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transverse myelitis trial

Michael Absoud, Benjamin M Greenberg, Ming Lim, Tim Lotze, Terrence Thomas, Kumaran Deiva
Pediatric acute transverse myelitis (ATM) is an immune-mediated CNS disorder and contributes to 20% of children experiencing a first acquired demyelinating syndrome (ADS). ATM must be differentiated from other presentations of myelopathy and may be the first presentation of relapsing ADS such as neuromyelitis optica (NMO) or multiple sclerosis (MS). The tenets of the diagnostic criteria for ATM established by the Transverse Myelitis Consortium Working Group can generally be applied in children; however, a clear sensory level may not be evident in some...
August 30, 2016: Neurology
Teresa M Crout, Laura P Parks, Vikas Majithia
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course...
August 2016: Current Rheumatology Reports
George Tackley, Fanny O'Brien, João Rocha, Mark Woodhall, Patrick Waters, Saleel Chandratre, Christopher Halfpenny, Cheryl Hemingway, Evangeline Wassmer, Warren Wasiewski, Maria Isabel Leite, Jacqueline Palace
OBJECTIVE: Neuromyelitis optica (NMO) is a rare antibody-mediated CNS disease characterised by disabling relapses leading to high morbidity and mortality. Understanding relapse activity and severity is important for treatment decisions and clinical trial design. We assessed (1) whether clinical and demographic factors associate with different relapse rates and (2) the relative impact of immunosuppressive treatments on relapse rates and on attack-related residual disability. METHODS: Clinical, demographic and treatment data were prospectively collected from 79 consecutive aquaporin 4 antibody positive patients seen in the nationally commissioned Oxford NMO service...
May 2016: Multiple Sclerosis and related Disorders
Maureen A Mealy, Kyong Shin, Gareth John, Michael Levy
OBJECTIVES: Neuromyelitis optica (NMO) is a relapsing autoimmune disease targeting the spinal cord and optic nerve leading to paralysis and blindness. Current treatment for acute NMO attacks is immunosuppression with high-dose corticosteroids and/or plasmapheresis. Preclinical animal studies suggest that bevacizumab might be beneficial in limiting the extent of inflammation during a NMO relapse by reducing the disruption of the blood-brain barrier. METHODS: We carried out an open-label phase 1b safety and proof-of-concept trial in 10 participants with NMO immunoglobulin G seropositive NMO, NMO spectrum disease and those at high risk for developing NMO/NMO spectrum disease who presented with an acute attack of transverse myelitis, optic neuritis or brainstem inflammation...
November 1, 2015: Clinical & Experimental Neuroimmunology
J Nicholas Brenton, Brenda L Banwell
Acquired pediatric demyelinating diseases manifest acutely with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, or with various other acute deficits in focal or polyfocal areas of the central nervous system. Patients may experience a monophasic illness (as in the case of acute disseminated encephalomyelitis) or one that may manifest as a chronic, relapsing disease [e.g., multiple sclerosis (MS)]. The diagnosis of pediatric MS and other demyelinating disorders of childhood has been facilitated by consensus statements regarding diagnostic definitions...
January 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Elena Sherman, May H Han
Neuromyelitis optica and neuromyelitis optica spectrum disorder (NMO/NMOSD) is a rare but clinically aggressive demyelinating disease of the central nervous system (CNS) caused by antibodies against water channel protein aquaporin 4 (AQP4) in the astrocytic foot processes. Patients typically present with optic neuritis (ON) or longitudinally extensive transverse myelitis (LETM). The majority of patients with NMOSD show good response to treatment with steroids and plasmapheresis in the acute setting; however, 90 % of patients will eventually have clinical relapses and accrue permanent disability...
November 2015: Current Treatment Options in Neurology
Zsolt Illés, Tobias Sejbaek, Tünde Csépány
The widening spectrum of MS treatment is partially due to increasing knowledge about the pathogenesis of MS. The humanized monoclonal antibody against CD52, alemtuzumab has been approved in Europe for the treatment of MS, which results in long-term depletion of B and T cells due to complement- and antibody-mediated cytotoxicity. Based on phase 2 and 3 clinical trials, alemtuzumob decreases the risk of sustained neurological deficit and progression compared to high-dose subcutaneous interferon-β1a in patients with active relapsing-remitting MS, either treatment-naïve or with breakthrough disease...
