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MULTICENTER STUDY
L Quack, S Glatter, A Wegener-Panzer, R Cleaveland, A Bertolini, V Endmayr, R Seidl, M Breu, E Wendel, M Schimmel, M Baumann, M Rauchenzauner, M Pritsch, N Boy, T Muralter, G Kluger, C Makoswski, V Kraus, S Leiz, C Loehr-Nilles, J H Kreth, S Braig, S Schilling, J Kern, C Blank, B Tro Baumann, S Vieth, M Wallot, M Reindl, H Ringl, K P Wandinger, F Leypoldt, R Höftberger, K Rostásy
BACKGROUND: Acute cerebellitis (AC) in children and adolescents is an inflammatory disease of the cerebellum due to viral or bacterial infections but also autoimmune-mediated processes. OBJECTIVE: To investigate the frequency of autoantibodies in serum and CSF as well as the neuroradiological features in children with AC. MATERIAL AND METHODS: Children presenting with symptoms suggestive of AC defined as acute/subacute onset of cerebellar symptoms and MRI evidence of cerebellar inflammation or additional CSF pleocytosis, positive oligoclonal bands (OCBs), and/or presence of autoantibodies in case of negative cerebellar MRI...
November 2023: European Journal of Paediatric Neurology: EJPN
#22
JOURNAL ARTICLE
Hana Mojžišová, Martin Elišák, David Krýsl, Jitka Hanzalová, Adam Kalina, Marko Petržalka, Irena Doležalová, Matěj Červenka, Barbora Cvičková, Robert Leško, Jan Šroubek, Daniela Sochůrková, Jan Hemza, Eva Brichtová, Justina Dargvainiene, Zdeněk Vojtěch, Milan Brázdil, Klaus-Peter Wandinger, Frank Leypoldt, Petr Marusič
OBJECTIVE: Refractory epilepsy may have an underlying autoimmune etiology. Our aim was to assess the prevalence of neural autoantibodies in a multicenter national prospective cohort of patients with drug-resistant epilepsy undergoing epilepsy surgery utilizing comprehensive clinical, serologic, and histopathological analyses. METHODS: We prospectively recruited patients undergoing epilepsy surgery for refractory focal epilepsy not caused by a brain tumor from epilepsy surgery centers in the Czech Republic...
January 27, 2024: Epilepsia
#23
JOURNAL ARTICLE
Irin Pervin, Sudarshini Ramanathan, Cecilia Cappelen-Smith, Steve Vucic, Stephen W Reddel, Todd A Hardy
BACKGROUND: Relapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports. OBJECTIVE: We examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs). METHODS: We used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient. RESULTS: We identified 18 cases (11F, 7 M)...
January 5, 2024: Multiple Sclerosis and related Disorders
#24
JOURNAL ARTICLE
Margherita Bellucci, Chiara Castellano, Lucio Marinelli, Lucia Garbarino, Matteo Gastaldi, Diego Franciotta, Luana Benedetti
An 82-year-old man presented with 2-year lasting widespread muscular fasciculations, cramps, and limb stiffness, with spontaneous movements in the right lower limb, unsteady gait (Video 1), and falls. Neurophysiologic studies disclosed signs of neuromuscular hyperexcitability. CSF analysis showed high tau protein concentration (543 pg/mL; reference values, <404) and unique-to-CSF oligoclonal bands. Serum and CSF anti-IgLON5 antibodies were positive (Figure 1). He carried the anti-IgLON5 disease-associated HLA-DRB1*10:01 allele...
February 13, 2024: Neurology
#25
JOURNAL ARTICLE
Lorenzo Gaetani, Giovanni Bellomo, Elena Di Sabatino, Silvia Sperandei, Andrea Mancini, Kaj Blennow, Henrik Zetterberg, Lucilla Parnetti, Massimiliano Di Filippo
Early diagnosis of multiple sclerosis (MS) relies on clinical evaluation, magnetic resonance imaging (MRI), and cerebrospinal fluid (CSF) analysis. Reliable biomarkers are needed to differentiate MS from other neurological conditions and to define the underlying pathogenesis. This study aimed to comprehensively profile immune activation biomarkers in the CSF of individuals with MS and explore distinct signatures between MS with and without oligoclonal bands (OCB). A total of 118 subjects, including relapsing-remitting MS with OCB (MS OCB+) ( n = 58), without OCB (MS OCB-) ( n = 24), and controls with other neurological diseases (OND) ( n = 36), were included...
