TY - JOUR. T1 - A case of paraneoplastic limbic encephalitis associated with small cell lung cancer. AU - Ryu, Ja Young. AU - Lee, Seung Hyeun. AU - Lee, Eun Joo. AU - Min, Kyung-Hoon. AU - Hur, Gyu Young. AU - Lee, Sung Yong. AU - Kim, Je Hyeong. AU - Lee, Sang Yeub. AU - Shin, Chol. AU - Shim, Jae Jeong. AU - In, Kwang Ho. AU - Kang, Kyung Ho. AU - Yoo, Se Hwa. PY - 2012/11/1. Y1 - 2012/11/1. N2 - Paraneoplastic limbic encephalitis (PLE) is a rare syndrome characterized by memory impairment, affective and behavioral disturbances and seizures. Among many different neoplasms known to cause PLE, small cell lung cancer (SCLC) is the most frequently reported. The pathogenesis is not fully understood but is believed to be autoimmune-related. We experienced a patient with typical clinical features of PLE. A 67-year-old man presented with seizure and disorientation. Brain magnetic resonance imaging demonstrated high signal intensity in the bilateral amygdala and hippocampus in flair and T2-weighted ...
Paraneoplastic limbic encephalitis (PLE) is a remote, nonmetastatic complication of carcinoma. Neuropsychiatric symptoms usually predate the diagnosis of cancer by 3 months to 6 years and very rarely the symptoms develop after the diagnosis of malignancy. We report the first case of limbic encephalitis associated with an immature ovarian teratoma. Within the month following the diagnosis of the tumor with pathologic stage Ia, somewhat acutely she developed neuropsychiatric symptoms that was exclusively a limbic disorder with impairments in almost every realm of limbic function. This case may show us that it is important to recognize the neuropsychiatric symptoms of PLE as the first manifestation of a very small malignant ovarian tumor and to aggressively try to identify the underlying cancer. ...
A thorough diagnostic evaluation typically includes cranial magnetic resonance imaging (MRI), electroencephalography (EEG), and cerebrospinal fluid (CSF) examination, but abnormalities may be nonspecific. This study was undertaken to define the spectrum of abnormalities found in the investigation of LE and to identify and compare any findings that may be specific to LE.3. PATIENTS AND METHODS. Clinical Material. Cases in which another neurological disorder was identified were excluded. Data from CT, MRI and CSF tests were considered to exclude other non-infective causes.. We retrospectively reviewed medical records of 57 patients with diagnosis of acute limbic encephalitis in the context of a febrile disease accompanied by headache, altered level of consciousness, and symptoms and signs of cerebral dysfunction (cognitive dysfunction, behavioural changes and seizures) with less than 7-day duration, from May 1994 to April 2010 and selected those with available EEG and MRI. We analyzed EEG ...
KEY POINTS Autoimmune limbic encephalitis is an inflammatory disease involving the medial temporal lobes; it classically presents with the subacute onset of short-term memory deficits, seizures or psychiatric symptoms. Brain magnetic resonance imaging can show medial temporal lobe abnormalities typical of autoimmune limbic encephalitis in suspected cases, but clinicians should be aware of other diseases that may have a similar imaging appearance. Analysis of both electroencephalogram (EEG) and cerebrospinal fluid can provide supportive evidence of neuro-inflammation in patients with suspected autoimmune limbic encephalitis, but a normal EEG or cerebrospinal fluid profile does not exclude the diagnosis. Testing for antibodies to onconeural, cell-surface and synaptic proteins represents a major advancement in the diagnosis of autoimmune limbic encephalitis, although false-positives are possible. Autoimmune limbic encephalitis (ALE) is an inflammatory disease involving the medial temporal lobes; it ...
