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Neuromyelitis optica, paraneoplastic, autoimmune

Eoin P Flanagan
Autoimmune myelopathies are a heterogeneous group of immune-mediated spinal cord disorders with a broad differential diagnosis. They encompass myelopathies with an immune attack on the spinal cord (e.g., aquaporin-4-IgG (AQP4-IgG) seropositive neuromyelitis optica (NMO) and its spectrum disorders (NMOSD)), myelopathies occurring with systemic autoimmune disorders (which may also be due to coexisting NMO/NMOSD), paraneoplastic autoimmune myelopathies, postinfectious autoimmune myelopathies (e.g., acute disseminated encephalomyelitis), and myelopathies thought to be immune-related (e...
2016: Handbook of Clinical Neurology
Michael H Silber
A number of autoantibodies, some paraneoplastic, are associated with sleep disorders. Morvan syndrome and limbic encephalitis, associated with voltage-gated potassium channel-complex antibodies, principally against CASPR2 and LGI1, can result in profound insomnia and rapid eye movement sleep behavior disorder (RBD). Patients with aquaporin-4 antibodies and neuromyelitis optica may develop narcolepsy in association with other evidence of hypothalamic dysfunction, sometimes as the initial presentation. Central sleep apnea and central neurogenic hypoventilation are found in patients with anti-N-methyl-d-aspartate receptor antibody encephalitis, and obstructive sleep apnea, stridor, and hypoventilation are prominent features of a novel tauopathy associated with IgLON5 antibodies...
2016: Handbook of Clinical Neurology
Agnieszka Prytuła, Johan Vande Walle, Helene Verhelst, Sunny Eloot, Stefaan Claus, Annick De Jaeger, Jo Dehoorne, Ann Raes
BACKGROUND: There is a growing evidence for autoimmunity in acute central nervous system (CNS) disorders and treatment with therapeutic plasma exchange (TPE) may be considered. The aim was to share our experience on the clinical application of TPE in these disorders and to present a reproducible protocol which can be used even in small children. METHODS: We present a series of 8 children aged 2-12 years with transverse myelitis, Bickerstaff's brainstem encephalitis, neuromyelitis optica, and acute paraneoplastic or unspecified encephalitis in whom TPE was used as a second-line or rescue treatment...
September 2015: International Journal of Artificial Organs
Anastasia Zekeridou, Vanda A Lennon
Neuromyelitis optica (NMO) and a related spectrum of inflammatory CNS disorders are unified by detection of a serum autoantibody specific for the aquaporin-4 (AQP4) water channel, which is abundant in astrocytic foot processes. The classic clinical manifestations of NMO are optic neuritis and longitudinally extensive transverse myelitis. Newly recognized manifestations of AQP4 autoimmunity include lesions of circumventricular organs and skeletal muscle. NMO is commonly relapsing, is frequently accompanied by other autoimmune disorders, and sometimes occurs in a paraneoplastic context...
August 2015: Neurology® Neuroimmunology & Neuroinflammation
Raffaele Iorio, Guido Rindi, Carmen Erra, Valentina Damato, Michela Ferilli, Mario Sabatelli
BACKGROUND: The observations of neuromyelitis optica spectrum disorders (NMOSD) occurring in the setting of cancer suggest that aquaporin-4 (AQP4) autoimmunity may in some cases be paraneoplastic. RESULTS: We describe a 72-year-old patient who developed a longitudinally extensive transverse myelitis associated with AQP4 autoantibodies in the setting of a lung adenocarcinoma recurrence. AQP4 expression was demonstrated in tumor cells. IgG in patient's cerebrospinal fluid bound to tumor cells co-localizing with AQP4 immunoreactivity...
May 2015: Multiple Sclerosis: Clinical and Laboratory Research
Tetsuya Akaishi, Ichiro Nakashima
Rituximab is a chimeric murine/human monoclonal antibody that specifically targets CD20 molecules on the surface of B-cells, thereby depleting B-cells and regulating humoral immunity. This antibody is mostly used in CD20-positive B-cell lymphoma, but is also widely used in many other connective tissue and neurological disorders. These neurological disorders include multiple sclerosis, neuromyelitis optica, myasthenia gravis, Lambert-Eaton myasthenic syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, paraneoplastic neurological syndromes, primary central nervous system lymphoma, inflammatory myopathy, and some other autoimmune-mediated neurological disorders...
