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immunotherapy and autoimmune epilepsy

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https://www.readbyqxmd.com/read/27686441/-an-analysis-and-literature-review-of-two-cases-of-autoimmune-encephalitis-with-gabab-receptor-antibodies
#1
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]
https://www.readbyqxmd.com/read/27643908/autoantibody-associated-central-nervous-system-neurologic-disorders
#2
Jenny Linnoila, Sean J Pittock
Autoimmune neurology is a rapidly evolving new subspecialty driven by the discovery of novel neural- (neuronal- or glial-) specific autoantibodies and their target antigens. The neurologic manifestations affecting the central nervous system include encephalitis, dementia, epilepsy, and movement and sleep disorders. Laboratory testing is now available for most of these neural-specific autoantibodies, which serve as diagnostic markers, in some instances directing the physician toward specific cancer types (e...
August 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27450643/antibody-associated-epilepsies-clinical-features-evidence-for-immunotherapies-and-future-research-questions
#3
REVIEW
Ochuko D Bakpa, Markus Reuber, Sarosh R Irani
PURPOSE: The growing recognition of epilepsies and encephalopathies associated with autoantibodies against surface neuronal proteins (LGI1, NMDAR, CASPR2, GABABR, and AMPAR) means that epileptologists are increasingly asking questions about mechanisms of antibody-mediated epileptogenesis, and about the use of immunotherapies. This review summarizes clinical and paraclinical observations related to autoimmune epilepsies, examines the current evidence for the effectiveness of immunotherapy, and makes epilepsy-specific recommendations for future research...
October 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27299785/-pathophysiology-and-prognostic-factors-of-autoimmune-encephalitis
#4
H Prüß
More and more forms of autoimmune encephalitis are being identified with the clinical spectrum ranging from epilepsy over movement disorders to psychosis. The increasing appreciation of clinical symptoms raises questions about the underlying pathophysiological mechanisms and prognostic factors. Numerous novel findings on the aetiology demonstrate that diverse tumours, but also infections of the central nervous system such as Herpes encephalitis can trigger autoimmune encephalitis. Antibodies against neuronal surface epitopes are directly pathogenic in the majority of cases...
May 2016: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/27112680/autoimmune-epilepsy
#5
Jeffrey Britton
Seizures are a common manifestation of autoimmune limbic encephalitis and multifocal paraneoplastic disorders. Accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of encephalitis. The autoimmune epilepsies are immunologically mediated disorders in which recurrent seizures are a primary and persistent clinical feature. When other etiologies have been excluded, an autoimmune etiology is suggested in a patient with epilepsy upon detection of neural autoantibodies and/or the presence of inflammatory changes on cerebrospinal fluid (CSF) or magnetic resonance imaging...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27112678/voltage-gated-potassium-channel-complex-autoimmunity-and-associated-clinical-syndromes
#6
Sarosh R Irani, Angela Vincent
Voltage-gated potassium channel (VGKC)-complex antibodies are defined by the radioimmunoprecipitation of Kv1 potassium channel subunits from brain tissue extracts and were initially discovered in patients with peripheral nerve hyperexcitability (PNH). Subsequently, they were found in patients with PNH plus psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) with prominent amnesia and frequent seizures. Most recently, they have been described in patients with pure epilepsies, especially in patients with the novel and distinctive semiology termed faciobrachial dystonic seizures (FBDS)...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/26996997/neuronal-antibodies-in-pediatric-epilepsy-clinical-features-and-long-term-outcomes-of-a-historical-cohort-not-treated-with-immunotherapy
#7
Sukhvir Wright, Ada T Geerts, Cornelia Maria Jol-van der Zijde, Leslie Jacobson, Bethan Lang, Patrick Waters, Maarten J D van Tol, Hans Stroink, Rinze F Neuteboom, Oebele F Brouwer, Angela Vincent
OBJECTIVE: In autoimmune encephalitis the etiologic role of neuronal cell-surface antibodies is clear; patients diagnosed and treated early have better outcomes. Neuronal antibodies have also been described in patients with pediatric epilepsy without encephalitis. The aim was to assess whether antibody presence had any effect on long-term outcomes in these patients. METHODS: Patients (n = 178) were recruited between 1988 and 1992 as part of the prospective Dutch Study of Epilepsy in Childhood; none received immunotherapy...
May 2016: Epilepsia
https://www.readbyqxmd.com/read/26940288/suspected-antibody-negative-autoimmune-limbic-encephalitis-outcome-of-immunotherapy
#8
B von Rhein, J Wagner, G Widman, M P Malter, C E Elger, C Helmstaedter
OBJECTIVES: Whether and when to immunologically treat epilepsy patients with suggested autoantibody (AB)-negative limbic encephalitis (LE) is clinically challenging. Therefore, we evaluated the clinical outcome and eventual outcome predictors of immunotherapy in a group of AB-negative patients with recent-onset temporal lobe epilepsy (TLE), magnetic resonance imaging (MRI) indicators of LE, subjective cognitive decline, and/or psychiatric symptoms. METHODS: This retrospective, observational, uncontrolled study monitored 28 TLE patients with suggested AB-negative LE along with methylprednisolone immunotherapy...
