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https://www.readbyqxmd.com/read/28432047/delayed-lgi1-seropositivity-in-voltage-gated-potassium-channel-vgkc-complex-antibody-limbic-encephalitis
#1
Michael Sweeney, Jonathan Galli, Scott McNally, Anne Tebo, Thomas Haven, Perla Thulin, Stacey L Clardy
We utilise a clinical case to highlight why exclusion of voltage-gated potassium channel (VGKC)-complex autoantibody testing in serological evaluation of patients may delay or miss the diagnosis. A 68-year-old man presented with increasing involuntary movements consistent with faciobrachial dystonic seizures (FBDS). Initial evaluation demonstrated VGKC antibody seropositivity with leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) seronegativity. Aggressive immunotherapy with methylprednisolone and plasmapheresis was started early in the course of his presentation...
April 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28424014/semi-quantitative-analysis-of-cerebral-fdg-pet-reveals-striatal-hypermetabolism-and-normal-cortical-metabolism-in-a-case-of-vgkcc-limbic-encephalitis
#2
Patrick Moloney, Ruth Boylan, Marwa Elamin, Sean O'Riordan, Ronan Killeen, Christopher McGuigan
In the context of delayed autoimmune encephalitis antibody results, functional imaging can support the diagnosis of limbic encephalitis associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies. Here we present a typical case of VGKCC encephalitis in a 69-year-old woman whose symptoms responded to plasmapheresis. A cerebral 18F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) scan performed prior to commencing treatment revealed striatal hypermetabolism assessed qualitatively and semi-quantitatively, with normal uptake in the cortex and cerebellum when analysed semi-quantitatively...
April 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28417344/biotherapy-in-inflammatory-diseases-of-the-cns-current-knowledge-and-applications
#3
REVIEW
Nicolas Collongues, Laure Michel, Jérôme de Seze
Biotherapy represents an innovative therapeutic approach that includes immunotherapy (vaccines, apheresis, and antibodies); gene therapy; and stem cell transplants. Their development helps to cross the bridge from bench to bedside and brings new hope of a cure for severe diseases in different fields of medicine. In neurology, a growing range of applications is being developed for these medications. Valuable results are now available in the field of autoimmunity, neuro-oncology, paraneoplastic manifestations, and neurodegenerative disorders...
May 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28413671/paraneoplastic-limbic-encephalitis-in-a-patient-with-extensive-disease-small-cell-lung-cancer
#4
Sebastian Ochenduszko, Bartosz Wilk, Joanna Dabrowska, Izabela Herman-Sucharska, Anna Dubis, Miroslawa Puskulluoglu
Paraneoplastic limbic encephalitis (PLE) is a rare disorder infrequently accompanying malignancy, coexisting in ~50% of the cases with small-cell lung cancer (SCLC). The pathomechanism of PLE is considered to be immune-mediated, with production of specific anti-Hu antibodies and activation of T-cells directed against onconeural antigens present on both tumor cells and neurons. We herein report the case of a 50-year-old male patient who, prior to being diagnosed with SCLC, presented with typical symptoms of PLE (seizures, subacute cognitive dysfunction with severe memory impairment, anxiety and hallucinations)...
April 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28396782/small-cell-lung-carcinoma-and-acute-onset-of-antiglial-nuclear-antibody-positive-limbic-encephalitis
#5
Melvin Chan, Rajesh Rangaswamy, Yen-Yi Peng
Limbic encephalitis (LE) can present as a nonspecific manifestation preceding neoplastic disease. Having high clinical suspicion and using newer onconeural antibodies, like antiglial nuclear antibody (AGNA), can lead to an earlier diagnosis. We report a patient with AGNA-positive LE who is later diagnosed and treated for small-cell lung carcinoma.
April 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28389060/paraneoplastic-limbic-encephalitis-with-sox1-and-pca2-antibodies-and-relapsing-neurological-symptoms-in-an-adolescent-with-hodgkin-lymphoma
#6
M Kunstreich, J H Kreth, P T Oommen, J Schaper, M Karenfort, O Aktas, D Tibussek, F Distelmaier, A Borkhardt, M Kuhlen
BACKGROUND: Immune cross-reactivity between malignant and normal tissues causes the rare, so called paraneoplastic syndrome (PS). In approximately 60% of the patients, various onconeural antibodies are detectable in the cerebrospinal fluid (CSF) and are associated with typical tumour entities. METHODS: We report an unusual case of paraneoplastic limbic encephalitis (PLE) in a 17-year-old adolescent with classical Hodgkin lymphoma. RESULTS: He presented with a variety of neurologic and neuropsychiatric symptoms, profound B-symptoms and typical MRI findings including hyperintense lesions with contrast enhancement in the medial temporal lobe and limbic system...
