apert syndrome is one of the most common craniosynostosis syndrome caused by mutation in genes encoding fibroblast growth factor receptor 2 (FGFR2). Craniosynostosis, midfacial hypoplasia, and syndactyly/symphalangism are features of this syndrome. Sturge-Weber syndrome (SWS) on the other hand is a congenital neurocutaneous disorder characterized by facial port-wine stains (PWSs) and leptomeningeal vascular capillary malformations. In 2013, the causative mutation underlying SWS (p...
February 23, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Livia Garzia, Noriyuki Kijima, A Sorana Morrissy, Pasqualino De Antonellis, Ana Guerreiro-Stucklin, Borja L Holgado, Xiaochong Wu, Xin Wang, Michael Parsons, Kory Zayne, Alex Manno, Claudia Kuzan-Fischer, Carolina Nor, Laura K Donovan, Jessica Liu, Lei Qin, Alexandra Garancher, Kun-Wei Liu, Sheila Mansouri, Betty Luu, Yuan Yao Thompson, Vijay Ramaswamy, John Peacock, Hamza Farooq, Patryk Skowron, David J H Shih, Angela Li, Sherine Ensan, Clinton S Robbins, Myron Cybulsky, Siddhartha Mitra, Yussanne Ma, Richard Moore, Andy Mungall, Yoon-Jae Cho, William A Weiss, Jennifer A Chan, Cynthia E Hawkins, Maura Massimino, Nada Jabado, Michal Zapotocky, David Sumerauer, Eric Bouffet, Peter Dirks, Uri Tabori, Poul H B Sorensen, Priscilla K Brastianos, Kenneth Aldape, Steven J M Jones, Marco A Marra, James R Woodgett, Robert J Wechsler-Reya, Daniel W Fults, Michael D Taylor
While the preponderance of morbidity and mortality in medulloblastoma patients are due to metastatic disease, most research focuses on the primary tumor due to a dearth of metastatic tissue samples and model systems. Medulloblastoma metastases are found almost exclusively on the leptomeningeal surface of the brain and spinal cord; dissemination is therefore thought to occur through shedding of primary tumor cells into the cerebrospinal fluid followed by distal re-implantation on the leptomeninges. We present evidence for medulloblastoma circulating tumor cells (CTCs) in therapy-naive patients and demonstrate in vivo, through flank xenografting and parabiosis, that medulloblastoma CTCs can spread through the blood to the leptomeningeal space to form leptomeningeal metastases...
February 22, 2018: Cell
Shannon E Rose, Harald Frankowski, Allison Knupp, Bonnie J Berry, Refugio Martinez, Stephanie Q Dinh, Lauren T Bruner, Sherry L Willis, Paul K Crane, Eric B Larson, Thomas Grabowski, Martin Darvas, C Dirk Keene, Jessica E Young
Patient-specific stem cell technology from skin and other biopsy sources has transformed in vitro models of neurodegenerative disease, permitting interrogation of the effects of complex human genetics on neurotoxicity. However, the neuropathologic changes that underlie cognitive and behavioral phenotypes can only be determined at autopsy. To better correlate the biology of derived neurons with age-related and neurodegenerative changes, we generated leptomeningeal cell lines from well-characterized research subjects that have undergone comprehensive postmortem neuropathologic examinations...
February 21, 2018: Journal of Neuropathology and Experimental Neurology
Hakyoung Kim, Do Hoon Lim, Tae Gyu Kim, Jung-Il Lee, Do-Hyun Nam, Ho Jun Seol, Doo-Sik Kong, Jung Won Choi, Yeon-Lim Suh, Sung Tae Kim
AIM: Leptomeningeal enhancement (LME) on preoperative brain magnetic resonance imaging (MRI) does not always indicate leptomeningeal seeding (LMS). With Stupp's regimen, authors have treated glioblastoma patients with LME on preoperative brain MRI but here we tried to find the clinical impact of LME. METHODS: From 2005 to 2015, 290 patients with glioblastoma have been treated with Stupp's regimen at Samsung Medical Center. Among these, 33 patients showed LME on preoperative brain MRI...
February 23, 2018: Asia-Pacific Journal of Clinical Oncology
Giang Huong Nguyen, Alexander Mohler, Michelle Leppert, Cindy Parra, Yue-Ping Zeng, Lori Prok, Jeffrey Schowinsky, Cynthia M Magro, Theresa Pacheco, Douglas Ney
Central nervous system involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages of the disease. We describe a patient with early-stage follicular MF who presented with changes in mental status. Despite an initial diagnosis of vasculitis based on clinical and brain biopsy results, the postmortem examination revealed extensive infiltration of MF cells throughout the brain with leptomeningeal involvement. This case in addition to the accompanied review of literature illustrates the importance of the awareness of central nervous system involvement by MF and highlights the need for an urgent neurologic evaluation in patients with a history of MF now presenting with neurologic signs or symptoms...
March 2018: American Journal of Dermatopathology
Sarah A Milgrom, Chelsea C Pinnix, T Linda Chi, Thinh H Vu, Jillian R Gunther, Tommy Sheu, Nathan Fowler, Jason R Westin, Loretta J Nastoupil, Yasuhiro Oki, Luis E Fayad, Sattva Neelapu, Maria Alma Rodriguez, Frederick B Hagemeister, Michelle A Fanale, Hun J Lee, Chitra Hosing, Sairah Ahmed, Yago Nieto, Elizabeth J Shpall, Bouthaina S Dabaja
PURPOSE: We assessed the efficacy of radiation therapy (RT) in the management of secondary central nervous system (CNS) lymphoma. METHODS AND MATERIALS: The cohort comprised 44 patients with systemic diffuse large B-cell lymphoma (DLBCL) secondarily involving the brain and/or leptomeninges at initial diagnosis or relapse that was treated with RT. RESULTS: Of these patients, 29 (66%) were in systemic remission when CNS disease was diagnosed...
January 6, 2018: International Journal of Radiation Oncology, Biology, Physics
Ana Maria Abreu-Velez, Wendy Gao, Michael S Howard
Background: The majority of the patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre EPF or pemphigus Abreu-Manu), have experienced vision problems; we have previously reported several ocular abnormalities. Methods: Here, we aimed to investigate reactivity to optic nerves in these patients. We utilized bovine, rat and mouse optic nerves, and performed immunofluorescence and confocal microscopy to test for optical nerve autoreactivity...
January 2018: Dermatology Practical & Conceptual
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
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