December 21, 2023: International Journal of Molecular Sciences
#26
JOURNAL ARTICLE
Kimberly A O'Neill, Andrew Dugue, Nicolas J Abreu, Laura J Balcer, Marc Branche, Steven Galetta, Jennifer Graves, Ilya Kister, Cynthia Magro, Claire Miller, Scott D Newsome, John Pappas, Janet Rucker, Connolly Steigerwald, Christopher M William, Scott S Zamvil, Scott N Grossman, Lauren B Krupp
A 16-year-old adolescent boy presented with recurrent episodes of weakness and numbness. Brain MRI demonstrated subcortical, juxtacortical, and periventricular white matter T2 hyperintensities with gadolinium enhancement. CSF was positive for oligoclonal bands that were not present in serum. Despite treatment with steroids, IV immunoglobulins, plasmapheresis, and rituximab, he continued to have episodes of weakness and numbness and new areas of T2 hyperintensity on imaging. Neuro-ophthalmologic examination revealed a subclinical optic neuropathy with predominant involvement of the papillomacular bundle...
March 2024: Neurology® Neuroimmunology & Neuroinflammation
#27
JOURNAL ARTICLE
Martina Miklušová, Tomáš Fürst, Dalibor Zimek, Jan Mareš
OBJECTIVE: Multiple sclerosis (MS) is a chronic, inflammatory, demyelinating disease of the central nervous system with varying progression rates among individuals. The ability to predict disease progression is crucial for treatment decisions with disease-modifying therapies (DMTs). A few cerebrospinal fluid (CSF) biomarkers have been investigated in relation to disease progression, but few have been effectively translated into clinical practice. The aim of this study was to evaluate the diagnostic and prognostic value of known CSF markers, to compare their sensitivity and specificity, and to develop a prognostic model using a combination of markers to predict disease progression...
December 22, 2023: Multiple Sclerosis and related Disorders
#28
Joe Senda, Ryota Hirao, Kentaro Maeda, Yoshinobu Amakusa, Tomoki Hirunagi, Masahisa Katsuno, Katsuhiro Kawaguchi
A 21-year-old Japanese woman presented with sudden eye movement disorders. An ophthalmic examination revealed bilateral hypotropia and esotropia complex. Brain magnetic resonance imaging revealed abnormal signals in the posterior and medial part of the lower pontine tegmentum (including periventricular and subcortical white matter) that were suggestive of demyelination. A cerebrospinal fluid test was positive for oligoclonal bands. She was subsequently diagnosed with multiple sclerosis and was administered intravenous methylprednisolone and oral dimethyl fumarate, with complete recovery from hypotropia and esotropia after two months...
November 2023: Nagoya Journal of Medical Science
#29
JOURNAL ARTICLE
Meltem Çobanoğulları Direk, Şeyda Besen, İbrahim Öncel, Ceren Günbey, Orhan Özdoğan, Leman Tekin Orgun, Sevim Sahin, Ali Cansu, Nihal Yıldız, Seda Kanmaz, Sanem Yılmaz, Hasan Tekgül, Dilşad Türkdoğan, Olcay Ünver, Gülten Öztürk Thomas, Salih Başıbüyük, Deniz Yılmaz, Ayşegül Neşe Kurt, Pembe Gültutan, Özlem Özsoy, Uluç Yiş, Semra Hız Kurul, Serdal Güngör, Bilge Özgör, Meral Karadağ, Nihal Olgaç Dündar, Pınar Gençpınar, Olgay Bildik, Sibğatullah Ali Orak, Çişil Çerçi Kabur, Bülent Kara, Ömer Karaca, Mehmet Canpolat, Hakan Gümüş, Hüseyin Per, Ünsal Yılmaz, Pakize Karaoğlu, Özlem Ersoy, Ayşe Tosun, Semra Büyükkorkmaz Öztürk, Deniz Yüksel, Ergin Atasoy, Kıvılcım Gücüyener, Miraç Yıldırım, Ömer Bektaş, Dilek Çavuşoğlu, Çoşkun Yarar, Olcay Güngör, Gülen Gül Mert, Esra Sarıgeçili, Selvinaz Edizer, İpek Dokurel Çetin, Seren Aydın, Betül Diler, Asena Ayça Özdemir, İlknur Erol, Çetin Okuyaz, Banu Anlar
BACKGROUND: Various etiologies may underlie optic neuritis, including autoantibody-mediated disorders described in the last decade. We re-examined demographic, clinical, laboratory features and prognostic factors in pediatric patients with autoimmune optic neuritis according to current knowledge. METHODS: Cases of pediatric ON from 27 centers in Türkiye diagnosed between 2009 and 2022 were included for retrospective evaluation. RESULTS: The study included 279 patients, 174 females and 105 males, with a female-to-male ratio of 1...
