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https://www.readbyqxmd.com/read/28340257/cd8-positive-t-cell-leukoencephalitis-with-astrocytopathy-clinically-presenting-as-neuromyelitis-optica
#1
Diana L Thomas, Jody Manners, Daniel Marker, Joseph Mettenburg, Geoffrey Murdoch, Bryan Stevens, Guoji Wang, Clayton Wiley
We describe a novel disease entity with the clinical and radiologic presentation of neuromyelitis optica (NMO) and widespread CD8-positive T-cell leukoencephalitis and astrocytopathy. The 59-year-old female patient had a complex 2-year neurological history that included early changes in cognition and memory, progressive lower extremity motor dysfunction, and multimodal sensory involvement. MRI of the spinal cord showed increased T2 signal in the central cord extending from C2 through T4. MRI of the brain showed symmetric radial enhancement in periventricular deep white matter without evidence of demyelinating lesions...
March 16, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28331329/successful-treatment-for-psychomotor-agitation-in-neuromyelitis-optica-spectrum-disorder-with-trazodone-risperidone-combination-a-case-report
#2
Zui Narita, Harumasa Takano, Tomiki Sumiyoshi
Neuromyelitis optica (NMO) is a relapsing disease that typically affects the spinal cord and optic nerves. So far, a few studies have reported pharmacologic treatment for psychiatric symptoms in patients with NMO spectrum disorder (NMOSD). However, no literature has described psychomotor agitation associated with the disease and its treatment. We report an 84-year-old woman with NMOSD whose psychomotor agitation was effectively treated with a combination of trazodone and risperidone. Our observation suggests the ability of augmentation of antipsychotic drugs with antidepressants to ameliorate psychotic symptoms associated with NMOSD...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28326889/aquaporin-4-autoimmunity-in-patients-with-systemic-lupus-erythematosus-a-predominantly-population-based-study
#3
Nasrin Asgari, Sven Jarius, Helle Laustrup, Hanne Pb Skejoe, Soeren T Lillevang, Brian G Weinshenker, Anne Voss
BACKGROUND: Serum immunoglobulin G targeting the astrocyte water channel aquaporin-4 (AQP4) in the central nervous system (CNS) is a biomarker for neuromyelitis optica spectrum disease (NMOSD). Co-existence of NMOSD with systemic lupus erythematosus (SLE) putatively suggests susceptibility to antibody-mediated autoimmune disease. OBJECTIVE: To estimate the prevalence of NMOSD in SLE and investigate the immunogenetic background for an association of NMOSD and SLE...
March 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28306572/neuromyelitis-optica-and-neuromyelitis-optica-spectrum-disorders
#4
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...
March 16, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28302609/enhancing-brain-lesions-during-acute-optic-neuritis-and-or-longitudinally-extensive-transverse-myelitis-may-portend-a-higher-relapse-rate-in-neuromyelitis-optica-spectrum-disorders
#5
G Orman, K Y Wang, Y Pekcevik, C B Thompson, M Mealy, M Levy, I Izbudak
BACKGROUND AND PURPOSE: Neuromyelitis optica spectrum disorders are inflammatory demyelinating disorders with optic neuritis and/or longitudinally extensive transverse myelitis episodes. We now know that neuromyelitis optica spectrum disorders are associated with antibodies to aquaporin-4, which are highly concentrated on astrocytic end-feet at the blood-brain barrier. Immune-mediated disruption of the blood-brain barrier may manifest as contrast enhancement on brain MR imaging. We aimed to delineate the extent and frequency of contrast enhancement on brain MR imaging within 1 month of optic neuritis and/or longitudinally extensive transverse myelitis attacks and to correlate contrast enhancement with outcome measures...
March 16, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28298841/etiological-profile-of-noncompressive-myelopathies-in-a-tertiary-care-hospital-of-northeast-india
#6
Ashok Kumar Kayal, Munindra Goswami, Marami Das, Lakhshya Jyoti Basumatary, Suvorit Subhas Bhowmick, Baiakmenlang Synmon
BACKGROUND: The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21(st) century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic. OBJECTIVE: To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India. MATERIALS AND METHODS: An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016...