OBJECTIVE: We describe a woman who presented with a psychiatric disorder post-partum and subsequently developed seizures and cognitive dysfunction prompting further investigation. A diagnosis of limbic encephalitis (LE) was made and antibodies to voltage-gated potassium channel complex (VGKC) detected. These antibodies are found in many non-paraneoplastic patients with LE. Although antibody-mediated conditions tend to present or relapse post-partum, VGKC-LE in the post-partum period has not been described. DESIGN: Case report. RESULTS: Clinical and imaging data were consistent with limbic encephalitis. High titres of anti-VGKC-complex antibodies confirmed the diagnosis of VGKC-LE. CONCLUSION: The similarities between the psychiatric symptomatology of VGKC-LE and post-partum psychiatric disorders raise the possibility that some instances of post-partum psychiatric conditions are manifestations of immune-mediated, non-paraneoplastic LE.
https://youtu.be/F8L1LQtfgVk Its been exactly 15 days since my last post and I feel as if its only been 2. Since Ive written, Ive visited Austin twice, accepted a 30 Day Squat Challenge, started my volunteer assignment at Coastal Bend Business Innovation Center, and began another MBA course. I also had my very first Blackberry Iced…
... is a form of encephalitis, a disease characterized by inflammation of the brain. Limbic encephalitis is caused by auto-immunity: an abnormal state where the body produces ...
Autoimmune encephalitis occurs in children and adolescents, often in association with other autoimmune diseases, and may be the cause of temporal lobe epilepsy. Immunomodulatory therapy may lead to a favorable outcome. So far, clinical data in children are rare and refer exclusively to paraneoplastic forms. The semiology of seizures involves temporal lobe and patients have behavioral and psychiatric problems. It is not rare for patients to be initially misdiagnosed. In a 16-year-old girl, non-paraneoplastic limbic encephalitis was diagnosed according to Bien and Elger. She had complex partial seizures and developed behavioral disturbances including anxiety, fear, feeling of unreality, auditory hallucinations and insomnia. Immune thrombocytopenia manifesting in chronic form was diagnosed at the age of six. EEG showed frontal fast beta activity. MRI scans demonstrated temporal swelling with T2/FLAIR signal increase. Cerebrospinal fluid showed pleocytosis and positive oligoclonal bands. Serologic ...
The Vaccine and Infectious Disease Divisions Dr. Joshua Hill is the recipient of the Biology of Blood and Marrow Transplantations 2012 George Santos Award for best clinical science article by a new investigator.. The editors of the journal, a publication of the American Society for Blood and Marrow Transplantation, selected Hill, a senior fellow in VIDDs Clinical Research Program, for his article Cord-Blood Hematopoietic Stem-Cell Transplantation Confers an Increased Risk for Human Herpesvirus-6-Associated Acute Limbic Encephalitis: A Cohort Analysis. Hill found that patients who receive unrelated cord-blood transplants are at particularly high risk for reactivation of human herpesvirus-6. They are also at risk for an associated complication called post-transplantation acute limbic encephalitis. Together the two are called HHV-6-PALE for short. HHV-6 is a ubiquitous virus that irreversibly infects the majority of children by 2 years of age and usually remains dormant throughout a persons ...
BRSK2 was initially identified through a computer screen of the human genome and shows significant homology to the C. elegans neuronal cell polarity regulator SAD1. BRSK2 is expressed in the brain and to a lesser extent in the testes. BRSK2 is a member of the AMP-activated protein kinase subfamily and can be activated by the tumor suppressor kinase LKB1. More recently, it has been shown that both BRSK2 and the related protein BRSK1 are required for mammalian neuronal polarization. While BRSK1- and BRSK2-null mice were viable, double-mutant mice died within two hours of birth. Neurons from these mice showed uniformly-sized neurites as opposed to the normal long axon and multiple shorter dendrites. These neurites also displayed both axonal and dendritic markers. BRSK2 has also been shown to be an autoantigen in paraneoplastic limbic encephalitis. At least four isoforms of BRSK2 are known to exist. ...
This case highlights autoimmune encephalitis as an important differential diagnosis in the management of optic neuritis not associated with MS or other demyelinating diseases.
If patients could recognise themselves, or anyone else could recognise a patient from your description, please obtain the patients written consent to publication and send them to the editorial office before submitting your response [Patient consent forms] ...