January 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/26921483/refractory-status-epilepticus-and-autoimmune-encephalitis-with-gabaar-and-gad65-antibodies-a-case-report
#9
LETTER
Maude-Marie Gagnon, Martin Savard, Karim Mourabit Amari
PURPOSE: Autoimmune encephalitis is an inflammatory disorder of the brain that may be associated with different neuronal antibodies. Recently, an increasing number of valuable autoantibodies have been identified, including GABAAR antibodies, which appear to be associated with a severe form of encephalitis with refractory status epilepticus. We report here on a patient with encephalitis associated with GAD65 and GABAAR antibodies, an entity that remains an understudied topic, with an unanticipated clinical presentation and we describe the longitudinal follow-up...
April 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/26886357/progress-in-autoimmune-epileptic-encephalitis
#10
Sukhvir Wright, Angela Vincent
PURPOSE OF REVIEW: Autoimmune epileptic encephalopathy is a potentially treatable neurological syndrome characterized by the coexistence of a neuronal antibody in serum and, often, cerebrospinal fluid. Patients present with combinations of seizures, neuropsychiatric features, movement disorder, and cognitive decline, but some patients have isolated seizures either at first presentation or during their illness. This review summarizes our current understanding of the roles of specific neuronal antibodies in epilepsy-related syndromes and aims to aid the clinician in diagnosis and treatment...
April 2016: Current Opinion in Neurology
https://www.readbyqxmd.com/read/26878120/refractory-status-epilepticus-and-glutamic-acid-decarboxylase-antibodies-in-adults-presentation-treatment-and-outcomes
#11
Ayaz M Khawaja, Brannon L Vines, David W Miller, Jerzy P Szaflarski, Amy W Amara
Glutamic acid decarboxylase antibodies (GAD-Abs) have been implicated in refractory epilepsy. The association with refractory status epilepticus in adults has been rarely described. We discuss our experience in managing three adult patients who presented with refractory status epilepticus associated with GAD-Abs. Case series with retrospective chart and literature review. Three patients without pre-existing epilepsy who presented to our institution with generalized seizures between 2013 and 2014 were identified...
March 1, 2016: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/26711563/intrathecal-specific-glutamic-acid-decarboxylase-antibodies-at-low-titers-in-autoimmune-neurological-disorders
#12
Jun-Sang Sunwoo, Kon Chu, Jung-Ick Byun, Jangsup Moon, Jung-Ah Lim, Tae-Joon Kim, Soon-Tae Lee, Keun-Hwa Jung, Kyung-Il Park, Daejong Jeon, Ki-Young Jung, Manho Kim, Sang Kun Lee
Autoantibodies to glutamic acid decarboxylase (Gad-Abs) are implicated in various neurological syndromes. The present study aims to identify intrathecal-specific GAD-Abs and to determine clinical manifestations and treatment outcomes. Nineteen patients had GAD-Abs in cerebrospinal fluid but not in paired serum samples. Neurological syndromes included limbic encephalitis, temporal lobe epilepsy, cerebellar ataxia, autonomic dysfunction, and stiff-person syndrome. Immunotherapy had beneficial effects in 57.1% of patients, and the patients with limbic encephalitis responded especially well to immunotherapy...
January 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/26626229/autoimmune-epilepsy
#13
REVIEW
Antonio Greco, Maria Ida Rizzo, Armando De Virgilio, Michela Conte, Andrea Gallo, Giuseppe Attanasio, Giovanni Ruoppolo, Marco de Vincentiis
Despite the fact that epilepsy is the third most common chronic brain disorder, relatively little is known about the processes leading to the generation of seizures. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Besides, recent studies show that epilepsy and autoimmune disease frequently co-occur. Autoimmune epilepsy is increasingly recognized in the spectrum of neurological disorders characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy...
March 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/26608981/limbic-encephalitis-associated-with-anti-%C3%AE-aminobutyric-acid-b-receptor-antibodies-a-case-series-from-china
#14
Hong-Zhi Guan, Hai-Tao Ren, Xun-Zhe Yang, Qiang Lu, Bin Peng, Yi-Cheng Zhu, Xiao-Qiu Shao, Yong-Qiang Hu, Dong Zhou, Li-Ying Cui
BACKGROUND: Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABA B R) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of Han Chinese patients for further clinical refinement. METHODS: Serum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence...
