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103 papers 0 to 25 followers
Kenneth E Remy, Jason W Custer, Joshua Cappell, Cortney B Foster, Nan A Garber, L Kyle Walker, Liliana Simon, Dayanand Bagdure
Purpose: Anti- N -methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series. Methods: We performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies...
2017: Frontiers in Pediatrics
Jonathan Douglas Santoro, Daniel V Lazzareschi, Cynthia Jane Campen, Keith P Van Haren
OBJECTIVE: To characterize the phenotype of pediatric Bickerstaff's brainstem encephalitis (BBE) and evaluate prognostic features in the clinical course, diagnostic studies, and treatment exposures. METHODS: We systematically reviewed PubMed, Web of Science, and SCOPUS databases as well as medical records at the Lucile Packard Children's Hospital to identify cases of pediatric BBE. Inclusion required all of the following criteria: age ≤ 20 years, presence of somnolence or alterations in mental status at the time of presentation or developed within 7 days of presentation, ataxia, and ophthalmoplegia...
January 2018: Journal of Neurology
Dean M Wingerchuk, Brian G Weinshenker
Multiple sclerosis (MS) is a common, disabling, putatively autoimmune neurological disease with worldwide distribution. It typically begins as a relapsing disorder that later evolves to a secondary progressive phase. Inflammatory and neurodegenerative mechanisms seem to operate in both phases, but their relative contributions and interactions are incompletely understood. Disease modifying therapies (DMTs) approved for relapsing multiple sclerosis interfere with a variety of immunological mechanisms to reduce rates of relapse, accumulation of disease burden measured by magnetic resonance imaging (MRI), and decline in neurological function over the two to three year duration of typical randomized controlled trials...
August 22, 2016: BMJ: British Medical Journal
B P Kelley, S C Patel, H L Marin, J J Corrigan, P D Mitsias, B Griffith
Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteristic findings within limbic structures suggestive of autoimmune encephalitis can be a key step in alerting clinicians to the potential diagnosis and ensuring a prompt and appropriate clinical work-up...
June 2017: AJNR. American Journal of Neuroradiology
Romain Marignier, Alvaro Cobo Calvo, Sandra Vukusic
PURPOSE OF REVIEW: The discovery of highly specific auto-antibodies directed against the water channel aquaporin 4 was a quantum leap in the definition, classification and management of neuromyelitis optica (NMO). Herein, we propose an update on epidemiological, clinical and therapeutic advances in the field, underlining unmet needs. RECENT FINDINGS: Large-scale epidemiological studies have recently provided a more precise evaluation of NMO prevalence and a better stratification regarding ethnicity and sex...
June 2017: Current Opinion in Neurology
John A Goodfellow, Hugh J Willison
In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts - novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS). 100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder, and that severe GBS could result in secondary axonal injury; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise...
December 2016: Nature Reviews. Neurology
Louise Wiblin, Joe Guadagno
Hermann Oppenheim described the 'Useless Hand' in 1911 as a classical but uncommon presentation of multiple sclerosis, in which a hand loses useful function due to proprioceptive loss, with relatively preserved motor function. Light touch perception may be subjectively altered or can be relatively intact. The lesion is (usually) a demyelinating plaque in the posterior columns of the cervical spinal cord. Depending on its location, it may affect one limb, or if more central, may produce a bilateral (if asymmetrical) picture...
December 2017: Practical Neurology
Elena Grebenciucova, Amy Pruitt
PURPOSE OF REVIEW: This paper will systemically review the risk of infections associated with current disease-modifying treatments and will discuss pre-treatment testing recommendations, infection monitoring strategies, and patient education. RECENT FINDINGS: Aside from glatiramer acetate and interferon-beta therapies, all other multiple sclerosis treatments to various degrees impair immune surveillance and may predispose patients to the development of both community-acquired and opportunistic infections...
September 22, 2017: Current Neurology and Neuroscience Reports
D Caputo, R Iorio, F Vigevano, L Fusco
BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. CASE STUDY: We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus...