Antibodies that immunoprecipitate (125)I-alpha-dendrotoxin-labelled voltage-gated potassium channels extracted from mammalian brain tissue have been identified in patients with neuromyotonia, Morvans syndrome, limbic encephalitis and a few cases of adult-onset epilepsy. These conditions often improve following immunomodulatory therapies. However, the proportions of the different syndromes, the numbers with associated tumours and the relationships with potassium channel subunit antibody specificities have been unclear. We documented the clinical phenotype and tumour associations in 96 potassium channel antibody positive patients (titres |400 pM). Five had thymomas and one had an endometrial adenocarcinoma. To define the antibody specificities, we looked for binding of serum antibodies and their effects on potassium channel currents using human embryonic kidney cells expressing the potassium channel subunits. Surprisingly, only three of the patients had antibodies directed against the potassium channel
Contactin-associated protein-like 2 (Caspr2), also known as CNTNAP2, is a cell adhesion molecule that clusters voltage-gated potassium channels (...)
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대상군을 질환의 경과에 따라 급성기와 안정기로 분류하여 각 시기마다 자세한 신경심리검사(Seoul Neuropsychological Screening Battery [SNSB] Korean version of Rey-Kim memory test)를 실시하였다. 모든 신경심리검사는 구조적, 기능적 뇌영상의 결과를 반영하고 결과를 비교할 수 있도록 뇌 MRI와 18FDG-PET을 시행한 시기로부터 1주 내에 시행하였다. 동일한 한 명의 검사자에 의하여 대상군의 모든 신경심리검사가 실시되었다. 검사 항목으로는 주의력(digit span), 언어 및 관련 기능(스스로 말하기, 알아듣기, 따라말하기, 이름대기, 쓰기, 읽기, 계산, 좌우 지남력, 신체부위인지검사, 그리고 실행증검사), 시공간능력(오각형 겹쳐 그리기, 레이-복합도형), 언어적 기억력(서울언어학습검사 중 즉각회상, 지연회상, 재인검사), 비언어적 기억력(레이-복합도형에 대한 즉각회상, 지연회상, ...
AK5-Abs should be systematically considered in aged patients with subacute anterograde amnesia. Recognition of this disorder is important to develop new treatment strategies to prevent irreversible limbic damage. ...
Methods We recruited 38 patients (26 men, 12 women; median age 63.06 years; interquartile range [IQR] 16.06 years) in the postacute phase of aLE who completed questionnaires probing emotion regulation. All patients underwent structural/functional MRI postacutely, along with 67 age- and sex-matched healthy controls (40 men, 27 women; median age 64.70 years; IQR 19.87 years). We investigated correlations of questionnaire scores with demographic, clinical, neuropsychological, and brain imaging data across patients. We also compared patients diagnosed with pathologic tearfulness and those without, along with healthy controls, on gray matter volume, resting-state functional connectivity, and activity. ...
Several CNS disorders associated with specific antibodies to ion channels, receptors, and other synaptic proteins have been recognised over the past 10 years, and can be often successfully treated with immunotherapies. Antibodies to components of voltage-gated potassium channel complexes (VGKCs), NMDA receptors (NMDARs), AMPA receptors (AMPARs), GABA type B receptors (GABA(B)Rs), and glycine receptors (GlyRs) can be identified in patients and are associated with various clinical presentations, such as limbic encephalitis and complex and diffuse encephalopathies. These diseases can be associated with tumours, but they are more often non-paraneoplastic, and antibody assays can help with diagnosis. The new specialty of immunotherapy-responsive CNS disorders is likely to expand further as more antibody targets are discovered. Recent findings raise many questions about the classification of these diseases, the relation between antibodies and specific clinical phenotypes, the relative pathological roles of
Nuclear Neuroimaging is becoming an important diagnostic tool with increasing impact in neurological and neuro-oncological practice. An emerging use of brain FDG-PET is the early recognition of a potentially treatable cause of rapid progressive dementia. This is the case of Autoimmune Limbic Encephalitis, where brain FDG-PET findings may appear even before characteristic brain MRI hyperintensities. On the other hand, PET is a well-established method in systemic oncology, and is being increasingly used to supplement MRI in the clinical management of brain tumors. The evidence-based recommendation by the PET- Response Assessment in Neuro-Oncology working group (RANO) and the European Association of Neuro-Oncology (EANO) on the clinical use of PET imaging in gliomas, and the recently published joint guidelines of the SNMMI and EANM focusing on radiotracers that are used in clinical practice imaging, i.e. glucose metabolism, 2-deoxy-2-[18F]fluoro-D-glucose (FDG), and system L amino acid transport ...
Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis. Because autoantibody test results and response to therapy are not available at disease onset, we based the initial diagnostic approach on neurological assessment and conventional tests that are accessible to most clinicians. Through logical differential diagnosis, levels of evidence ...
Med Mal Infect. 2009 Nov 24. [Epub ahead of print] [Anti-NMDA-receptor encephalitis.] [Article in French] de Broucker T, Martinez-Almoyna L. Service de neurologie, hôpital Delafontaine, 2, rue du Dr-Delafontaine, 93200 Saint-Denis, France. Anti-NMDA-receptor encephalitis has been described only recently among other causes of paraneoplastic and auto-immune limbic encephalitis. Its frequency is probably underestimated. The…
Autoimmune encephalitis causes subacute deficits of memory and cognition, often followed by suppressed level of consciousness or coma. A careful history and examination may show early clues to particular autoimmune causes, such as neuromyotonia, hyperekplexia, psychosis, dystonia, or the presence of particular tumors. ...
Volunteer with INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY. Find INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY volunteering opportunities at VolunteerMatch!
Recommended Articles:. Horresh I, Poliak S, Grant S, et al.. 2008. Multiple Molecular Interactions Determine the Clustering of Caspr2 and Kv1 Channels in Myelinated Axons. JOURNAL OF NEUROSCIENCE 28(52): 14213-14222 Goldberg EM, Clark BD, Zagha E, et al.. 2008. K+ channels at the axon initial segment dampen near-threshold excitability of neocortical fast-spiking GABAergic interneurons. NEURON 58 (3): 387-400 Kole MHP, Letzkus JJ, Stuart GJ. 2007. Axon initial segment Kv1 channels control axonal action potential waveform and synaptic efficacy. NEURON 55( 4): 633-647 Gu C, Zhou W, Puthenveedu MA, et al. 2006. The microtubule plus-end tracking protein EB1 is required for Kv1 voltage-gated K+ channel axonal targeting. NEURON 52(5 ): 803-816 Kleopa KA, Elman LB, Lang B, et al. 2006. Neuromyotonia and limbic encephalitis sera target mature Shaker-type K+ channels: subunit specificity correlates with clinical manifestations. BRAIN 129(Part 6):1570-1584 Hasband MN, Park EW, Zhen DK, et al. 2002. ...
Recommended Articles:. Horresh I, Poliak S, Grant S, et al.. 2008. Multiple Molecular Interactions Determine the Clustering of Caspr2 and Kv1 Channels in Myelinated Axons. JOURNAL OF NEUROSCIENCE 28(52): 14213-14222 Goldberg EM, Clark BD, Zagha E, et al.. 2008. K+ channels at the axon initial segment dampen near-threshold excitability of neocortical fast-spiking GABAergic interneurons. NEURON 58 (3): 387-400 Kole MHP, Letzkus JJ, Stuart GJ. 2007. Axon initial segment Kv1 channels control axonal action potential waveform and synaptic efficacy. NEURON 55( 4): 633-647 Gu C, Zhou W, Puthenveedu MA, et al. 2006. The microtubule plus-end tracking protein EB1 is required for Kv1 voltage-gated K+ channel axonal targeting. NEURON 52(5 ): 803-816 Kleopa KA, Elman LB, Lang B, et al. 2006. Neuromyotonia and limbic encephalitis sera target mature Shaker-type K+ channels: subunit specificity correlates with clinical manifestations. BRAIN 129(Part 6):1570-1584 Hasband MN, Park EW, Zhen DK, et al. 2002. ...
