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tumefactive ms

S Abdullah, W F Wong, C T Tan
Background. There have been inconsistent reports on the prevalence and pathogenicity of anti-Aquaporin 4 (AQP4) in patients presented with idiopathic inflammatory demyelinating diseases (IIDDs). Objective. To estimate the prevalence of anti-AQP4 antibody in patients with IIDDs presented to University Malaya Medical Centre in terms of patients' clinical and radiological presentations and prognoses. Methods. Retrospective data review of IIDDs patients presented from 2005 to 2015. Patients were classified into classical multiple sclerosis (CMS), opticospinal (OS) presentation, optic neuritis (ON), transverse myelitis (TM), brainstem syndrome (BS), and tumefactive MS...
2017: Multiple Sclerosis International
Joshua Barton, Todd A Hardy, Sean Riminton, Stephen W Reddel, Yael Barnett, Alasdair Coles, Michael H Barnett
No abstract text is available yet for this article.
March 7, 2017: Neurology
Hisakazu Majima, Tsuyoshi Ito, Norihisa Koyama
Pediatric multiple sclerosis accompanied by a tumefactive demyelinating lesion (TDL) is extremely rare. Because it is very difficult to distinguish TDLs from neoplasms, invasive brain biopsies are required for a definitive diagnosis. MR spectroscopy (MRS) without brain biopsy was recently shown diagnostic in some patients with TDLs, based on the elevation of glutamate/glutamine peaks. This report describes the clinical course of a 9-year-old girl with multiple sclerosis following a TDL and discusses the usefulness of MRS and brain biopsies to diagnose TDLs...
February 25, 2017: Rinshō Shinkeigaku, Clinical Neurology
Masataka Nakamura, Kumi Itani, Kousuke Miyake, Takenobu Kunieda, Satoshi Kaneko, Hirofumi Kusaka
We herein report the case of a 57-year-old woman presenting with a biopsy-proven tumefactive demyelinating lesion as her first clinical event. Subsequently, she displayed a relapsing-remitting course with recurrence of large demyelinating lesions exceeding 2 cm in diameter rather than the small ovoid lesions characteristic of multiple sclerosis. Administration of interferon beta did not suppress the disease activity. Finally, treatment with natalizumab, which is a humanized monoclonal antibody against the cell-adhesion molecule α4-integrin, was initiated, resulting in clinical and radiological stabilization...
2017: Internal Medicine
Kristen Lew, Nishith Mewada, Sahana Ramanujam, Bahareh Hassanzadeh, John E Donahue, Leema Reddy Peddareddygari, Robert Moser, Charles Kososky, Raji P Grewal
We report a 35-year-old healthy male who developed central nervous system inflammatory demyelinating disease consistent with tumefactive multiple sclerosis. About 2 weeks after onset of symptoms and prior to initiation of therapy, the patient had lymphopenia and low CD4 and CD8 levels. His lymphocyte count was 400 cells/µl (850-3,900 cells/µl), CD4 was 193 cells/µl (490-1,740 cells/µl) and CD8 was 103 cells/µl (180-1,170 cells/µl). He was treated with intravenous methylprednisolone followed by therapeutic plasma exchange, the levels of CD4 and CD8 normalized, and ultimately, he recovered completely...
May 2016: Case Reports in Neurology
Carrie M Gould, Anjum Sayyad, Grace L Maloney
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Aleksi Tarkkonen, Eero Rissanen, Terhi Tuokkola, Laura Airas
We present a case where a 30-year-old man with a history of combined MS and Charcot-Marie-Tooth (CMT I) disease was additionally diagnosed and treated for grade II glioma (astrocytoma). Tumefactive MS and gliomas are sometimes difficult to distinguish from one another based on conventional magnetic resonance imaging (MRI). In our case, positron emission tomography (PET) scans with(11)C-methionine ((11)C-MET) and (11)C-PK11195 radioligands were performed to aid in differential diagnostics. The diagnosis was confirmed finally by brain biopsy...
September 2016: Multiple Sclerosis and related Disorders
Nicolae Sarbu, Robert Y Shih, Robert V Jones, Iren Horkayne-Szakaly, Laura Oleaga, James G Smirniotopoulos
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease)...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Renata Conforti, Raffaella Capasso, Rosario Galasso, Mario Cirillo, Gemma Taglialatela, Luigi Galasso
BACKGROUND: Tumefactive multiple sclerosis (MS) is an unusual variant of demyelinating disease characterized by lesions with pseudotumoral appearance on radiological imaging mimicking other space-occupying lesions, such as neoplasms, infections, and infarction. Especially when the patient's medical history is incompatible with MS, the differential diagnosis between these lesions constitutes a diagnostic challenge often requiring histological investigation. An older age at onset makes distinguishing tumefactive demyelinating lesion (TDL) from tumors even more challenging...
September 2016: Medicine (Baltimore)
Seema Bangalore Hanumanthe, Carla Francisco, Janace Hart, Jennifer Graves, Emmanuelle Waubant
Pediatric tumefactive lesions remains challenging to clinicians in terms of diagnosis and treatment. The authors describe 11 children with biopsy-proven central nervous system tumefactive demyelination. The mean age of onset was 11 years. Clinical and radiological data coupled with biopsy aided in the diagnosis of tumefactive demyelination. Of the 6 cases in which oligoclonal band data were available, only 3 showed oligoclonal band in the cerebrospinal fluid. Due to poor recovery despite treatment with high-dose glucocorticosteroids, intravenous immunoglobulin, and/or plasmapheresis, 6 cases went on to receive cyclophosphamide with marked improvement...