November 26, 2023: Multiple Sclerosis and related Disorders
#30
JOURNAL ARTICLE
Jerome J Graber
OBJECTIVE: Progress is ongoing in understanding paraneoplastic neurologic disorders, with new syndromes and antibodies being described and more detailed evidence available to guide workup for diagnosis and treatment to improve outcomes. Many excellent reviews have summarized the molecular features of different antibodies, but this article emphasizes the clinical features of each syndrome that may help guide initial diagnosis and treatment, which often should occur before an antibody or cancer is found to confirm the diagnosis...
December 1, 2023: Continuum: Lifelong Learning in Neurology
#31
Turan Poyraz, Armağan Varol, Hasan Armağan Uysal
Ocular flutter (OF) is a rare oculomotor syndrome. The most common etiologies are paraneoplastic, postinfectious and toxic-metabolic. However post-vaccinal etiology was rarely reported in OF. Here, we reported a post-vaccinal clinical syndrome characterized by OF-myoclonus and ataxia associated with oligoclonal bands (OCBs). A 60-year-old male who presented with dizziness, unsteady gait, involuntary movements, involuntary conjugate eye oscillations and extremity jerks that started 3 days after the second dose of mRNA BNT162b2 Covid-19 vaccine...
2023: Noro Psikiyatri Arsivi
#32
JOURNAL ARTICLE
Iliana Georganta, Despoina Chasapi, Charlotte Jayne Smith, Konstantinos Kopsidas, Andrew Tatham
BACKGROUND: This study aims to characterise the symptoms and clinical features of optic neuritis (ON) following SARS-CoV-2 infection and vaccination. METHOD: A literature search was conducted in four databases (PubMed, Medline, Embase and Google Scholar) to identify relevant case reports and case series. The records were screened and articles adhering to the inclusion criteria were critically appraised. RESULTS: Sixty-eight studies were found to be eligible for inclusion, including 34 reporting ON following SARS-CoV-2 infection and an equal number reporting cases postvaccination...
December 6, 2023: BMJ Open Ophthalmology
#33
JOURNAL ARTICLE
Vadym Biloshytsky, Anna Skorokhoda, Inna Buvailo, Maryna Biloshytska
BACKGROUND: Trigeminal neuralgia (TN) associated with a focal pontine lesion is a rare but challenging condition. The origin of the lesion, which does not fulfill the diagnostic criteria for multiple sclerosis, remains disputable. Pain in such conditions is often refractory to treatment, including microvascular decompression. OBSERVATIONS: A 36-year-old female presented with a 3.5-year history of shooting pain in the right V2 distribution triggered by talking and chewing...
December 4, 2023: J Neurosurg Case Lessons
#34
MULTICENTER STUDY
Xiang Zhang, Hongjun Hao, Tao Jin, Wei Qiu, Huan Yang, Qun Xue, Jian Yin, Ziyan Shi, Hai Yu, Xiaopei Ji, Xiaobo Sun, Qiuming Zeng, Xiaoni Liu, Jingguo Wang, Huining Li, Xiaoyan He, Jing Yang, Yarong Li, Shuangshuang Liu, Alexander Y Lau, Feng Gao, Shimin Hu, Shuguang Chu, Ding Ding, Hongyu Zhou, Haifeng Li, Xiangjun Chen
BACKGROUND: Cerebrospinal fluid oligoclonal band (CSF-OCB) is an established biomarker in diagnosing multiple sclerosis (MS), however, there are no nationwide data on CSF-OCB prevalence and its diagnostic performance in Chinese MS patients, especially in the virtue of common standard operation procedure (SOP). METHODS: With a consensus SOP and the same isoelectric focusing system, we conducted a nationwide multi-center study on OCB status in consecutively, and recruited 483 MS patients and 880 non-MS patients, including neuro-inflammatory diseases (NID, n = 595) and non-inflammatory neurological diseases (NIND, n=285)...
2023: Frontiers in Immunology
#35
JOURNAL ARTICLE
Cerebrospinal fluid findings in patients with neurological manifestations in post-COVID-19 syndrome.
