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autoimmune seizures

Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...
September 30, 2016: Neurology International
Deepa Dash, Madhavi Tripathi, Kavish Ihtisham, Manjari Tripathi
Limbic encephalitis is a group of immune-mediated disorders that includes the classic paraneoplastic encephalitic syndrome and the recently described non-paraneoplastic autoimmune encephalitis most of which target the extracellular antigens. We present a case of 70-year-old man who presented with rapidly progressive cognitive decline and refractory faciobrachial dystonic seizures and demonstrated seropositivity for leucine-rich, glioma-inactivated protein 1 antibodies. After immunomodulation, the patient had dramatic improvement in the cognitive functioning and in seizure control...
October 13, 2016: BMJ Case Reports
Kantha Medepalli, Cody M Lee, Lauryn A Benninger, Jean M Elwing
OBJECTIVE: Anti-N-methyl-d-aspartate receptor encephalitis is a rare but emerging cause of autoimmune encephalitis. Our objective is to present a case of this rare disease while highlighting the importance of an aggressive search for underlying malignancy as well as the common mischaracterization of primary psychiatric illness that occurs in these patients. METHODS: A young Caucasian female with no known psychiatric history presented with acute onset of seizures and psychosis...
2016: SAGE Open Med Case Rep
M Zhang, H J Hao, L P Liu, H H Zhang, Y Y Zhou
Autoimmune encephalitis with GABAB receptor antibodies has been rarely reported. Two cases of GABAB receptor antibodies encephalitis were presented here.Epilepsy was the onset symptom, followed by declined consciousness and frequent seizures. Fever was presented in the whole course of the disease. Myorhythmia of the two hands and pilomotor seizures were shown in the later course of the disease. No specificity was demonstrated in electroencephalograms and magnetic resonance imaging. Sensitive response was shown to the first-line immunotherapy...
October 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Jean-Marie Beis, Morgane Renard
OBJECTIVE: Anti-NMDA (N-Methyl-D-Aspartate) receptor encephalitis (NMDA-E) is the second cause of autoimmune encephalitis in adults. The objectives of this presentation are to describe the main clinical elements and rehabilitation specificities. OBSERVATIONS: Three female inpatients (mean age: 25) present with moderate to severe behavioural and/or psychiatric disorders, seizures and presence of associated tumor in one patient. Presence of anti-NMDA antibodies in the cerebrospinal liquid confirmed diagnosis...
September 2016: Annals of Physical and Rehabilitation Medicine
Shigemi Nagayama, Keiko Tanaka
Recently, the search for diagnostic antibody markers has drawn considerable attention in relation to autoimmune encephalitis. Among the antibody markers, the most frequently detected is the anti-N-methyl-D-aspartate receptor (NMDAR)antibody. Patients with this antibody develop characteristic clinical features. This disease tends to affect young women, and starts with psychiatric symptoms followed by seizures, involuntary movements, autonomic failure, and respiratory failure. Nearly half of these female patients have ovarian teratoma...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Vikram A Londhey
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder of unknown aetiology which can present with myriad clinical presentation. The neurological manifestations of SLE consist of central nervous system (CNS) and peripheral nervous system manifestations (PNS). The CNS manifestations are aseptic meningitis, cerebrovascular accidents (stroke), demyelinating disorders, headache, involuntary movements like chorea, myelopathy, acute confusional states, cognitive dysfunction, mood disorder, seizures, psychosis and cranial nerve palsies...
December 2015: Journal of the Association of Physicians of India
Atsurou Yamada, Nobuhiro Miyachi, Toshiyasu Miura, Masako Suzuki, Norio Watanabe, Tatsuo Akechi
INTRODUCTION: Post-infectious encephalitis/encephalopathy is a neurological syndrome that sometimes develops following common viral or bacterial infections. The most common form is acute disseminated encephalomyelitis (ADEM). ADEM is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalitis. Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is another type of severe autoimmune disorder, characterized by seizures, movement disorders and psychiatric symptoms...
