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Opsoclonus myoclonus

Xu Ding, Wei Han, Jing Wang, Wei Yang, Xiao-Feng Chang, Zhi-Yun Zhu, Hong Qin, Jin-Zhe Zhang, Xu Wang, Huan-Min Wang
BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder, usually accompanied by neuroblastoma (NB). There is no targeted treatment and animal model of OMS. We aimed to investigate whether insulin-like growth factor 1 (IGF-1)/phosphoinositide 3-kinase (PI3K) signaling alleviates neuronal cytolysis in pediatric OMS. METHODS: Cultured rat cerebral cortical neurons and cerebellar neurons were incubated with sera or IgG isolated from sera of children with OMS and NB...
December 11, 2018: Pediatric Research
Bastien Joubert, Jerome Honnorat
PURPOSE OF REVIEW: The current review develops the clinical presentations of nonparaneoplastic autoimmune cerebellar ataxia (ACA) and analyzes the association with autoantibodies. RECENT FINDINGS: Emerging evidence suggests that autoimmunity is involved in a significant proportion of sporadic ataxia cases. Moreover, numerous autoantibodies have recently been described in association with sporadic cerebellar ataxia, improving diagnosis and patient categorization...
January 28, 2019: Current Opinion in Neurology
M Gibaud, O Pauvert, S Gueden, J Durigneux, P Van Bogaert
Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e., opsoclonus-myoclonus syndrome (OMS). We report the case of a 9-year-old girl who suffered from headaches for several days and was shown to have opsoclonus and left peripheral facial palsy. Work-up excluded the diagnosis of neuroblastoma, but CSF analysis showed aseptic meningitis, and serology for Borrelia burgdorferi (Lyme) was positive. The outcome was favorable with complete regression of symptoms after treatment with ceftriaxone 2g/day for 3 weeks...
January 14, 2019: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Sun-Young Oh, Ji-Soo Kim, Marianne Dieterich
Opsoclonus-myoclonus syndrome in adults is a rare and heterogeneous disorder with the clinical features of opsoclonus, myoclonus, ataxia, and behavioral and sleep disturbances. The pathophysiology is thought to be immunological on the basis of paraneoplastic or infectious etiologies. Immunomodulatory therapies should be performed although the response may be incomplete. A number of autoantibodies have been identified against a variety of antigens, but no diagnostic immunological marker has yet been identified...
November 27, 2018: Journal of Neurology
Paul Maddison, Maarten J Titulaer, Jan J Verschuuren, Paul Gozzard, Bethan Lang, Sarosh R Irani, Lidia Sabater, Francesc Graus, Andrea Murray, Caroline J Chapman
Antibodies to SOXB1 proteins in patients with paraneoplastic disorders are associated with small-cell lung cancer (SCLC), particularly in Lambert-Eaton myasthenic syndrome (LEMS). We aimed to establish if SOX2 antibodies could be used to identify SCLC and other tumours found in a range of paraneoplastic disorders and controls. SOX2 antibodies were detectable in 61% of patients with LEMS-SCLC, and in other paraneoplastic disorders, such as opsoclonus-myoclonus and paraneoplastic cerebellar degeneration, only when there was an underlying SCLC...
November 8, 2018: Journal of Neuroimmunology
Răzvan Alexandru Radu, Elena Oana Terecoasă, Amalia Ene, Ovidiu Alexandru Băjenaru, Cristina Tiu
Opsoclonus-myoclonus syndrome (OMS) is a very rare condition with different autoimmune, infectious and paraneoplastic aetiologies or in most cases idiopathic. We report the case of a 75-year-old woman who was admitted in our department in early fall for altered mental status, opsoclonus, multifocal myoclonus, truncal titubation and generalized tremor, preceded by a 5 day prodrome consisting of malaise, nausea, fever and vomiting. Brain computed tomography and MRI scans showed no significant abnormalities and cerebrospinal fluid changes consisted of mildly increased protein content and number of white cells...
2018: Frontiers in Neurology
Nicolás X Urriola, Jacob Helou, Joel Maamary, Jacob Pogson, Frederick Lee, Kaitlyn Parratt, David Gillis, Michael J Fulham, G Michael Halmágyi
Opsoclonus-myoclonus syndrome (OMS) is a brainstem/cerebellar syndrome producing disabling multi-directional saccadic oscillations with oscillopsia, with or without somatic myoclonus and cerebellar ataxia (Wong et al., 2001; Armangué et al., 2016). OMS is presumed to have an autoimmune basis and patients with it are tested for antineuronal antibodies and have imaging to locate any tumors. Here we report a unusual case of a young woman who had NMDAR antibody (NMDAR-ab) positive, teratoma-related, isolated OMS without encephalopathy...
October 16, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Shabbir Hussain Merchant, Felipe Vial, Giorgio Leodori, Stanley Fahn, Seth L Pullman, Mark Hallett
BACKGROUND: Many different oligosynaptic reflexes are known to originate in the lower brainstem which share phenomenological and neurophysiological similarities. OBJECTIVE: To evaluate and discuss the differences and aberrancies among these reflexes, which are hard to discern clinically using neurophysiological investigations with the help of a case report. METHODS: We describe the clinical and neurophysiological assessment of a young man who had a childhood history of opsoclonus-myoclonus syndrome with residual mild ataxia and myoclonic jerks in the distal extremities presenting with subacute onset total body jerks sensitive to sound and touch (in a limited dermatomal distribution), refractory to medications...
October 6, 2018: Parkinsonism & related Disorders
I K Sharawat, A G Saini, A Kasinathan, S S Mandava, N Sankhyan
No abstract text is available yet for this article.