January 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28293214/different-phenotypes-at-onset-in-neuromyelitis-optica-spectrum-disorder-patients-with-aquaporin-4-autoimmunity
#7
Youming Long, Junyan Liang, Linzhan Wu, Shaopeng Lin, Cong Gao, Xiaohui Chen, Wei Qiu, Yu Yang, Xueping Zheng, Ning Yang, Min Gao, Yaotang Chen, Zhanhang Wang, Quanxi Su
BACKGROUND: Although rare, brain abnormalities without optic neuritis (ON) or transverse myelitis (TM) diagnosed with neuromyelitis optica spectrum disorder (NMOSD) have been reported in patients positive for the aquaporin-4 (AQP4) antibody. OBJECTIVE: To analyze demographic and clinical differences among NMOSD patients without ON or TM, those with either ON or TM, and patients with simultaneous ON and TM at disease onset. METHODS: In this retrospective study, patients who were positive for the AQP4 antibody, as detected using a cell-based assay, at the Second Affiliated Hospital of Guangzhou Medical University in China were recruited...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28289949/clinical-characteristics-of-optic-neuritis-in-hong-kong-population-10-year-review
#8
Bonnie Nga Kwan Choy, Alex Lap Ki Ng, Jimmy Shiu Ming Lai
PURPOSE: To review the clinical course of adult patients with acute optic neuritis over 10 years in Hong Kong, and the results were compared with other studies among Asian and Caucasian patients. METHODS: This study retrospectively analysed the clinical features of 38 adult patients (51 eyes) presented with optic neuritis in a Hong Kong hospital over 10 years (2001-2010). RESULTS: Optic neuritis had a female predominance (68%). The mean age of presentation was 40 years old...
March 13, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28289845/what-s-new-in-neuromyelitis-optica-a-short-review-for-the-clinical-neurologist
#9
Daniel Whittam, Martin Wilson, Shahd Hamid, Geoff Keir, Maneesh Bhojak, Anu Jacob
The evolution of neuromyelitis optica spectrum disorder (NMOSD) from a rare, incurable and misunderstood disease with almost universally poor outcomes to its present state in just over a decade is unprecedented in neurology and possibly in medicine. Our knowledge of NMOSD biology has led to the recognition of wider phenotypes, new disease mechanisms, and thus clinical trials of new and effective treatments. This article aims to update readers on the recent developments in NMOSD with particular emphasis on clinical advances, the 2015 diagnostic criteria, biomarkers, imaging, and therapeutic interventions...
March 13, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28284329/soluble-cd40-ligand-contributes-to-blood-brain-barrier-breakdown-and-central-nervous-system-inflammation-in-multiple-sclerosis-and-neuromyelitis-optica-spectrum-disorder
#10
Hiroki Masuda, Masahiro Mori, Tomohiko Uchida, Akiyuki Uzawa, Ryohei Ohtani, Satoshi Kuwabara
Soluble CD40 ligand (sCD40L) is reported to disrupt the blood-brain barrier (BBB). Cerebrospinal fluid (CSF) and serum sCD40L levels were measured in 29 multiple sclerosis (MS), 29 neuromyelitis optica spectrum disorder (NMOSD), and 27 disease control (DC) patients. In MS, serum sCD40L levels were higher than in DCs and positively correlated with the CSF/serum albumin ratio (Qalb). In NMOSD, CSF sCD40L levels were significantly increased compared to DCs, and were correlated to Qalb, CSF cell counts, protein concentrations, and interleukin-6 levels...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28283439/interleukin-6-analysis-of-572-consecutive-csf-samples-from-neurological-disorders-a-special-focus-on-neuromyelitis-optica
#11
Akiyuki Uzawa, Masahiro Mori, Hiroki Masuda, Ryohei Ohtani, Tomohiko Uchida, Setsu Sawai, Satoshi Kuwabara
BACKGROUND: Increases of cerebrospinal fluid (CSF) interleukin (IL)-6 has been reported in various neurological disorders but has never been systematically analyzed. We compared the CSF IL-6 concentrations among various neurological disorders and to evaluate the significance of CSF IL-6 measurements for the diagnosis of neuromyelitis optica (NMO). METHODS: We retrospectively investigated the IL-6 concentrations of 572 consecutive CSF samples in patients with various neurological disorders...