March 27, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28369215/focal-ca3-hippocampal-subfield-atrophy-following-lgi1-vgkc-complex-antibody-limbic-encephalitis
#7
Thomas D Miller, Trevor T-J Chong, Anne M Aimola Davies, Tammy W C Ng, Michael R Johnson, Sarosh R Irani, Angela Vincent, Masud Husain, Saiju Jacob, Paul Maddison, Christopher Kennard, Penny A Gowland, Clive R Rosenthal
Magnetic resonance imaging has linked chronic voltage-gated potassium channel (VGKC) complex antibody-mediated limbic encephalitis with generalized hippocampal atrophy. However, autoantibodies bind to specific rodent hippocampal subfields. Here, human hippocampal subfield (subiculum, cornu ammonis 1-3, and dentate gyrus) targets of immunomodulation-treated LGI1 VGKC-complex antibody-mediated limbic encephalitis were investigated using in vivo ultra-high resolution (0.39 × 0.39 × 1.0 mm3) 7.0 T magnetic resonance imaging [n = 18 patients, 17 patients (94%) positive for LGI1 antibody and one patient negative for LGI1/CASPR2 but positive for VGKC-complex antibodies, mean age: 64...
March 28, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28365191/-thymoma-and-autoimmune-diseases
#8
Y Jamilloux, H Frih, C Bernard, C Broussolle, P Petiot, N Girard, P Sève
The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later...
March 29, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28363946/emergence-of-new-onset-psychotic-disorder-following-recovery-from-lgi1-antibody-associated-limbic-encephalitis
#9
Thomas A Pollak, Nick Moran
Neuronal autoantibodies targeting cell surface antigens have been described in association with autoimmune encephalitides which frequently feature psychosis and other psychiatric disturbances alongside neurological signs and symptoms. Little has been written however about the long-term psychiatric status of individuals following recovery from the acute phase of autoimmune encephalitis, despite case series and anecdotal evidence suggesting this may be a cause of considerable disability. Here, we describe a man aged 58 years with no psychiatric history who developed a severe and acute psychotic disorder following resolution of a protracted course of limbic encephalitis associated with antibodies to leucine-rich glioma inactivated 1 protein...
March 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28360986/confusion-faciobrachial-dystonic-seizures-and-critical-hyponatremia-in-a-patient-with-voltage-gated-potassium-channel-encephalitis
#10
Julian Yaxley
Autoimmune limbic encephalitis is a rare cause of encephalitic disease. It is associated with various target antigens and is difficult to diagnose, and experience with its treatment is limited. This case report describes a 69-year-old man, who presented with life-threatening hyponatremia and confusion, following several months of gradually worsening faciobrachial dystonic seizures. Faciobrachial dystonic seizures are a well-described feature classically observed in voltage-gated potassium channel autoimmune encephalitis...
March 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28306571/cns-syndromes-associated-with-antibodies-against-metabotropic-receptors
#11
Eric Lancaster
PURPOSE OF REVIEW: Autoantibodies to Central nervous system (CNS) metabotropic receptors are associated with a growing family of autoimmune brain diseases, including encephalitis, basal ganglia encephalitis, Ophelia syndrome, and cerebellitis. The purpose of this review is to summarize the state of knowledge regarding the target receptors, the neurological autoimmune disorders, and the pathogenic mechanisms. RECENT FINDINGS: Antibodies to the γ-aminobutyric acid B receptor are associate with limbic encephalitis and severe seizures, often with small cell lung cancers...
March 16, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28284337/autoimmune-limbic-encephalitis-with-anti-contactin-associated-protein-like-2-antibody-secondary-to-pembrolizumab-therapy
#12
Michael P Brown, Pravin Hissaria, Amy Hc Hsieh, Christopher Kneebone, Wilson Vallat
Immune checkpoint inhibitors such as Pembrolizumab are used to restore antitumour immune response. It is important to be vigilant of immune mediated adverse events related to such therapy. We report a case of autoimmune limbic encephalitis with Contactin-Associated Protein-like 2 (CASPR2) antibody secondary to Pembrolizumab therapy for metastatic melanoma.
