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Opsoclonus myoclonus

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.
October 3, 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), anti-glutamate 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...
September 16, 2018: 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
Georgina Berridge, David A Menassa, Teresa Moloney, Patrick J Waters, Imogen Welding, Selina Thomsen, Sameer Zuberi, Roman Fischer, A Radu Aricescu, Michael Pike, Russell C Dale, Benedikt Kessler, Angela Vincent, Ming Lim, Sarosh R Irani, Bethan Lang
OBJECTIVE: To identify neuronal surface antibodies in opsoclonus myoclonus ataxia syndrome (OMAS) using contemporary antigen discovery methodology. METHODS: OMAS patient serum immunoglobulin G immunohistochemistry using age-equivalent rat cerebellar tissue was followed by immunoprecipitation, gel electrophoresis, and mass spectrometry. Data are available via ProteomeXchange (identifier PXD009578). This generated a list of potential neuronal surface cerebellar autoantigens...
August 21, 2018: Neurology
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. Although these syndromes are rare, the clinical manifestations can herald an unsuspected, underlying malignancy. The associated antibodies and histopathology of each syndrome are presented to help in the understanding of these autoimmune phenomena. Outlined in this review article are diagnostic features useful in differentiating cancer-associated retinopathy, melanoma-associated retinopathy, paraneoplastic polymorphous vitelliform maculopathy, bilateral diffuse uveal melanocytic proliferation and paraneoplastic neurologic syndromes such as optic neuropathy, opsoclonus-myoclonus, Lambert-Eaton myasthenia and paraneoplastic cerebellar degeneration...
June 2018: Journal Français D'ophtalmologie
Bastien Joubert, Kevin Rostásy, Jérôme Honnorat
Immune-mediated cerebellar ataxia (CA) comprises a group of rare diseases that are still incompletely described, and are probably underdiagnosed. Both acute and progressive progressions are possible. Different syndromes have been identified, including CA associated with anti-GAD antibodies, the cerebellar type of Hashimoto encephalopathy, primary autoimmune CA, gluten ataxia, opsoclonus-myoclonus syndrome, and paraneoplastic cerebellar degenerations. Most of these syndromes are associated with autoantibodies targeting neuronal antigens...
2018: Handbook of Clinical Neurology
A Bussat, S Langner-Lemercier, A Salmon, F Mouriaux
Paraneoplastic syndromes involving the visual system are a heterogeneous group of disorders occurring in the setting of systemic malignancy. They are unrelated to local tumor invasion or metastasis and unexplained by nutritional, metabolic, infectious or iatrogenic causes. The presence of antibodies may aid in the diagnosis of a paraneoplastic syndrome, although this is not an absolute requirement. Chorioretinal involvement is seen in CAR (cancer-associated retinopathy) syndrome, MAR (melanoma-associated retinopathy) syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation...
May 2018: Journal Français D'ophtalmologie
Thomas Simon, Emmanuel Cheuret, Léa Fiedler, Catherine Mengelle, Eloïse Baudou, Kumaran Deiva
Opso-myoclonus syndrome (OMS) is a very rare and severe condition. Ataxia, opsoclonus, myoclonus and/or behavioral and sleeping disturbances define that autoimmune disorder syndrome which is paraneoplastic or triggered by an infection. Here, we report a 3 year-old immunocompetent boy who developed an atypical OMS which was later complicated by an acute transverse myelitis. Screening for neuroblastoma was negative and extensive infectious screening revealed an active HHV-6 infection confirmed by blood and cerebrospinal fluid PCR...
September 2018: European Journal of Paediatric Neurology: EJPN
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