August 29, 2016: Journal of Child Neurology
Lucia Patriarca, Silvia Torlone, Fabiana Ferrari, Caterina Di Carmine, Rocco Totaro, Ernesto di Cesare, Alessandra Splendiani
Tumefactive multiple sclerosis is an inflammatory demyelinating disease of the central nervous system. It has recently been described as a rare subtype of multiple sclerosis (MS) characterised by the appearance of solitary or multiple space-occupying lesions associated with imaging characteristics mimicking neoplasm. Atypical features include plaque size >2 cm with mass effect, oedema, and/or ring enhancement on magnetic resonance (MR) images.This study is a retrospective review designed to evaluate the prevalence of tumefactive plaques in a selected population of 440 MS patients referred to our MS centre in Southern Italy between 2005 and 2014...
October 2016: Neuroradiology Journal
Todd A Hardy, Stephen W Reddel, Michael H Barnett, Jacqueline Palace, Claudia F Lucchinetti, Brian G Weinshenker
Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or MRI findings or poor response to treatments used for multiple sclerosis. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Baló's concentric sclerosis, Schilder's disease, and Marburg's multiple sclerosis. The overlapping features of these syndromes with multiple sclerosis and with each other complicate diagnosis and their categorisation as distinct or related conditions...
August 2016: Lancet Neurology
Jennifer Massey, Michael E Buckland, Yael Barnett, Ian Sutton
Neuromyelitis optica (NMO) spectrum disorder typically associates with a pathognomonic antibody response directed against aquaporin 4 and a unique immunopathological signature characterised by loss of aquaporin 4 expression. We describe a tumefactive presentation of NMO in which a biopsy specimen demonstrated active demyelination and reactive astrocytes with preserved surface aquaporin 4 immunoreactivity and dystrophic processes. While the astrocytic pathology is more typical of classical multiple sclerosis, a positive NMO-IgG (1:80) and subsequent clinical presentation with an area postrema syndrome is consistent with a diagnosis of NMO spectrum disorder...
July 28, 2016: BMJ Case Reports
Zachary Nicholas, Michael Sughrue, James Battiste, Ozer Algan
We aimed to evaluate the clinical and pathologic features of two common medical illnesses and their appropriate workup and pathognomonic findings. A 57-year-old white male presented with a new onset expressive aphasia while traveling abroad. He was evaluated at an outside facility and underwent workup for a stroke. The evaluation included a CT and MRI of the brain demonstrating three new enhancing lesions, the largest of which was a 2.5 cm ring-enhancing cystic lesion. A CT of the chest noted a 4-cm cystic thyroid lesion that was diagnosed as a thyroid cancer with brain metastases...
2016: Curēus
Sharfaraz Salam, Tatiana Mihalova, Rekha Siripurapu
No abstract text is available yet for this article.
June 3, 2016: BMJ Case Reports
Ujjawal Roy, Dinesh Satyanarayan Saini, Koushik Pan, Alak Pandit, Goutam Ganguly, Ajay Panwar
Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome. Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum. Rarely, there may be hemispheric tumefactive lesions that enhance in a "Cloud-like" fashion on gadolinium injection unlike in tumefactive multiple sclerosis where there is incomplete ring enhancement...
2016: Frontiers in Neurology
Mark D Kvarta, Deva Sharma, Rudolph J Castellani, Robert E Morales, Stephen G Reich, Amy S Kimball, Robert K Shin
BACKGROUND: Primary central nervous system lymphoma (PCNSL) may rarely be preceded by "sentinel demyelination," a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions-contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively-can be a significant diagnostic challenge...
May 21, 2016: BMC Neurology
Amrou Ali Idris, Tahmina Begum, Kenneth Rowland Verlage, Mashrafi Ahmed
No abstract text is available yet for this article.
April 26, 2016: BMJ Case Reports
Rocco Totaro, C Di Carmine, A Splendiani, S Torlone, L Patriarca, C Carrocci, S Sciamanna, C Marini, A Carolei
Although tumefactive multiple sclerosis is a well recognized variant of multiple sclerosis, prognostic uncertainty still exists about long term prognosis. The aim of this study was to estimate the occurrence and long term outcome of tumefactive demyelinating lesions (TDLs) in a cohort of multiple sclerosis patients. We reviewed brain MRI of 443 patients referred to our MS clinic. All patients meeting the McDonald criteria for multiple sclerosis and showing at least one TDL were included. Kaplan-Meier estimates of disease-free survival in patient cohort were compared with control group without TDLs using a log-rank test...
July 2016: Neurological Sciences
Todd A Hardy, W Oliver Tobin, Claudia F Lucchinetti
The availability of magnetic resonance imaging (MRI) has led to increasing recognition that multiple sclerosis (MS), tumefactive demyelination (TD) and Baló's concentric sclerosis (BCS) share many overlapping features. Baló-like lesions, which exhibit limited features of BCS, may represent an intermediate between BCS and typical MS demyelination. Lesions labeled as tumefactive are typically larger, but otherwise have much in common with conventional MS lesions, and TD and BCS lesions can also overlap. In this article, we explore the similarities between typical MS, TD and BCS cases, and reflect on the potential insights that intermediate or overlapping phenotypes may contribute towards an understanding of MS immunopathogenesis, and question whether these atypical forms of demyelination should be classified as separate demyelinating diseases, as different lesional manifestations of demyelination of any cause or as part of a spectrum with conventional MS...
July 2016: Multiple Sclerosis: Clinical and Laboratory Research
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