Fabian Boesl, Yasemin Goereci, Ameli Gerhard, Benno Bremer, Vanessa Raeder, Finja Schweitzer, Uta Hoppmann, Janina Behrens, Judith Bellmann-Strobl, Friedemann Paul, Brigitte Wildemann, Sven Jarius, Harald Prüss, Heinrich J Audebert, Clemens Warnke, Christiana Franke
BACKGROUND: Information on cerebrospinal fluid (CSF) findings in patients with neurological manifestations in post-COVID-19 syndrome is scarce. METHODS: Retrospective evaluation of 84 CSF samples in patients fulfilling post-COVID-19 criteria in two neurological post-COVID-19 outpatient clinics. RESULTS: In 68% of samples, all CSF parameters were normal. The most frequent pathological CSF finding was elevation of total protein (median total protein 33...
January 2024: Journal of Neurology
#36
JOURNAL ARTICLE
Amy C Buck, Sapna Khemka, Kailey A Remien, Francesca Carra, Ian Rossman, Kelsey Merison
Myelitis is a rare inflammatory myelopathy, and known associated etiologies only account for a small number of causes. A significant percentage of cases have an unknown etiology and are considered idiopathic. With 64% to 68% of cases fitting into the idiopathic category, helminth infections, and specifically pinworm parainfections, should be considered in cases that would otherwise be classified as idiopathic. This case report outlines a pediatric patient diagnosed with myelitis given her progressive weakness, fussiness, refusal to bear weight as well as magnetic resonance imaging (MRI) demonstrating T2-hyperintense signal and/or T1 gadolinium enhancement, and/or positive cerebrospinal fluid (CSF) inflammatory markers...
October 13, 2023: Pediatric Neurology
#37
JOURNAL ARTICLE
Kamila Zondra Revendova, Krystof Svub, Radovan Bunganic, Ondrej Pelisek, Ondrej Volny, Aravind Ganesh, Michal Bar, David Zeman, Pavlina Kusnierova
BACKGROUND AND OBJECTIVES: To evaluate the diagnostic performance of the measles-rubella-zoster reaction (MRZR) in a large real-world multiple sclerosis (MS) cohort. Second, to compare MRZR with the determination of oligoclonal IgG bands (OCB), oligoclonal kappa free light chain bands (oKFLC), and the KFLC index. METHODS: A single-center retrospective study was conducted at the University Hospital Ostrava (Czech Republic). Patients were eligible if aged ≥18 years with a determined clinical diagnosis...
November 5, 2023: Multiple Sclerosis and related Disorders
#38
JOURNAL ARTICLE
Enric Monreal, José Ignacio Fernández-Velasco, Ana García-Soidán, Susana Sainz de la Maza, Mercedes Espiño, Noelia Villarrubia, Fernando Rodríguez-Jorge, Juan Luís Chico-García, Raquel Sainz-Amo, Jaime Masjuan, Lucienne Costa-Frossard, Luisa María Villar
INTRODUCTION: The immunoglobulin kappa free light chain (KFLC) index has been proposed as a potentially suitable alternative to oligoclonal IgG bands (OCGB) for diagnosing multiple sclerosis (MS), offering automation and reduced processing time. However, there is no consensus on the preferred approach or how to combine both techniques. METHODS: This prospective cohort study aimed to determine the best utilization of OCGB and KFLC index in patients with a clinically isolated syndrome (CIS) followed for at least two years...
2023: Frontiers in Immunology
#39
REVIEW
Peter G E Kennedy, Woro George, Xiaoli Yu
Intrathecal immunoglobulin G (IgG) and oligoclonal bands (OCBs) detected in both the brain and cerebrospinal fluid (CSF) are seminal features of multiple sclerosis (MS). The presence of OCBs correlates with elevated disease burden and severity and supports the diagnosis of MS. Despite numerous investigations into the potential viral and autoantigen targets, the precise antigenic specificity of OCBs has remained elusive. We have little knowledge of the nature regarding these oligoclonal IgG bands. Here, we present compelling evidence highlighting the key findings that both OCBs and intrathecal IgG antibodies are under genetic control and that OCBs originate from clonal B-cells in both the periphery and CNS...
January 2024: Journal of Neurology
#40
Manuel Salavisa, Bader Mohamed, Kimberley Allen-Philbey, Andrea M Stennett, Thomas Campion, Klaus Schmierer
OBJECTIVES: Parakinesia brachialis oscitans (PBO) is the involuntary movement of an otherwise paretic upper limb triggered by yawning. We describe the first case of PBO in a patient with a first manifestation of tumefactive multiple sclerosis (MS). METHODS: A 35-year-old man presented to the emergency department with a first episode of generalized seizure. Neurologic examination revealed left-sided spastic hemiparesis, predominantly affecting his upper limb. Brain MRI showed a tumefactive right hemisphere lesion consistent with demyelination...
December 2023: Neurology. Clinical Practice
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