October 2014: Brain and Nerve, Shinkei Kenkyū No Shinpo
Hee Kyung Yang, Se Joon Woo, Woong-Yang Park, Jeong-Min Hwang
BACKGROUND: Thymoma is associated with various paraneoplastic autoimmune disorders. Herein, we report paraneoplastic neuromyelitis optica (NMO) associated with both anti-aquaporin-4 (AQP4) immunoglobulin G (IgG) and type 1 antineuronal nuclear antibody (ANNA-1) in an invasive thymoma patient. CASE PRESENTATION: A woman presented with a sudden onset of bilateral progressive visual loss associated with recurrence of invasive thymoma, 6 years after thymectomy and immunosuppressive treatment...
2014: BMC Ophthalmology
Talal Al-Harbi, Adnan Al-Sarawi, Mohamed Binfalah, Said Dermime
Neuromyelitis optica (NMO), or Devic's syndrome, is an autoimmune central nervous system demyelinating disorder primarily affecting the spinal cord and the optic nerves. It is characterized by the presence of NMO antibodies, alongside clinical and radiological findings. NMO and NMO-spectrum disorders (NMO-SD) have been reported in autoimmune disorders, and are infrequently described as a paraneoplastic syndrome with cancers of lung, breast, and carcinoid tumors of the thyroid. We report a patient who presented with severe vomiting, blurring of vision, vertigo, diplopia, left hemiparesis and hemisensory loss and ataxia...
September 2014: Hematology/oncology and Stem Cell Therapy
Michelle Figueroa, Yong Guo, Alexandros Tselis, Sean J Pittock, Vanda A Lennon, Claudia F Lucchinetti, Robert P Lisak
IMPORTANCE: Reports of neuromyelitis optica spectrum disorder (NMOSD) occurring in the setting of neoplasia suggest that aquaporin-4 autoimmunity may in some cases have a paraneoplastic basis. OBSERVATIONS: In this case report, we describe a patient with NMOSD whose test results were seropositive for aquaporin-4 IgG and who had a hepatic metastasis from a small-bowel neuroendocrine tumor. The tumor cells expressed aquaporin-4 immunoreactivity. She presented to the Neurology Department at Wayne State University with bilateral leg weakness, ascending paresthesias, and decreased sensation...
April 2014: JAMA Neurology
Anand Iyer, Liene Elsone, Richard Appleton, Anu Jacob
Neuromyelitis optica (NMO) is an immune-mediated neurological disorder characterised by recurrent episodes of optic neuritis and longitudinally extensive transverse myelitis. A serum biomarker, aquaporin-4 IgG, the autoantibody against aquaporin-4 water channel, has been specifically associated with NMO and has assisted early recognition and prediction of relapses. Less commonly, a monophasic course, associated with antibodies to myelin oligodendrocyte glycoprotein has been reported. Specific diagnostic criteria have been defined; however, some cases that do not fulfil these criteria (but are nevertheless associated with aquaporin-4 IgG) are classified as NMO spectrum disorder and follow the same relapsing course...
May 2014: Autoimmunity
N Borisow, H Prüss, F Paul
Autoantibodies to neuronal tissue are becoming increasingly more important in the evaluation and classification of several neurological diseases, e.g. neuromyelitis optica, paraneoplastic syndromes of the central nervous system (CNS), stiff person syndrome or autoimmune epilepsy. As these disorders are rare, no evidence-based recommendations for therapy are available. Currently, immunomodulating or immunosuppressive drugs are administered in most cases. In paraneoplastic syndromes treatment of the underlying cancer is of considerable importance...
April 2013: Der Nervenarzt
Eoin P Flanagan, Vanda A Lennon, Sean J Pittock
The differential diagnosis of inflammatory myelopathies is broad. Autoimmune myelopathies represent a heterogeneous but significant portion of inflammatory myelopathies. The discovery of serologic biomarkers of autoimmune myelopathies (including aquaporin-4 and collapsin response-mediator protein-5 [CRMP-5] immunoglobulin [Ig]Gs) supports the concept of an autoimmune attack targeting the spinal cord. Neuroimaging, in particular MRI, may reveal distinctive patterns of signal abnormality suggesting an autoimmune etiology, such as longitudinally extensive transverse myelitis in neuromyelitis optica spectrum disorders or tract-specific changes in paraneoplastic disorders...
August 2011: Continuum: Lifelong Learning in Neurology
Carlo Antozzi
Therapeutic plasmapheresis (TPE) has an established role in disorders of the peripheral nervous system, but its use in disorders of the central nervous system (CNS) does not rely upon evidence-based data. Nevertheless, TPE is currently used in severe acute forms of demyelinating disease (multiple sclerosis/acute encephalomyelitis) unresponsive to corticosteroids. Recently, antibodies against the water channel aquaporin-4 have been detected in patients affected by neuromyelitis optica (Devic syndrome) and their pathogenetic role has been demonstrated, supporting the use of TPE in this disease...