March 7, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28283104/comparing-epidemiology-and-baseline-characteristic-of-multiple-sclerosis-and-neuromyelitis-optica-a-case-control-study
#12
COMPARATIVE STUDY
Sharareh Eskandarieh, Saharnaz Nedjat, Ibrahim Abdollahpour, Abdorreza Naser Moghadasi, Amir Reza Azimi, Mohammad Ali Sahraian
BACKGROUND: Autoimmune syndromes such as Multiple Sclerosis (MS) and Neuromyelitis Optica (NMO) are chronic, demyelinating diseases of the central nervous system which usually affect young adults. Both environmental risk factors and genetic susceptibility have been proposed to explain the etiology of these diseases. The aim of this study was to compare epidemiology and possible risk factors in MS and NMO in Tehran, Iran. METHOD: A population-based incident case-control study was performed in Tehran based on a study conducted on1513 MS patients from Iranian MS Society registry center of Tehran, from 2014 to 2015, and 83 NMO patients diagnosed with NMO in 2015 at Sina hospital, a tertiary care referral center in Tehran...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28283103/there-is-less-mri-brain-lesions-and-no-characteristic-mri-brain-findings-in-iidds-patients-with-positive-aqp4-serology-among-malaysians
#13
Suhailah Abdullah, Farhana Fadzli, Norlisah Ramli, Chong Tin Tan
BACKGROUND AND OBJECTIVE: The recently introduced International Consensus diagnostic criteria for diagnosis of neuromyelitis spectrum disorder include patients who are seronegative for AQP4 antibody. The criteria are dependent on typical MRI changes in the spinal cord, optic nerve and brain. This study aims to determine whether there are significant differences in the MRI brain images between AQP4 positive and negative patients with IIDDs. METHOD: MRI brain of patients with a diagnosis of IIDDs presented to the Hospital from 2010 to 2015 was analysed...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28283100/antecedent-anti-nmda-receptor-encephalitis-in-two-patients-with-multiple-sclerosis
#14
A Baheerathan, W J Brownlee, D T Chard, K Shields, R Gregory, S A Trip
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterised by psychiatric symptoms, movement disorder and seizures often evolving into a severe encephalopathy. An overlap has recently been recognised between anti-NMDAR encephalitis and inflammatory demyelinating disorders, particularly neuromyelitis optical spectrum disorder (NMOSD). In this case report, we describe two patients with an initial presentation consistent with anti-NMDAR encephalitis who have subsequently developed relapsing-remitting multiple sclerosis (MS) and discuss the literature pertaining to potential overlap between NMDAR encephalitis and inflammatory demyelinating disorders...
February 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28282627/anti-nmdar-encephalitis-followed-by-seropositive-neuromyelitis-optica-spectrum-disorder-a-case-report-and-literature-review
#15
REVIEW
Ye Ran, Lu Wang, Fangfang Zhang, Ran Ao, Zhao Dong, Shengyuan Yu
Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory central nervous system syndrome, and encephalitis associated with anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is an autoimmune encephalopathy. A patient with both diseases, separately or simultaneously, is rare as a clinical phenomenon, but cannot be ignored. We report the clinical characteristics and imaging features of a special case with anti-NMDAR encephalitis followed by NMOSD. We subsequently reviewed the English language literature about demyelinating disorders with anti-NMDAR encephalitis...
