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Tumefactive multiple sclerosis

Hugh Kearney, Tucker Price, Jane Cryan, Alan Beausang, Seamus Looby, Francesca M Brett, Michael Farrell
BACKGROUND: Knowledge of the clinical outcome in tumefactive demyelination remains limited. AIMS: This study aims to characterise the natural history of biopsy-proven, pathogen-free, cerebral demyelination in an adult Irish population. METHODS: We identified all patients with biopsy-proven demyelination in a single neuropathology centre between 1999 and 2017. A baseline, and at least one follow-up MRI scan was available in each instance (mean of 3 scans per patient), together with both the presenting and most recent clinical details including disability level and disease-modifying drugs...
February 15, 2019: Irish Journal of Medical Science
Pekes Codjia, Xavier Ayrignac, Clarisse Carra-Dalliere, Mikael Cohen, Mahmoud Charif, Anais Lippi, Nicolas Collongues, Lucas Corti, Jerome De Seze, Christine Lebrun, Sandra Vukusic, Francoise Durand-Dubief, Pierre Labauge
BACKGROUND: The recent 2017 modification have increased the sensitivity of McDonald criteria for MS. Nevertheless, some MS patients with atypical MRI findings have been identified, leading to prolonged delay to diagnosis and high costs to look for alternative diagnoses. OBJECTIVE: To describe a series of MS patients with atypical MRI presentation. MATERIAL AND METHODS: Patients with atypical MS were identified through a nationwide retrospective study...
December 18, 2018: Multiple Sclerosis and related Disorders
Ibrahim Omerhodžić, Almir Džurlić, Dino Lisica, Nevena Mahmutbegović, Maida Nikšić, Nurija Bilalović, Enra Suljić
OBJECTIVE: We present a case of relapsing tumefactive demyelination in a young female patient, that posed a real diagnostic challenge, with a heterogeneous clinical picture, atypical for multiple sclerosis (MS) presentation, and neuroradiological manifestations with a high suspicion of neoplastic diseases. CASE REPORT: An 18-year old female patient presented to our Neurosurgical Out-patients' Clinic with symptoms atypical for multiple sclerosis, unremarkable neurological deficit, one tumefactive lesion on MRI, followed by relapse and another two lesions within a period of six months...
November 2018: Acta Medica Academica
Kunal Vakharia, Haris Kamal, Gursant S Atwal, James L Budny
Background: Multiple sclerosis (MS) is a chronic central nervous system inflammatory demyelinating disease characterized by multiple lesions disseminated in time and space. The lesions often have characteristic imaging findings on magnetic resonance (MR) imaging and cerebrospinal fluid findings that lead to their diagnosis. At times, these lesions may resemble tumors due to their large size (>2 cm), significant vasogenic edema, and ring-enhancing MR imaging findings. Such lesions are described as tumefactive demyelinating lesions or tumefactive MS, and they are generally seen in aggressive forms of MS associated with rapid progression...
2018: Surgical Neurology International
Mary Iype, Anish Ts, Pa Mohammed Kunju, Geetha Saradakutty, Mini Sreedharan, Shahanaz M Ahamed
OBJECTIVE: To study the long-term outcome of Acute disseminated encephalomyelitis (ADEM). METHODS: A retrospective cohort study was done among children diagnosed with ADEM (fulfilling IPMSSG criteria). Major outcome variables were motor deficit, scholastic underperformance and behavioral abnormality. RESULTS: The inclusion criteria were fulfilled by 102 children. Three died in hospital. The follow up ranged from 1-10 years (median 4 years)...
September 21, 2018: Pediatric Neurology
Franca Wagner, Lorenz Grunder, Arsany Hakim, Nicole Kamber, Michael P Horn, Julia Muellner, Robert Hoepner, Roland Wiest, Imke Metz, Andrew Chan, Anke Salmen
Objective: Differential diagnosis of neuromyelitis optica spectrum disorders (NMOSD) and multiple sclerosis (MS) or mimics can be challenging, especially in patients with atypical presentations and negative serostatus for aquaporin-4 antibodies (AQP4-Ab). This brief research report describes magnetic resonance imaging (MRI) findings focusing on quantitative apparent diffusion coefficient (ADC) histogram analysis as a potential tool to differentiate NMOSD from MS. Methods: Longitudinal MRI data obtained during routine clinical examinations were retrospectively analyzed in a patient with histologically determined cerebral NMOSD, a patient with an acute tumefactive MS lesion, and a patient with ischemic stroke...