January 2018: European Journal of Paediatric Neurology: EJPN
Nitish Chourasia, Michael W Watkins, Jeremy E Lankford, Joseph S Kass, Ankur Kamdar
BACKGROUND: Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that often affects women of childbearing age, and maternal-fetal transfer of anti-NMDAR antibodies during pregnancy has been documented in both symptomatic and asymptomatic women. The effects of these antibodies on the fetus, however, are incompletely understood. PATIENT DESCRIPTION: This term infant exhibited depressed respiratory effort, poor feeding, and abnormal movements after birth...
November 22, 2017: Pediatric Neurology
Alan J Thompson, Brenda L Banwell, Frederik Barkhof, William M Carroll, Timothy Coetzee, Giancarlo Comi, Jorge Correale, Franz Fazekas, Massimo Filippi, Mark S Freedman, Kazuo Fujihara, Steven L Galetta, Hans Peter Hartung, Ludwig Kappos, Fred D Lublin, Ruth Ann Marrie, Aaron E Miller, David H Miller, Xavier Montalban, Ellen M Mowry, Per Soelberg Sorensen, Mar Tintoré, Anthony L Traboulsee, Maria Trojano, Bernard M J Uitdehaag, Sandra Vukusic, Emmanuelle Waubant, Brian G Weinshenker, Stephen C Reingold, Jeffrey A Cohen
The 2010 McDonald criteria for the diagnosis of multiple sclerosis are widely used in research and clinical practice. Scientific advances in the past 7 years suggest that they might no longer provide the most up-to-date guidance for clinicians and researchers. The International Panel on Diagnosis of Multiple Sclerosis reviewed the 2010 McDonald criteria and recommended revisions. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical clinically isolated syndrome, define what is needed to fulfil dissemination in time and space of lesions in the CNS, and stress the need for no better explanation for the presentation...
December 21, 2017: Lancet Neurology
Sarah Hallowell, Esli Tebedge, Margaret Oates, Elizabeth Hand
Anti-N-methyl d-aspartate receptor (anti-NMDAR) encephalitis is a devastating disease that is increasingly being identified in both children and adults with psychosis, language disturbances, behavioral changes, and motor deficits. Currently no consensus guidelines exist for the optimal management of patients with this disease, although intravenous immune globulin (IVIG) therapy is often considered first-line pharmacotherapy. We present a case of an otherwise healthy 4 year-old-child who presented with seizures, loss of age-appropriate language skills, and behavioral changes, in whom anti-NMDAR was subsequently diagnosed...
March 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
Kristin N Varhaug, Christian Barro, Kjetil Bjørnevik, Kjell-Morten Myhr, Øivind Torkildsen, Stig Wergeland, Laurence A Bindoff, Jens Kuhle, Christian Vedeler
Objective: To investigate whether serum neurofilament light chain (NF-L) and chitinase 3-like 1 (CHI3L1) predict disease activity in relapsing-remitting MS (RRMS). Methods: A cohort of 85 patients with RRMS were followed for 2 years (6 months without disease-modifying treatment and 18 months with interferon-beta 1a [IFNB-1a]). Expanded Disability Status Scale was scored at baseline and every 6 months thereafter. MRI was performed at baseline and monthly for 9 months and then at months 12 and 24...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
Alberto Gajofatto
Siponimod (BAF312) is a synthetic molecule belonging to the sphingosine-1-phosphate (S1P) modulator family, which has putative neuroprotective properties and well-characterized immunomodulating effects mediated by sequestration of B and T cells in secondary lymphoid organs. Compared to fingolimod (ie, precursor of the S1P modulators commercially available for the treatment of relapsing-remitting [RR] multiple sclerosis [MS]), siponimod exhibits selective affinity for types 1 and 5 S1P receptor, leading to a lower risk of adverse events that are mainly induced by S1P3 receptor activation, such as bradycardia and vasoconstriction...