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28272206/limbic-encephalitis-associated-with-anti-nh2-terminal-of-%C3%AE-enolase-antibodies-a-clinical-subtype-of-hashimoto-encephalopathy
#13
Toru Kishitani, Akiko Matsunaga, Masamichi Ikawa, Kouji Hayashi, Osamu Yamamura, Tadanori Hamano, Osamu Watanabe, Keiko Tanaka, Yasunari Nakamoto, Makoto Yoneda
Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagnosed with HE and respond to immunotherapy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28268128/mr-imaging-of-adult-acute-infectious-encephalitis
#14
REVIEW
A Bertrand, D Leclercq, L Martinez-Almoyna, N Girard, J-P Stahl, T De-Broucker
BACKGROUND: Imaging is a key tool for the diagnosis of acute encephalitis. Brain CT scan must be urgently performed to rule out a brain lesion with mass effect that would contraindicate lumbar puncture. Brain MRI is less accessible than CT scan, but can provide crucial information with patients presenting with acute encephalitis. METHOD: We performed a literature review on PubMed on April 1, 2015 with the search terms "MRI" and "encephalitis". RESULTS: We first described the various brain MRI abnormalities associated with each pathogen of acute encephalitis (HSV, VZV, other viral agents targeting immunocompromised patients or travelers; tuberculosis, listeriosis, other less frequent bacterial agents)...
March 3, 2017: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/28259863/-review-of-the-psychiatric-aspects-of-anti-nmda-n-methyl-d-aspartic-acid-receptor-encephalitis-case-report-and-our-plans-for-a-future-study
#15
Levente Herman, Ildiko Reka Zsigmond, Laszlo Peter, Janos M Rethelyi
Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. In most of the cases, typical neurological symptoms appear later. Besides the clinical picture and typical symptoms, verifying presence of IgG antibodies in the serum or CSF is necessary to set up the diagnosis...
December 2016: Neuropsychopharmacologia Hungarica
https://www.readbyqxmd.com/read/28248701/autoimmune-encephalitis-with-anti-leucine-rich-glioma-inactivated-1-or-anti-contactin-associated-protein-like-2-antibodies-formerly-called-voltage-gated-potassium-channel-complex-antibodies
#16
Anna E M Bastiaansen, Agnes van Sonderen, Maarten J Titulaer
PURPOSE OF REVIEW: Twenty years since the discovery of voltage-gated potassium channel (VGKC)-related autoimmunity; it is currently known that the antibodies are not directed at the VGKC itself but to two closely associated proteins, anti-leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2). Antibodies to LGI1 and Caspr2 give well-described clinical phenotypes. Anti-LGI1 encephalitis patients mostly have limbic symptoms, and anti-Caspr2 patients have variable syndromes with both central and peripheral symptoms...
February 28, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28223808/n-methyl-d-aspartate-receptor-antibody-could-be-a-cause-of-catatonic-symptoms-in-psychiatric-patients-case-reports-and-methods-for-detection
#17
Ko Tsutsui, Takashi Kanbayashi, Manabu Takaki, Yuki Omori, Yumiko Imai, Seiji Nishino, Keiko Tanaka, Tetsuo Shimizu
The symptoms of catatonia have been reported to be similar to the initial symptoms of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Subsequently, this autoimmune limbic encephalitis has been noticed by many psychiatrists. For a differential diagnosis of catatonic state, it is important to detect anti-NMDAR encephalitis. This encephalitis is expected to be in remission by early detection and treatment. We should be more cautious about catatonic symptoms of schizophrenia. When a patient is suspected of having encephalitis, we should screen for anti-NMDAR antibodies in cerebrospinal fluid samples using a cell-based assay...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28222692/hashimoto-s-encephalitis-associated-with-ampar2-antibodies-a-case-report
#18
Mingqin Zhu, Xuefan Yu, Caiyun Liu, Chenchen Duan, Chunxiao Li, Jie Zhu, Ying Zhang
BACKGROUND: Hashimoto's encephalitis (HE) is a rare neurological complication of Hashimoto's thyroiditis (HT), while limbic encephalitis (LE) is an autoimmune inflammatory disorder frequently associated with anti-neuronal antibodies. The glutamate receptor α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptor (AMPAR) is important for synaptic transmission, memory, and learning. The etiology of HE remains unclear. We present a case of HE with antibodies to AMPAR2 both in the serum and cerebrospinal fluid...
February 21, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28194064/therapeutic-plasma-exchange-in-patients-with-neurologic-disorders-review-of-63-cases
#19
Anil Tombak, Mehmet Ali Uçar, Aydan Akdeniz, Arda Yilmaz, Hakan Kaleagası, Mehmet Ali Sungur, Eyup Naci Tiftik
Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1)...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28183838/autoimmune-encephalitis-pathophysiology-and-imaging-review-of-an-overlooked-diagnosis
#20
REVIEW
B P Kelley, S C Patel, H L Marin, J J Corrigan, P D Mitsias, B Griffith
Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteristic findings within limbic structures suggestive of autoimmune encephalitis can be a key step in alerting clinicians to the potential diagnosis and ensuring a prompt and appropriate clinical work-up...
February 9, 2017: AJNR. American Journal of Neuroradiology
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