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https://www.readbyqxmd.com/read/29057855/a-rare-case-of-autoimmune-limbic-encephalitis-an-uncharted-territory
#1
Hatim Ibrahim, Abdulelah N Al Jasser, Sonia A Khan, Kalthoum G Tlili
Autoimmune encephalitis is rare. Several auto- antibodies are described in autoimmune encephalitis. We describe a case of autoimmune limbic encephalitis associated with positive voltage gated potassium channel (VGKC) antibodies and positive leucine-rich glioma inactivated protein 1 antibodies (LGI1). A 33-year-old Saudi housewife, she presented with 2 months history of cognitive deterioration and recurrent left facio-brachial dystonic seizures followed by generalized tonic clonic seizures. At times the seizures are preceded by rising epigastric aura and shortness of breath...
October 2017: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29053037/possible-autoantibody-negative-limbic-encephalitis-after-anterior-temporal-lobectomy-for-hippocampal-sclerosis
#2
Sara Casciato, Alfredo D'Aniello, Addolorata Mascia, Pier Paolo Quarato, Marco De Risi, Liliana G Grammaldo, Vincenzo Esposito, Marco Zoccarato, Giancarlo Di Gennaro
Amnestic syndromes are acknowledged to be associated to bilateral hippocampal damage. We briefly report the case of a young man who underwent anterior left temporal lobectomy for a medically refractory temporal lobe epilepsy due to hippocampal sclerosis with an excellent seizure and neuropsychological outcome. Approximately 10 years later, he presented with a subacute severe global amnesia and MRI findings of a damage involving the contralateral mesial temporal lobe structures. A diagnosis of a possible autoimmune encephalitis was made...
October 20, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29050866/seizure-semiology-in-leucine-rich-glioma-inactivated-protein-1-antibody-associated-limbic-encephalitis
#3
Chao Chen, Xiu Wang, Chao Zhang, Tao Cui, Wei-Xiong Shi, Hong-Zhi Guan, Hai-Tao Ren, Xiao-Qiu Shao
OBJECTIVE: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE). METHODS: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated. RESULTS: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8)...
October 16, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29042524/-autoimmune-and-epilepsy
#4
Riki Matsumoto, Mitsuhiro Sakamoto, Akio Ikeda
The recent discovery of autoimmune antibodies to the neuronal cell surface membrane and extra- or intra-cellular proteins, such as NMDAR and LGI1, shed light on a proposed new etiology of epilepsy, namely, "autoimmune epilepsy". A large part of this entity most likely belongs to a forme fruste of autoimmune (limbic) encephalitis. Seizures are usually subacute in onset and refractory to antiepileptic medications. Patients occasionally manifest multiple seizure semiologies, such as autonomic or faciobrachial dystonic seizures...
October 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/28988523/immunopathology-in-drug-resistant-mesial-temporal-lobe-epilepsy-with-different-types-of-hippocampal-sclerosis
#5
F Irsel Tezer, Aysegul Firat, Erdem Tuzun, Isik Unal, Figen Soylemezoglu, Burcak Bilginer, Figen Kaymaz, Kader K Oguz, Serap Saygi
PURPOSE: There is evidence that autoimmunity has a specific role in temporal lobe seizures of limbic encephalitis patients. Our aim in this study was to investigate any histopathological clues of autoimmune process in refractory temporal lobe epilepsy (TLE) patients with different pathologically proven hippocampal sclerosis (HS) types. METHODS: 22 patients who had undergone to epilepsy surgery due to mesial TLE-HS were included. The sera of patients are tested for neuronal antibodies to NMDAR, LGI1, CASPR2, AMPAR, GABABR and GAD...
October 7, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28987176/immune-mediated-disorders
#6
Romana Höftberger, Hans Lassmann
Paraneoplastic and autoimmune encephalitis comprise a group of immune-mediated disorders that are associated with different immune effector mechanisms. Classic paraneoplastic neurologic syndromes are triggered by an antitumor immune response. The disease is considered to result from a T-cell response; in addition, patients harbour high titers of autoantibodies against intracellular antigens that are considered as epiphenomenon but are useful diagnostic markers. Neuropathology consists of T-cell-dominated inflammation, marked neuronal loss, and microglial activation with upregulation of HLA-DR...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28984777/anti-ma2-associated-limbic-encephalitis-with-coexisting-chronic-inflammatory-demyelinating-polyneuropathy-in-a-patient-with-non-hodgkin-lymphoma-a-case-report
#7
Weina Ju, Baochang Qi, Xu Wang, Yu Yang
RATIONALE: We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. PATIENT CONCERNS: The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28974152/encephalitis-with-antibodies-against-the-gabab-receptor-seizures-as-the-most-common-presentation-at-admission
#8
Xueping Chen, Fan Liu, Jin-Mei Li, Xiao-Qi Xie, Qiong Wang, Dong Zhou, Huifang Shang
OBJECTIVE: Autoimmune encephalitis associated with antibodies against gamma-aminobutyric-acid B receptor (GABABR) has not been described in detail in Chinese patients. METHODS: Patients with anti-GABABR encephalitis treated between January 2013 and December 2015 were analyzed in terms of clinical characteristics, laboratory findings, tumor presence, autoantibody patterns, treatment response and outcomes. RESULTS: Eleven patients were identified (male, N = 8; female, N = 3), with the median age of 51 years...