February 27, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28281110/headache-in-neuromyelitis-optica
#16
REVIEW
Alina Masters-Israilov, Matthew S Robbins
PURPOSE OF REVIEW: Neuromyelitis optica (NMO) classically features a clinical presentation that includes longitudinally extensive transverse myelitis and optic neuritis. However, many other pathognomonic phenomena have more recently been described in patients diagnosed with NMO, including intractable hiccups, vomiting, and painful tonic spasms, but less has been reported regarding the relationship between NMO and headache. Though headache is well established as both a symptom and comorbidity of multiple sclerosis (MS), it has been much less described thus far in the NMO literature and warrants more careful evaluation...
April 2017: Current Pain and Headache Reports
https://www.readbyqxmd.com/read/28281033/a-treatable-cause-of-myelopathy-and-vision-loss-mimicking-neuromyelitis-optica-spectrum-disorder-late-onset-biotinidase-deficiency
#17
Sanem Yilmaz, Mine Serin, Ebru Canda, Cenk Eraslan, Hande Tekin, Sema Kalkan Ucar, Sarenur Gokben, Hasan Tekgul, Gul Serdaroglu
Biotinidase deficiency is characterized by severe neurological manifestations as hypotonia, lethargy, ataxia, hearing loss, seizures and developmental retardation in its classical form. Late-onset biotinidase deficiency presents distinctly from the classical form such as limb weakness and vision problems. A 14-year-old boy presented with progressive vision loss and upper limb weakness. The patient was initiated steroid therapy with a preliminary diagnosis of neuromyelitis optica spectrum disorder due to the craniospinal imaging findings demonstrating optic nerve, brainstem and longitudinally extensive spinal cord involvement...
March 9, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28274814/the-role-of-aquaporin-4-in-synaptic-plasticity-memory-and-disease
#18
REVIEW
Jacqueline A Hubbard, Jenny I Szu, Devin K Binder
Since the discovery of aquaporins, it has become clear that the various mammalian aquaporins play critical physiological roles in water and ion balance in multiple tissues. Aquaporin-4 (AQP4), the principal aquaporin expressed in the central nervous system (CNS, brain and spinal cord), has been shown to mediate CNS water homeostasis. In this review, we summarize new and exciting studies indicating that AQP4 also plays critical and unanticipated roles in synaptic plasticity and memory formation. Next, we consider the role of AQP4 in Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), multiple sclerosis (MS), neuromyelitis optica (NMO), epilepsy, traumatic brain injury (TBI), and stroke...
March 6, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28272192/optic-neuropathy-associated-with-primary-sj%C3%A3-gren-s-syndrome-a-case-series
#19
Eunoo Bak, Hee Kyung Yang, Jeong-Min Hwang
PURPOSE: To determine the diverse clinical features of optic neuropathy associated with primary Sjögren's syndrome in Korean patients. METHODS: Five women with acute and/or chronic optic neuropathy who were diagnosed as primary Sjögren's syndrome were retrospectively evaluated. Primary Sjögren's syndrome was diagnosed by signs and symptoms of keratoconjunctivitis sicca, positive serum anti-Ro/SSA and/or anti-La/SSB antibodies, and/or minor salivary gland biopsy...
March 7, 2017: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/28271642/large-scale-in-house-cell-based-assay-for-evaluating-the-serostatus-in-patients-with-neuromyelitis-optica-spectrum-disorder-based-on-new-diagnostic-criteria
#20
Yeseul Kim, Gayoung Kim, Byung Soo Kong, Ji Eun Lee, Yu Mi Oh, Jae Won Hyun, Su Hyun Kim, AeRan Joung, Byoung Joon Kim, Kyungho Choi, Ho Jin Kim
BACKGROUND AND PURPOSE: The detection of aquaporin 4-IgG (AQP4-IgG) is now a critical diagnostic criterion for neuromyelitis optica spectrum disorder (NMOSD). To evaluate the serostatus of NMOSD patients based on the 2015 new diagnostic criteria using a new in-house cell-based assay (CBA). METHODS: We generated a stable cell line using internal ribosome entry site-containing bicistronic vectors, which allow the simultaneous expression of two proteins (AQP4 and green fluorescent protein) separately from the same RNA transcript...
March 6, 2017: Journal of Clinical Neurology
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