November 20, 2015: Chinese Medical Journal
https://www.readbyqxmd.com/read/26597039/chronological-evolution-of-magnetic-resonance-imaging-findings-in-children-with-febrile-infection-related-epilepsy-syndrome
#15
Marianna S Rivas-Coppola, Namrata Shah, Asim F Choudhri, Robin Morgan, James W Wheless
OBJECTIVE: To describe and analyze the chronological evolution of the radiological findings in seven children with febrile infection-related epilepsy syndrome. METHODS: This is a retrospective study describing the radiological findings and evolution in seven children with febrile infection-related epilepsy syndrome who presented from 2009 to 2013. The children all fit the defined clinical criteria for febrile infection-related epilepsy syndrome; all had a history of normal psychomotor development who presented with acute-onset catastrophic partial status epilepticus associated with a febrile illness or unspecific infectious process...
February 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/26369378/-clinical-characteristics-of-four-patients-with-temporal-lobe-epilepsy-associated-with-elevated-anti-gad-antibodies
#16
Tetsuya Akaishi, Kazutaka Jin, Kazuhiro Kato, Hisashi Itabashi, Tatsuro Misu, Maki Tateyama, Masaki Iwasaki, Masashi Aoki, Nobukazu Nakasato
Anti-glutamic acid decarboxylase (GAD) antibodies are known to be associated with insulin-dependent diabetes mellitus (IDDM), stiff-person syndrome, and other neurological symptoms including temporal lobe epilepsy (TLE), known as autoimmune epilepsy. We treated four patients with TLE who had elevated titers of serum anti-GAD antibody (anti-GAD-Ab), higher than 100 U/ml. Three of the four patients started to have epileptic seizures in their 5th or 6th decade. Characteristic symptoms suggesting encephalitis or encephalopathy were absent at onset of these symptoms, which led to delayed diagnosis...
2015: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/26312868/the-active-intrathecal-b-cell-response-in-lgi1-antibody-encephalitis
#17
Sarosh R Irani, Klaus Lehmann-Horn, Michael Geschwind, Shengzhi Wang, Angela Vincent, H-Christian von Büdingen
BACKGROUND: Leucine-rich glioma inactivated 1 (LGI1) is a component of the voltage-gated potassium channel complex. IgG antibodies against LGI1 are associated with immunotherapy-responsive encephalitis and epilepsies. LGI1-antibody concentrations are 10-100 times greater in serum than in cerebrospinal fluid (CSF). Oligoclonal IgG bands are rarely found in patients with LGI1-antibody encephalitis or epilepsy. These observations raise questions about the sources of the B cells that result in production of LGI1 antibodies and how the IgGs reach the brain...
February 26, 2015: Lancet
https://www.readbyqxmd.com/read/26190012/anti-ma2-encephalitis-in-a-2-year-old-child-a-newly-diagnosed-case-and-literature-review
#18
Saloua Mrabet, Nedia Ben Achour, Ichraf Kraoua, Hanène Benrhouma, Hedia Klaa, Aida Rouissi, Malika Ben Ahmed, Ilhem Ben Youssef Turki
BACKGROUND: Anti-Ma2-associated encephalitis is a rare paraneoplastic neurological syndrome characterized by isolated or combined limbic, diencephalic, or brainstem dysfunction. It is rarely reported in children. CASE STUDY: We describe the clinical data of a 2-year-old girl referred to our department for refractory focal seizures associated with fever, followed by behavioural changes, speech disturbances and confusional episodes. Brain magnetic resonance imaging (MRI) showed left temporoparietal brain involvement...
November 2015: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/26060904/autoimmune-epilepsy
#19
REVIEW
Michel Toledano, Sean J Pittock
Seizures are recognized as a common manifestation of autoimmune limbic encephalitis and multifocal paraneoplastic disorders, but accumulating evidence supports an autoimmune basis for seizures in the absence of syndromic manifestations of encephalitis. Autoimmune encephalitis and epilepsy have been linked to neural-specific autoantibodies targeting both intracellular and plasma membrane antigens. The detection of these antibodies can serve as a diagnostic marker directing physicians toward specific cancers and can assist in therapeutic decision-making, but are not necessary to establish the diagnosis...
June 2015: Seminars in Neurology
https://www.readbyqxmd.com/read/25867463/clinical-manifestations-of-patients-with-caspr2-antibodies
#20
Jun-Sang Sunwoo, Soon-Tae Lee, Jung-Ick Byun, Jangsup Moon, Jung-Won Shin, Da-Eun Jeong, Gun-Hee Lee, Seong Ho Jeong, Yong-Won Shin, Keun-Hwa Jung, Doo Young Lee, Daejong Jeon, Ki-Young Jung, Manho Kim, Sang Kun Lee, Kon Chu
Contactin-associated protein-like 2 (CASPR2) is one of the target antigens of voltage-gated potassium channels (VGKC) complex antibodies. There has been relatively little information in the literature regarding CASPR2 autoimmunity, especially in Asian population. We investigated the presence of CASPR2 antibodies in patients with presumed autoimmune neurological disorders and described the clinical features, laboratory findings, and responses to immunotherapy. Five patients were identified to be positive for CASPR2 antibodies...
April 15, 2015: Journal of Neuroimmunology
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