November 2017: Neurological Research
https://www.readbyqxmd.com/read/28965295/comprehensive-and-methodical-diagnostic-and-management-approaches-to-rapidly-progressive-dementia
#9
REVIEW
Supriya Mahajan, Brian S Appleby
Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone. Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer's disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD...
September 30, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28954337/-clinical-analysis-of-paraneoplastic-neurological-syndrome-associated-with-thymoma
#10
H S Liu, H T Ren, L X Zhou, S Q Li, B Peng, L Y Cui, H Z Guan
Objectives: To investigate the clinical features, diagnosis and treatment of antibody mediated paraneoplastic neurological syndrome associated with thymoma. Methods: From 2012 to 2017, the paraneoplastic antibody and neuron antibody were tested from both blood and cerebrospinal fluid (CSF) in consecutive patients clinically suspected with neurological paraneoplastic syndromes/unknown encephalitis in Peking Union Medical College Hospital.The clinical data, lab results, electrophysiological examinations, imaging features, treatment and clinical prognosis were collected...
September 19, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28948076/peripheral-neuropathy-in-limbic-encephalitis-with-anti-glutamate-receptor-antibodies-case-report-and-systematic-literature-review
#11
Yi-Chia Wei, Chin-Chang Huang, Chi-Hung Liu, Hung-Chou Kuo, Jainn-Jim Lin
INTRODUCTION: Autoantibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor and N-methyl-d-aspartate (NMDA) receptor are known to be the causes of autoimmune encephalitis particularly limbic encephalitis. The involvement of the peripheral nervous system is rarely reported. METHODS: We analyzed the serial nerve conduction studies of a previously reported case of anti-AMPA receptor encephalitis, who was presented with conscious disturbance and quadriplegia...
September 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28926420/seronegative-paraneoplastic-limbic-encephalitis-associated-with-thymoma
#12
Jaime Toro, David Cuellar-Giraldo, Alejandra Duque, Karla Minota, Jorge Patiño, Manuel García
Paraneoplastic limbic encephalitis is an autoimmune syndrome characterized by the acute or subacute onset of encephalopathy, memory loss, confusion, temporal lobe seizures, and behavioral and mood changes. Although most patients with paraneoplastic limbic encephalitis have antineuronal antibodies, advances in the field now permit the diagnosis without autoantibody test results. In this case illustrating the new diagnostic criteria, we report a 70-year-old woman who was brought to the emergency room after the acute onset of cognitive impairment, altered mental status, and choreoathetoid movements...
September 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28919330/limbic-encephalitis-with-lgi1-antibodies-in-a-14-year-old-boy
#13
M Schimmel, M C Frühwald, C G Bien
Limbic encephalitis (LE) with antibodies against leucine-rich glioma inactivated protein 1 (LGI1) is an auto-antibody mediated disorder with characteristic symptoms as dysfunction of memory, faciobrachial dystonic seizures and neuropsychiatric symptoms as emotional lability. Limbic encephalitis with LGI1 antibodies has been known so far as a disease of adults. We describe the case of a 14-year-old boy presenting with typical dysfunction of memory and LGI1 antibodies. To the best of our knowledge, this is the youngest patient with LGI1 antibody mediated limbic encephalitis described so far...