January 2012: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
Yu Kitazawa, Yoko Warabi, Mitsuaki Bandoh, Toshiyuki Takahashi, Shiro Matsubara
We report a case of elderly-onset neuromyelitis optica (NMO) positive for the anti-aquaporin-4 (AQP-4) antibody; symptoms developed after the diagnosis of prostate adenocarcinoma and relapsed after a 23-valent pneumococcal polysaccharide vaccination. We suggest that activation of CD4-positive T cells and secretion of interferon-gamma induced by adenocarcinoma and complement activation induced by vaccination are responsible for the onset and relapse of NMO, even if a patient is positive for the anti-AQP-4 antibody...
2012: Internal Medicine
Canan Ulusoy, Erdem Tüzün, Murat Kürtüncü, Recai Türkoğlu, Gülşen Akman-Demir, Mefküre Eraksoy
Levels of several cytokines were evaluated in the sera of patients with neuromyelitis optica (NMO) and paraneoplastic or nonparaneoplastic autoimmune encephalitis (AE) and healthy controls (HC). AE patients had higher serum interleukin-17 (IL-17), interferon-gamma (IFN-γ), and IL-12 levels than NMO patients and HC. Also, AE patients with antibodies to cell surface antigens (voltage-gated potassium channel, N-methyl-d-aspartate receptor) displayed increased serum Th17 cytokine (IL-17, IL-23) levels as compared with AE patients with antibodies to intracellular antigens (Hu, Yo), NMO patients, and HC...
June 2012: International Journal of Neuroscience
M E Evangelopoulos, G Koutsis, E Andreadou, C Potagas, A Dimirakopoulos, C Sfagos
A 45-year-old female suffering from severe thoracic pain was admitted to the emergency department of our hospital. Thorough clinical examination revealed paresis of the left lower limb and sensory deficit at the level of the Th4 vertebra. MRI of the thoracic spine demonstrated a lesion at the level of Th1-Th7. Despite initial improvement following i.v. corticosteroid administration, the patient's clinical status deteriorated, with recurrence of myelitis and extension of the lesion to Th12. She developed paraparesis, hyperreflexia and spasticity of both legs, symmetrical sensory deficit below Th4, and sphincter dysfunction...
2011: Case Reports in Medicine
Keiko Tanaka, Masami Tanaka
Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that preferentially affects the optic nerves and spinal cord. NMO-IgG/anti-aquaporin 4 antibody (AQP4-Ab) is considered as a specific diagnostic marker for NMO. A previous study using animal models passively transferred with AQP4-Ab has partially proven that NMO-IgG/AQP4-Ab has an effector function in the pathogenesis of NMO, exemplifying the diagnostic significance of this antibody. Further, this marker can be used to differentiate the limited forms of NMO, such as recurrent myelitis or optic neuritis or NMO with isolated cerebral/brainstem lesions during the early course of the disease, from other diseases with a different etiology...
September 2010: Brain and Nerve, Shinkei Kenkyū No Shinpo
Roopa Bhat, Lawrence Steinman
The immune system has two major components, an innate arm and an adaptive arm. Certain autoimmune diseases of the brain represent examples of disorders where one of these constituents plays a major role. Some rare autoimmune diseases involve activation of the innate arm and include chronic infantile neurologic, cutaneous, articular (CINCA) syndrome. In contrast, adaptive immunity is prominent in multiple sclerosis, neuromyelitis optica, and the paraneoplastic syndromes where highly specific T cell responses and antibodies mediate these diseases...
October 15, 2009: Neuron
Maria José Sá
Acute transverse myelitis (ATM), a subgroup of various conditions that cause transverse spinal cord syndromes, is an inflammatory and usually idiopathic spinal cord disease. Idiopathic ATM is diagnosed according to established criteria which mainly assure a clinical picture of bilateral symptoms and signs attributable to spinal cord disease, existence of focal cord inflammation by magnetic resonance imaging and cerebrospinal fluid studies, and exclusion of many diseases, especially cord compression and "disease-associated ATM", as Sjögren disease...
December 2009: Autoimmunity Reviews
Bruce Cree
Although the co-occurrence of myelitis and optic neuritis that characterizes neuromyelitis optica (NMO) was recognized over a century ago, distinguishing NMO from multiple sclerosis relied solely on clinical criteria until recently. The identification of a biomarker that has high specificity for NMO is clinically useful for distinguishing NMO from multiple sclerosis and identifying patients at high risk for recurrent myelitis and optic neuritis. That fact that the biomarker is an autoantibody that recognizes aquaporin 4 (AQP4), a water channel expressed on astrocyte podocytes, has substantially contributed to the hypothesis that NMO is a humorally mediated autoimmune disease...
September 2008: Current Neurology and Neuroscience Reports
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