2018: Frontiers in Neurology
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara
Tumefactive multiple sclerosis (MS) is characterized by the presence of a single MS-plaque in the brain. It mimics tumors due to large size, mass effect, and enhancement patterns. Refractory intracranial hypertension due to tumefactive MS requiring decompressive craniectomy (DC) was reported in five cases. However, none of these cases were documented new lesions during the follow-up. We report a case of a 28-year-old female admitted with acute right hemiparesis, headache, and nausea. A brain magnetic resonance imaging (MRI) revealed a left parietal lobe lesion...
July 2018: Asian Journal of Neurosurgery
Alaa Nabil Turkistani, Foziah Jabbar Alshamrani, Ghadah Faisal Shareefi, Abdulla Alsulaiman
Introduction: Tumefactive multiple sclerosis is a demyelinating disorder that appears tumor-like on MRI. To most physicians, diagnosing tumefactive MS by applying clinical, radiological, or laboratory examination like Cerebrospinal fluid (CSF) analysis, can be challenging and ultimately biopsy is necessary to confirm the diagnosis. Case presentation: This paper reports a case of a 37-year-old woman who presented with progressive headache and a strong family history of cancer and was misdiagnosed as having a CNS glioma...
August 2018: Electronic Physician
Jenelle Raynowska, Dhanashri P Miskin, Bidyut Pramanik, Saeed Asiry, Todd Anderson, John Boockvar, Souhel Najjar, Asaff Harel
OBJECTIVE: We report a series of 2 brothers who each developed tumefactive brain lesions, initially thought to have brain tumors or tumefactive multiple sclerosis (MS), but who were ultimately diagnosed with a rare autosomal dominant condition known as retinal vasculopathy with cerebral leukoencephalopathy (RVCL). METHODS: Case series and literature review. RESULTS: We present 2 brothers who developed tumefactive right frontal brain lesions leading to gait disturbances and cognitive changes...
October 9, 2018: Neurology
Shoji Yasuda, Hirohito Yano, Akio Kimura, Natsuko Suzui, Noriyuki Nakayama, Jun Shinoda, Toru Iwama
BACKGROUND: Tumefactive demyelinating lesion (TDL) is often reported as a rare variation of multiple sclerosis (MS). TDL is difficult to diagnose solely by magnetic resonance imaging (MRI) in patients with no history of MS. This is because the lesion often shows ring enhancement with perifocal brain edema on gadolinium MRI, thus mimicking glioblastoma multiforme (GBM). CASE DESCRIPTION: A 54-year-old healthy woman complained of headache 1 month before admission...
November 2018: World Neurosurgery
Pedro Sánchez, Virginia Meca-Lallana, José Vivancos
BACKGROUND AND OBJECTIVE: Fingolimod is a sphingosine 1-phosphate receptor modulator, which sequesters lymphocytes in lymph nodes and prevents them from entering the central nervous system. There have been increasing reports of severe rebounds with tumefactive demyelinatinglesions (TDLs) in patients with multiple sclerosis under fingolimod treatment, as well as following therapy discontinuation. Our objective is to review the clinico-radiological characteristics of patients with TDLs associated with fingolimod...
October 2018: Multiple Sclerosis and related Disorders
G Balloy, J Pelletier, L Suchet, C Lebrun, M Cohen, P Vermersch, H Zephir, E Duhin, O Gout, R Deschamps, E Le Page, G Edan, P Labauge, C Carra-Dallieres, L Rumbach, E Berger, P Lejeune, P Devos, J-B N'Kendjuo, M Coustans, E Auffray-Calvier, B Daumas-Duport, L Michel, F Lefrere, D A Laplaud, C Brosset, P Derkinderen, J de Seze, S Wiertlewski
BACKGROUND: Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. METHODS: Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively...
October 2018: Journal of Neurology
Ramy Mando, Emile Muallem, Shaiva G Meka, Ramona Berghea
Tumefactive Multiple Sclerosis (TMS) is a rare variant with 1 per 1000 cases of MS and 3 per million cases per year. TMS can mimic clinical and radiological features of a neoplasm, infarction, or abscess and therefore can be diagnostically challenging for clinicians. We present a clinical scenario of a patient presenting with left homonymous hemianopia with atypical radiological features initially thought to be more consistent with neoplasm or infraction. Ultimately, biopsy was done which led to the diagnosis of tumefactive multiple sclerosis...