September 2, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28900443/hsv-1-encephalitis-high-index-of-clinical-suspicion-prompt-diagnosis-and-early-therapeutic-intervention-are-the-triptych-of-success-report-of-two-cases-and-comprehensive-review-of-the-literature
#14
Dimitrios Patoulias, Georgios Gavriiloglou, Konstantinos Kontotasios, Mairi Tzakri, Petros Keryttopoulos, Christos Koutras
Herpes Simplex Virus (HSV) encephalitis is an acute infectious disease of the Central Nervous System (CNS), usually affecting the limbic structures, the median temporal cortex, and the orbitofrontal regions. Its annual incidence has significantly increased over the last 20 years and the mortality rate is 7%, if early diagnosed and treated, and 70%, if left untreated, while it is associated with high rates of morbidity. It should be noted that even when Cerebrospinal fluid (CSF) analysis seems normal, imaging studies are not specific and HSV Polymerase Chain Reaction (PCR) test is negative; the clinician should be more aggressive, if clinical presentation is indicative for HSV encephalitis, by administrating acyclovir early after patient's admission...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28879090/faciobrachial-dystonic-seizures-result-from-fronto-temporo-basalganglial-network-involvement
#15
Rajesh Shankar Iyer, T C R Ramakrishnan, Karunakaran, Ajit Shinto, Koramadai Karuppuswamy Kamaleshwaran
•Faciobrachial dystonic seizures (FBDS) are caused by autoantibodies to leucine-rich glioma-inactivated1 proteins, a component of the voltage-gated potassium channel complex (VGKC-complex) and precede the clinical presentation of limbic encephalitis.•The exact pathophysiology of FBDS is not known and whether they are seizures or movement disorder is still debated.•We suggest the fronto-temporo-basal ganglia network involving the medial frontal and temporal regions along with the corpus striatum and substantia nigra being responsible for the clinical phenomenon of FBDS...
2017: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/28860940/limbic-encephalitis-a%C3%A2-report-of-four-cases
#16
Żanna Pastuszak, Adam Stępień, Kazimierz Tomczykiewicz, Renata Piusińska-Macoch, Joanna Kordowska, Dariusz Galbarczyk, Jarosław Świstak
Usually limbic encephalitis (LE) is a paraneoplastic neurologic syndrome. LE symptoms can precede cancer even by a few years. Almost 50% of LE cases are connected with small cell lung carcinoma. Testis and breast cancers, granulomatous disease, thymoma, and teratomas are also often connected with LE. Other cases have infectious and autoimmunological aetiology. In LE limbic system dysfunction is observed, and it is accompanied by cerebellum and brain stem abnormalities as well as polyneuropathy. Paraneoplastic limbic encephalitis is sometimes a part of larger syndrome in which brain stem and spinal cord are involved in an inflammatory process called paraneoplastic encephalomyelitis...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28795551/-gabab-paraneoplastic-encephalitis-about-a-clinical-case
#17
V Reiters, N Garzaniti, K Windhausen
The paraneoplastic limbic encephalitis is a rare disease. It is caused by the presence of autoantibodies creating an inflammatory reaction of the predominant brain parenchyma in the meso-temporal lobe and in other parts of the limbic system. Its presence requires looking for an underlying tumour. The management of this paraneoplastic syndrome includes an immunosuppressive therapy in addition to the treatment of the underlying tumour. However, the management of this disease is not yet standardized.
July 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28789769/-autoimmune-encephalitis-presenting-with-drug-resistant-status-epilepticus
#18
Mette Nissen, Morten Blaabjerg
Autoimmune encephalitis is characterized by formation of antibodies against cell surface proteins. Antibodies against the gamma-aminobuturic acidB (GABAB) receptor lead to limbic encephalitis, drug-resistant seizures, confusion, cognitive impairment and changed behaviour. Some patients present with status epilepticus. GABAB encephalitis is associated with small-cell lung cancer or neuroendocrine lung tumour. This is a case report of a patient having status epilepticus due to GABAB encephalitis. In cases which are presumed to be autoimmune it is important that treatment starts immediately instead of awaiting antibody confirmation...
July 24, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28781798/paraneoplastic-limbic-encephalitis-with-late-onset-magnetic-resonance-imaging-findings-a-case-report
#19
Yoshiya Tsunoda, Takumi Kiwamoto, Shinsuke Homma, Yuuki Yabuuchi, Haruna Kitazawa, Toshihiro Shiozawa, Kensuke Nakazawa, Takashi Hosaka, Kazuhiro Ishii, Akiko Ishii, Akira Tamaoka, Nobuyuki Hizawa
Paraneoplastic limbic encephalitis (PLE), a paraneoplastic neurological syndrome (PNS), is a rare nervous system disorder that results from the indirect effects of tumors and is commonly associated with small-cell lung cancer (SCLC). Previous studies have reported that magnetic resonance imaging (MRI) may be useful for diagnosing LE. Temporal lobe abnormalities are observed using T2-weighted and fluid-attenuated inversion recovery sequences; however, such abnormalities are detected in only 60% of patients with PLE...
August 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28750836/a-likely-case-of-limbic-encephalitis-in-a-patient-with-voltage-gated-potassium-channel-complex-antibody-without-a-known-antigenic-target-a-review-of-the-disease-state-and-value-of-antibody-titers
#20
David R Spiegel, Anastasia Samaras, Cameron L Oldham, Meghana Kaloji, Alyssa Warren, Shannon Maj, Sharmeen R Husain, Jeremy I Solomons, Stephanie Le
No abstract text is available yet for this article.
June 1, 2017: Psychosomatics
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