May 30, 2015: Ideggyógyászati Szemle
M Absoud, J Gadian, J Hellier, P A Brex, O Ciccarelli, G Giovannoni, J Kelly, P McCrone, C Murphy, J Palace, A Pickles, M Pike, N Robertson, A Jacob, M Lim
INTRODUCTION: Transverse myelitis (TM) is an immune-mediated disorder of the spinal cord which causes motor and sensory disturbance and limited recovery in 50% of patients. Standard treatment is steroids, and patients with more severe disease appear to respond to plasma exchange (PLEX). Intravenous immunoglobulin (IVIG) has also been used as an adjunct to steroids, but evidence is lacking. We propose the first randomised control trial in adults and children, to determine the benefit of additional treatment with IVIG...
2015: BMJ Open
Benjamin M Greenberg, Elliot M Frohman
PURPOSE OF REVIEW: This article presents data about inflammatory myelopathies, also referred to as transverse myelitis. While the idiopathic form of this condition is rare, the prevalence of transverse myelitis is relatively common when including all of the secondary causes, such as multiple sclerosis (MS). Thus, clinicians and researchers should be familiar with the presentations, diagnostic algorithms, treatment options, and long-term symptom management for patients with transverse myelitis...
February 2015: Continuum: Lifelong Learning in Neurology
Kumaran Deiva, Michael Absoud, Cheryl Hemingway, Yaiza Hernandez, Béatrice Hussson, Hélène Maurey, Giorgios Niotakis, Evangeline Wassmer, Ming Lim, Marc Tardieu
OBJECTIVE: To identify early prognostic factors of relapse and disability in children presenting with an acute idiopathic transverse myelitis (TM). METHODS: Ninety-five children with acute idiopathic TM from 2 national European cohorts (France and United Kingdom) of CNS demyelinating diseases in children were identified and studied for early factors that predict relapse and disability using logistic regression models. RESULTS: Sixteen (17%) relapsed, with a diagnosis of multiple sclerosis in 13 (14%) and neuromyelitis optica in 3 (3%)...
January 27, 2015: Neurology
Michael Levy, Maureen A Mealy
OBJECTIVE: To minimize complement-mediated damage in acute relapses of neuromyelitis optica (NMO) by adding treatment with a complement inhibitor, purified C1-esterase inhibitor, to the current standard of care (high-dose glucocorticoids). METHOD: We conducted an open-label phase 1b safety and proof-of-concept trial in 10 patients with NMO-immunoglobulin G seropositive NMO or NMO spectrum disease (NMOSD) who presented with acute transverse myelitis and/or optic neuritis...
June 2014: Neurology® Neuroimmunology & Neuroinflammation
Silvia R Delgado, Janice Maldonado, Kottil W Rammohan
An African-American male presented with bilateral visual impairment, gait difficulties, and bladder and bowel incontinence raising concerns for multiple sclerosis (MS) or neuromyelitis optica (NMO). He was identified to be HIV-1 infected with high viral load and low CD4+ counts. Magnetic resonance imaging (MRI) of the brain was abnormal, but atypical for MS. MRI of the cervical and thoracic spinal cord showed multiple areas of myelitis with a longitudinally extensive thoracic transverse myelitis that showed enhancement with gadolinium suggestive of NMO...
October 2014: Journal of Neurovirology
M J Magraner, F Coret, B Casanova
OBJECTIVE: Evaluate safety and tolerance levels for intravenous immunoglobulins (IVIG) as treatment for neuromyelitis optica (NMO). METHODS: Eight patients meeting Wingerchuk's revised diagnostic criteria were treated with IVIG every 2 months (0.7 g per kg body weight per day for 3 days). The primary outcome measure was the occurrence of serious adverse effects, defined according to NIH guidelines for clinical trials. Secondary outcome measures were changes in the yearly rate of attacks and in the degree of neurological disability measured with the Expanded Disability Status Scale (EDSS)...