2016: SpringerPlus
Vivek Joseph, Rahul Anil, Sary Aristy
A 70-year-old man presented with complaints of rapid cognitive decline and new onset leukopenia. The patient had a 17-year history of refractory seizures. Detailed review of symptoms and investigations revealed the patient met American College of Rheumatology (ACR) diagnostic criteria for systemic lupus erythematosus (SLE). The patient had high titer ANA with a strongly positive dsDNA. Immunosuppressive therapy with hydroxychloroquine and mycophenolate mofetil led to significant improvement in cognition and seizures...
October 2016: Journal of Clinical Medicine Research
Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 16, 2016: Neuro Endocrinology Letters
Jing W Hughes, Jennifer A Wyckoff, Abby S Hollander, Colin P Derdeyn, Janet B McGill
CONTEXT: Moyamoya syndrome is an idiopathic brain vasculopathy characterized by stenosis of major intracranial arteries. It often presents in patients with type 1 diabetes or thyroid disease and may have an autoimmune etiology. Moyamoya-related stroke poses a diagnostic challenge as initial symptoms and deficits vary greatly from classic ischemic stroke to encephalopathy, psychiatric, or seizure disorder. CASE DESCRIPTION: We report 4 patients with type 1 diabetes and other autoimmune diseases who developed moyamoya-related stroke at a young age...
November 2016: Journal of Diabetes and its Complications
Zeynep Özözen Ayas, Dilcan Kotan, Yeşim Güzey Aras
INTRODUCTION: Autoimmune neurological syndrome is a group of disorders caused by cancer affecting nervous system by different immunological mechanisms. In this study, we aim to study the clinical symptoms, cerebrospinal fluid (CSF) findings, autoantibody tests, computed tomography (CT), magnetic resonance imaging (MRI) signs and treatment outcome of patients with autoimmune syndromes. METHODS: In this study, 7 patients (4 male, 3 female) diagnosed with autoimmune neurological syndrome were retrospectively examined...
October 6, 2016: Neuroscience Letters
Margaret Rozier, Denise Morita, Marta King
BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune disorder characterized by behavioral changes, dyskinesia, and autonomic instability. PATIENT DESCRIPTION: We describe a 14-year-old girl who initially presented with acute behavioral changes and seizures and who over a 2-week period developed high fever, tachycardia, and elevated blood pressures. RESULTS: Because she received multiple medications including anticonvulsants and a neuroleptic, our patient was initially diagnosed with neuroleptic malignant syndrome, a disorder characterized by autonomic dysfunction, hyperthermia, muscle rigidity, and mental status changes usually caused by the use of a neuroleptic agent...
October 2016: Pediatric Neurology
Divyanshu Dubey, Zehra Farzal, Ryan Hays, L Steven Brown, Steven Vernino
BACKGROUND: The objective of this study was to analyze published literature on autoimmune epilepsy and assess predictors of seizure outcome. METHODS: From PubMed and EMBASE databases, two reviewers independently identified publications reporting clinical presentations, management and outcomes of patients with autoimmune epilepsy. A meta-analysis of 46 selected studies was performed. Demographic/clinical variables (sex, age, clinical presentation, epilepsy focus, magnetic resonance imaging [MRI] characteristics, time to diagnosis and initiation of immunomodulatory therapy, and type of immunomodulatory therapy) were compared between two outcome groups (responders and nonresponders)...
September 2016: Therapeutic Advances in Neurological Disorders
Roger Kelsey Halbert
Anti-N-methyl-D-aspartate receptor encephalitis is an autoimmune syndrome that presents with personality changes, autonomic dysfunction, and neurologic deterioration. Most patients with this syndrome progress from psychosis to seizure to catatonia, often associated with abnormal movements, autonomic instability, and hypoventilation. First-line treatment constitutes resection of the associated neoplasm, corticosteroids, intravenous immunoglobulin, and plasma exchange. Second-line treatment includes rituximab and cyclophosphamide...