2018: Case Reports in Neurological Medicine
Afsaneh Shirani, Gregory F Wu, Caterina Giannini, Anne H Cross
Tumefactive appearing lesions on brain imaging can cause a diagnostic dilemma. We report a middle-aged man who presented with right-sided optic neuritis. A brain MRI showed enhancement of the right optic nerve, and non-enhancing white matter lesions including a 3 cm right frontal lesion with adjacent gyral expansion. Cerebrospinal fluid analysis showed five oligoclonal bands not present in serum. Glatiramer acetate was started for suspected tumefactive multiple sclerosis (MS). A follow-up brain MRI 6 months later showed persistence of the frontal gyral expansion...
June 28, 2018: BMJ Case Reports
Dániel Sandi, Edit Bereg, Tamás Biernacki, Erika Vörös, Péter Klivényi, Csaba Bereczki, László Vécsei, Krisztina Bencsik
Multiple sclerosis (MS) is the autoimmune, neurodegenerative disease of the central nervous system (CNS). Typically, it affects the young adult population, however, up to 10% of the cases, it can develop in childhood. Atypical manifestations, such as the tumefactive variant (tMS) or acute disseminated encephalomyelitis (ADEM), especially coupled with fulminant disease course, are even more rare and pose a considerable differential diagnostic and therapeutic challenge. Recently, the therapeutic strategy on the use of disease modifying therapies (DMTs) in MS has shifted to the direction of a more individualized approach, that takes the personal differences heavily into account, in particular regard to the activity and prognosis of the disease...
July 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Kazunori Sato, Masaaki Niino, Atsushi Kawashima, Moemi Yamada, Yusei Miyazaki, Toshiyuki Fukazawa
Objective In Japan, following the launch of dimethyl fumarate (DMF) after fingolimod as a disease-modifying drug in multiple sclerosis (MS), some patients switched from fingolimod to DMF. The aim of this study was to determine the follow-up status of MS patients who switched to DMF after fingolimod cessation. Methods Clinical and magnetic resonance imaging (MRI) data in 19 patients with MS who switched to DMF were collected for at least for 6 months after fingolimod cessation. Results Ten patients (52.6%) experienced clinical or MRI exacerbation after fingolimod cessation...
September 15, 2018: Internal Medicine
Maria Teresa Giordana, Paola Cavalla, Antonio Uccelli, Alice Laroni, Fabio Bandini, Marco Vercellino, Gianluigi Mancardi
We present the neuropathological description of an autoptic case of fatal rebound of disease activity after fingolimod discontinuation in a multiple sclerosis patient. MRI prior to the fatal outcome showed several large tumefactive demyelinating lesions. These lesions were characterized by prominent astrocytic gliosis, with a remarkable preponderance of large hypertrophic reactive astrocytes showing intense expression of sphingosine-1-phosphate receptor 1. Prominent astrocytic gliosis was also diffusely observed in the normal-appearing white matter...
July 2018: Multiple Sclerosis: Clinical and Laboratory Research
Michelle Lin, Patrick Reid, Joshua Bakhsheshian
Tumefactive multiple sclerosis is a demyelinating lesion that can radiographically mimic high-grade gliomas during acute episodes, thus affecting clinical decision making. A delay in appropriate diagnoses can result in unnecessary invasive resections. The following case is a patient with unilateral weakness and radiologic findings that were concerning for a high-grade glioma. Peripheral studies were equivocal. The decision was made to proceed with a stereotactic biopsy, yielding a definitive diagnosis of tumefactive demyelinating lesion (TDL)...
April 2018: World Neurosurgery
Sameen Bin Naeem, Farheen Niazi, Atif Baig, Hina Sadiq, Mubbasher Sattar
Primary CNS (central nervous system) lymphoma is a rare condition with the incidence of less than 1% of all non-Hodgkin lymphomas (NHLs) and approximately 2% of all primary brain tumours. Diagnosis can be challenging and necessitates brain biopsy for definitive diagnosis. A 41-year male presented with history of impaired cognition, facial asymmetry, visual impairment and left sided body weakness. MRI brain demonstrated multiple enhancing lesions with one larger lesion in right basal ganglia with surrounding oedema and mass effect...
January 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Alejandro Enriquez-Marulanda, Jaime Valderrama-Chaparro, Laura Parrado, Juan Diego Vélez, Ana Maria Granados, Jorge Luis Orozco, Jairo Quiñones
Multiple Sclerosis (MS) is an autoimmune disease in which lymphocytes target putative myelin antigens in the CNS, causing inflammation and neurodegeneration. Fingolimod (FTY720) is an immunosuppressive drug used as a second line therapy for relapsing forms of MS due to its safety profile and good response to treatment. Despite its safety, there are still concerns about the possibility of Fingolimod being linked to the development of opportunistic infections like disseminated varicella zoster infections and herpes simplex encephalitis...
November 2017: Multiple Sclerosis and related Disorders
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