December 2018: Lupus
Flora Dresco, François Aubin, Elise Deveza, Eugeniu Revenco, Laurent Tavernier, Eve Puzenat
No abstract text is available yet for this article.
October 3, 2018: Acta Dermato-venereologica
Akshata Huddar, Parayil S Bindu, Madhu Nagappa, Rose D Bharath, Sanjib Sinha, Pavagada S Mathuranath, Arun B Taly
Background: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder; there is limited experience regarding its clinical course and therapeutic response. Aims and Objectives: To describe the clinical profile, investigations, and therapeutic outcome in pediatric OMAS. Patients and Methods: Fourteen children (age: 27.1 ± 7 months; male: female = 1:2.3) suffering from OMAS seen over a period of 10 years (2006-2015) were included in the study...
September 2018: Neurology India
Ravindra Kumar Garg, Imran Rizvi, Hardeep Singh Malhotra, Neeraj Kumar
No abstract text is available yet for this article.
September 2018: Neurology India
Satish Khadilkar, Rajesh Benny
No abstract text is available yet for this article.
September 2018: Neurology India
Hiroshi Mitoma, Mario Manto, Christiane S Hampe
Immune-mediated cerebellar ataxias (IMCAs), a clinical entity reported for the first time in the 1980s, include gluten ataxia (GA), paraneoplastic cerebellar degenerations (PCDs), antiglutamate decarboxylase 65 (GAD) antibody-associated cerebellar ataxia, post-infectious cerebellitis, and opsoclonus myoclonus syndrome (OMS). These IMCAs share common features with regard to therapeutic approaches. When certain factors trigger immune processes, elimination of the antigen( s) becomes a priority: e.g., gluten-free diet in GA and surgical excision of the primary tumor in PCDs...
2019: Current Neuropharmacology
Michael R Pranzatelli, Elizabeth D Tate, Nathan R McGee, Craig A MacArthur
BACKGROUND: Rituximab (anti-CD20) has been used as B-cell-targeted intervention to treat opsoclonus-myoclonus syndrome. Due to isolated reports of chronic hypogammaglobulinemia and B lymphopenia following rituximab in several disorders, and rapid B-cell depletion after a few doses, we reduced the dosage 20% in our clinical practice. METHODS: In this Institutional Review Board-approved retrospective study, 32 children with opsoclonus-myoclonus syndrome and cerebrospinal fluid B-cell expansion had received front-loaded adrenocorticotropic hormone, intravenous immunoglobulin, and rituximab combination immunotherapy for de novo opsoclonus-myoclonus syndrome...
August 2018: Pediatric Neurology
Brian H Kushner, Yasmin Khakoo
No abstract text is available yet for this article.
January 2018: Lancet Child & Adolescent Health
N S Burlakova, Yu V Mikadze, Yu N Fedorova, E S Ilyina, R Ts Bembeeva, N N Zavadenko
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder with unknown etiology and pathogenesis. There is not enough knowledge about psychological development of children with OMS. AIM: To study an influence of the time of disease onset and children's age and type of disease course on psychological development of children with OMS. MATERIAL AND METHODS: The study included 26 children with OMS of age between 1 year 7 months and 13 years, 10 boys and 16 girls...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Omer Fatih Olmez, Oguzcan Kinikoglu, Nesrin Helvacı Yilmaz, Ahmet Bilici, Erdem Cubukcu, Mehmet Seker, Tansel Cakir, Ozcan Yildiz, Jamshid Hamdard
Paraneoplastic neurological syndrome is associated with anti-Ri antibodies, which are typically present with opsoclonus-myoclonus-ataxia. Human epidermal growth factor receptor 2 (HER2) overexpression is present in 15%-25% of breast cancer and is associated with poor prognosis. There are a few reports of paraneoplastic neurological syndrome associated with HER2-positive breast cancer in the literature, of which most are anti-Yo-associated paraneoplastic neurological syndrome. We present herein the case of a female patient with HER2-positive breast cancer who had atypical anti-Ri antibody associated with opsoclonus-myoclonus paraneoplastic neurological syndrome...
August 19, 2018: Journal of Oncology Pharmacy Practice
Mateus Santana do Rosário, Marta Giovanetti, Pedro Antonio Pereira de Jesus, Daniel Santana Farias, Nuno R Faria, Clayton Pereira S de Lima, Sandro Patroca da Silva, Marcio Roberto Nunes, Luiz Carlos Junior Alcantara, Isadora Cristina de Siqueira
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare neurological disorder characterized by irregular multidirectional eye movements, myoclonus, cerebellar ataxia, sleep disturbances, and cognitive dysfunction. Although most commonly related to paraneoplastic syndrome, this condition has occasionally been described following infectious illnesses. This article reports the first case of OMAS in association with chikungunya and dengue virus co-infection. The genetic analysis identified chikungunya virus of East/Central/South African genotype and dengue serotype 4 virus of genotype II...
October 2018: International Journal of Infectious Diseases: IJID
Çağla Aydin, Şenay Yıldız Çelik, Sema İçöz, Canan Ulusoy, Tuncay Gündüz, Gülşen Akman Demir, Murat Kürtüncü, Erdem Tüzün
Introduction: Anti-neuronal antibodies (ANA) are found in paraneoplastic neurological syndrome and autoimmune encephalitis patients. Our aim was to analyze prognostic factors related with ANA seropositivity. Methods: Twenty-seven consecutive ANA seropositive patients were included in the study. ANA were detected by immunofluorescent staining, immunoblot and cell-based assay methods. All patients were followed with a standard treatment protocol. Clinical syndromes, tumor types, modified Rankin scores, cranial MRI and oligoclonal band (OCB) findings were recorded...
2018: Noro Psikiyatri Arsivi
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