October 2016: Journal of Neuroscience Nursing: Journal of the American Association of Neuroscience Nurses
Alessandra Dodich, Chiara Cerami, Sandro Iannaccone, Alessandra Marcone, Pierpaolo Alongi, Chiara Crespi, Nicola Canessa, Francesca Andreetta, Andrea Falini, Stefano F Cappa, Daniela Perani
BACKGROUND: Limbic encephalitis (LE) is characterized by an acute or subacute onset with memory impairments, confusional state, behavioral disorders, variably associated with seizures and dystonic movements. It is due to inflammatory processes that selectively affect the medial temporal lobe structures. Voltage-gate potassium channel (VGKC) autoantibodies are frequently observed. In this study, we assessed at the individual level FDG-PET brain metabolic dysfunctions and neuropsychological profiles in three autoimmune LE cases seropositive for neuronal VGKC-complex autoantibodies...
October 2016: Brain and Cognition
C R Newey, A Sarwal, S Hantus
Introduction. Autoimmune encephalitis (AE) is a clinically challenging diagnosis with nonspecific neurological symptoms. Prompt diagnosis is important and often relies on neuroimaging. We present a case series of AE highlighting the importance of an early [(18)F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET) scan. Methods. Retrospective review of seven consecutive cases of autoimmune encephalitis. Results. All patients had both magnetic resonance imaging (MRI) and FDG-PET scans. Initial clinical presentations included altered mental status and/or new onset seizures...
2016: Autoimmune Diseases
Albrecht Günther, Julia Schubert, Dirk Brämer, Otto Wilhelm Witte
Autoimmune encephalitis, an inflammatory disease of the brain, is usually attributed to antibody-mediated damage and dysfunction of neuronal structures. A distinction is made between onconeuronal antibodies (directed against intracellular neuronal antigens with resulting paraneoplastic neurological syndromes) and antibodies directed against neuronal cell surface proteins (with resulting synaptic encephalopathies). Anti-NMDA-Receptor-Encephalitis, the most common form of autoimmune encephalopathy, is characterized by a phased course of disease...
August 2016: Deutsche Medizinische Wochenschrift
Jakob Kreye, Nina K Wenke, Mariya Chayka, Jonas Leubner, Rajagopal Murugan, Nikolaus Maier, Betty Jurek, Lam-Thanh Ly, Doreen Brandl, Benjamin R Rost, Alexander Stumpf, Paulina Schulz, Helena Radbruch, Anja E Hauser, Florence Pache, Andreas Meisel, Lutz Harms, Friedemann Paul, Ulrich Dirnagl, Craig Garner, Dietmar Schmitz, Hedda Wardemann, Harald Prüss
SEE ZEKERIDOU AND LENNON DOI101093/AWW213 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently discovered autoimmune syndrome associated with psychosis, dyskinesias, and seizures. Little is known about the cerebrospinal fluid autoantibody repertoire. Antibodies against the NR1 subunit of the NMDAR are thought to be pathogenic; however, direct proof is lacking as previous experiments could not distinguish the contribution of further anti-neuronal antibodies...
October 2016: Brain: a Journal of Neurology
Hafez Mohammad Ammar Abdullah, Waqas Ullah, Ejaz Ahmad, Faiz Anwer
Posterior reversible encephalopathy syndrome (PRES) is a neurological condition that occurs secondary to a variety of causes like autoimmune diseases, uncontrolled hypertension and immunosuppressive agents. We report an unusual association of PRES and malignant hypertension secondary to focal segmental glomerulosclerosis in a young woman, presenting with sudden loss of vision and seizures. She had uncontrolled hypertension and a Glasgow Coma Scale of 6/15. Brain MRI revealed high signals in cortical and subcortical white matter and some involvement of the periventricular areas...
2016: BMJ Case Reports
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