March 2013: Neurología: Publicación Oficial de la Sociedad Española de Neurología
Maureen A Mealy, Dean M Wingerchuk, Benjamin M Greenberg, Michael Levy
BACKGROUND: Rare diseases require integrated multicenter clinical networks to facilitate clinical research. Neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSDs) are uncommon neuroinflammatory syndromes that are distinct from multiple sclerosis and associated with NMO-IgG, a serologic antibody against aquaporin 4. OBJECTIVE: To develop a national multicenter NMO clinical consortium and report initial demographic, clinical, and radiographic features of a cohort of patients with NMO/NMOSD in the United States...
September 2012: Archives of Neurology
Douglas Sato, Dagoberto Callegaro, Marco Aurélio Lana-Peixoto, Kazuo Fujihara
Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. Aquaporin-4 antibody is positive in a high percentage of NMO patients and it is directed against this water channel richly expressed on foot processes of astrocytes. Due to the severity of NMO attacks and the high risk for disability, treatment should be instituted as soon as the diagnosis is confirmed. There is increasing evidence that NMO patients respond differently from patients with multiple sclerosis (MS), and, therefore, treatments for MS may not be suitable for NMO...
January 2012: Arquivos de Neuro-psiquiatria
Maureen A Mealy, Scott Newsome, Benjamin M Greenberg, Dean Wingerchuk, Peter Calabresi, Michael Levy
BACKGROUND: Low 25-hydroxyvitamin D levels have been associated with a higher risk of developing multiple sclerosis and increased relapse rates in patients with multiple sclerosis. As a sterol hormone involved in multiple immunologic pathways, vitamin D may play a role in preventing monophasic immune-mediated central nervous system attacks from developing into recurrent disease. OBJECTIVE: To investigate the association between low serum vitamin D levels and recurrent spinal cord disease...
March 2012: Archives of Neurology
Mario Habek, Ivan Adamec, Vesna V Brinar
BACKGROUND CONTEXT: Longitudinally extensive transverse myelitis (LETM) is one of the defining features of neuromyelitis optica (NMO). Despite the well-established criteria, clinical and paraclinical features, the disease is often misdiagnosed and erroneously treated. PURPOSE: We report on a case of LETM in a patient with spatially limited NMO spectrum disorder that was misdiagnosed as spinal cord tumor and underwent spinal cord biopsy. STUDY DESIGN: A 43-year-old female patient is described...
December 2011: Spine Journal: Official Journal of the North American Spine Society
Emilio Benavente, Sergio Paira
Devic disease (neuromyelitis optica [NMO]) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by optic neuritis and transverse myelitis, either simultaneously or in isolation. NMO is often idiopathic but may also be associated with systemic autoimmune disease. The prognosis of NMO is severe, especially in those with early and recurrent relapses. MRI studies have revealed that most frequently, there is a long spinal cord lesion that extends through three or more vertebral segments in length...
December 2011: Current Rheumatology Reports
Yanqing Feng, Ning Guo, Junxiu Liu, Xi Chen, Qiaosong Sun, Rong Lai, Fan Huang
Incomplete transverse myelitis (ITM) of unknown origin is associated with high rates of morbidity and mortality. This prospective, open-label study was undertaken to determine whether antituberculous treatment (ATT) might help patients with ITM whose condition continues to deteriorate despite receiving IV methylprednisolone treatment. The study consisted of 67 patients with steroid-refractory ITM in whom Mycobacterium tuberculosis (MTB) was suspected clinically and in whom other known causes of myelopathy were excluded...
2011: Clinical & Developmental Immunology
Neeraj Kumar, B Mark Keegan, Fausto J Rodriguez, Julie E Hammack, Orhun H Kantarci
Intravascular lymphoma is a rare disorder that commonly involves the central nervous system. Neurologic involvement may be the presenting and only manifestation. Identifying intravascular lymphoma as the cause of neurologic disease is diagnostically challenging. We report an elderly woman presenting with subacute onset paraparesis due to spinal cord involvement by an intravascular lymphoma. Progressive worsening was associated with extension of a longitudinally-extensive thoracic intramedullary spinal cord lesion...
April 15, 2011: Journal of the Neurological Sciences
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