2017: Drug Design, Development and Therapy
Margherita Nosadini, Stefano Sartori, Suvasini Sharma, Russell C Dale
Beyond the major advances produced by careful clinical-radiological phenotyping and biomarker development in autoimmune central nervous system disorders, a comprehensive knowledge of the range of available immune therapies and a deeper understanding of their action should benefit therapeutic decision-making. This review discusses the agents used in neuroimmunology and their mechanisms of action. First-line treatments typically include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while for severe disease second-line "induction" agents such as rituximab or cyclophosphamide are used...
August 2017: Seminars in Pediatric Neurology
Rinze Neuteboom, Colin Wilbur, Danielle Van Pelt, Moses Rodriguez, Ann Yeh
Acquired demyelinating syndromes in childhood comprise a spectrum of monophasic and recurrent inflammatory conditions of the central nervous system. Examples of monophasic conditions include, clinically isolated syndromes such as optic neuritis and transverse myelitis, as well as acute disseminated encephalomyelitis, whereas recurrent disorders include entities such as multiple sclerosis and neuromyelitis optica spectrum disorder. Knowledge about these disorders has expanded due to rigorously evaluated diagnostic criteria, magnetic resonance imaging features, outcomes, and serum biomarkers in these disorders...
August 2017: Seminars in Pediatric Neurology
Bettina Balint, Kailash P Bhatia
PURPOSE OF REVIEW: This review highlights the recent developments in immune-mediated movement disorders and how they reflect on clinical practice and our understanding of the underlying pathophysiological mechanisms. RECENT FINDINGS: The antibody spectrum associated with stiff person syndrome and related disorders (SPSD) has broadened and, apart from the classic glutamic acid decarboxylase (GAD)- and amphiphysin-antibodies, includes now also antibodies against dipeptidyl-peptidase-like protein-6 (DPPX), gamma-aminobutyric acid type A receptor (GABAAR), glycine receptor (GlyR) and glycine transporter 2 (GlyT2)...
August 2016: Current Opinion in Neurology
Alexander Carvajal-González, M Isabel Leite, Patrick Waters, Mark Woodhall, Ester Coutinho, Bettina Balint, Bethan Lang, Philippa Pettingill, Aisling Carr, Una-Marie Sheerin, Rayomand Press, Raomand Press, Michael P Lunn, Ming Lim, Paul Maddison, H-M Meinck, Wim Vandenberghe, Angela Vincent
The clinical associations of glycine receptor antibodies have not yet been described fully. We identified prospectively 52 antibody-positive patients and collated their clinical features, investigations and immunotherapy responses. Serum glycine receptor antibody endpoint titres ranged from 1:20 to 1:60 000. In 11 paired samples, serum levels were higher than (n = 10) or equal to (n = 1) cerebrospinal fluid levels; there was intrathecal synthesis of glycine receptor antibodies in each of the six pairs available for detailed study...
August 2014: Brain: a Journal of Neurology
Sarah L Patterson, Sarah E Goglin
Neuromyelitis optica (NMO), formerly known as Devic disease, is an autoimmune astrocytopathic disease characterized by transverse myelitis and optic neuritis. Most patients demonstrate a relapsing course with incomplete recovery between attacks, resulting in progressive disability. The pathogenesis involves production of aquaporin-4 antibodies (AQP4-IgG) by plasmablasts in peripheral circulation, disruption of the blood-brain barrier, complement-mediated astrocyte injury, and secondary demyelination. The diagnosis relies on characteristic clinical manifestations in the presence of serum AQP4-IgG positivity or specific neuroimaging findings, and exclusion of alternative etiologies...
November 2017: Rheumatic Diseases Clinics of North America
Federica Provini
Agrypnia (from the Greek: to chase sleep) excitata (AE) is a syndrome characterized by loss of sleep and permanent motor and autonomic hyperactivation (excitata). Disruption of the sleep-wake rhythm consists in the disappearance of spindle-delta activities, and the persistence of stage 1 non-rapid eye movement (NREM) sleep. Rapid eye movement (REM) sleep persists but fails to stabilize, appearing in short recurrent episodes, isolated, or mixed with stage 1 NREM sleep. Diurnal and nocturnal motor, autonomic and hormonal overactivity is the second hallmark of AE...
April 2013: Current Neurology and Neuroscience Reports
2017-11-16 13:39:05
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