Encephalitis, Patients, Antibodies, Syndromes, Potassium, Nmda, Limbic Encephalitis, Report, Cell, Cancer, Paraneoplastic Syndromes, Brain, Immunotherapy, Syndrome, Diagnosis, Potassium Channels, Treatment, Serum, Aspartate, D-aspartate
The importance of a wide ranging approach to assessment is well illustrated by the example of dementia, a primarily cognitive and behavioural disorder: the clue to diagnosis may come from general medical examination (revealing, for example, the testicular tumour causing limbic encephalitis or the bradycardia of hypothyroidism), from traditional neurological examination (subtle chorea in early Huntingtons disease), from cognitive assessment (isolated anterograde memory impairment in early Alzheimers disease), or from observation of behaviour (the patient with a frontal lobe dementia who leans over your desk and takes apart your pen). Moreover, this type of assessment is essential if one is to do justice to the symptoms of dementia which most bother patients: these are more often "psychiatric" than "neurological".. In this article we first drive home the general point that CNS disorders tend to give rise to cognitive and behavioural as well as "neurological" manifestations by considering some of ...
In article ,3f2vh5$2rq at news.rhrz.uni-bonn.de,, heinz at jersey.meb.uni-bonn.de (Heinz Beck) wrote: , Hi. , , Does anyone know of case reports in which hippocampal damage restricted to , or predominantly involving specific subfields has been related to memory , dysfunction. I am aware of some literature dealing with hippocampal damage , and memory dysfunction following ischemia or limbic encephalitis. However, , those studies mostly reported diffuse damage or complete destruction of , hippocampal structures. , , Thanks , Heinz Patient R.B. had damage restricted primarily to area CA1 (bilaterally). Check out Larry Squires paper in Psychological Review (early 1992 I think for references and histology). Shane OMara smomara at mail.tcd.ie ...
BACKGROUND AND PURPOSE: Autoimmune voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and suggest it in the differential diagnosis because this seizure etiology is likely under-recognized. Our purpose was to characterize MR imaging findings in this patient population. ...
Autoimmune encephalitis is usually a rare and newly described group of diseases involving autoantibodies directed against synaptic and neuronal cell surface antigens. autoimmune encephalitis incidence in psychiatric disease and the general guidelines for BMS-690514 the management of psychiatric manifestations. Rabbit polyclonal to ASH2L. For the majority of autoimmune encephalitis, the prognosis depends on the rapidity of the detection, identification, and the management of the disease. Because the presence of pronounced psychiatric symptoms drives patients to psychiatric institutions and can hinder the diagnosis, the aim of this work is to provide clues to help earlier detection by physicians and thus provide better medical care to patients. Keywords: neuroimmunology, autoantibodies, organic psychosis, dementia, schizophrenia Introduction Autoimmune encephalitis is usually a new and rare disease, characterized by brain inflammation and circulating autoantibodies. Numerous autoimmune ...
The widely-read journal Nature Reviews, in its specialized Neurology publication recently featured an article with a summary of the current state of knowledge and research related to neuroimmune illnesses that occur in children, including autoimmune encephalitis. Over a dozen experts collaborated on this paper. A sample of interesting points, which also largely apply to adults includes the following:. ...
TY - JOUR. T1 - Hippocampal epileptogenesis in autoimmune encephalitis. AU - Romoli, M.. AU - Krashia, P.. AU - Sen, A.. AU - Franciotta, D.. AU - Gastaldi, M.. AU - Nobili, A.. AU - Mancini, A.. AU - Nardi Cesarini, E.. AU - Nigro, P.. AU - Tambasco, N.. AU - Mercuri, N.B.. AU - Parnetti, L.. AU - Di Filippo, M.. AU - DAmelio, M.. AU - Irani, S.R.. AU - Costa, C.. AU - Calabresi, P.. N1 - cited By 0. PY - 2019. Y1 - 2019. U2 - 10.1002/acn3.50919. DO - 10.1002/acn3.50919. M3 - Articolo. VL - 6. SP - 2261. EP - 2269. JO - Annals of Clinical and Translational Neurology. JF - Annals of Clinical and Translational Neurology. SN - 2328-9503. IS - 11. ER - ...
OBJECTIVE: We tested whether antibody screening samples of patients with suspected autoimmune encephalitis with additional research assays would improve the detection of autoimmune encephalitis compared with standard clinical testing alone. METHODS: We examined 731 samples (333 CSF, 182 sera, and 108 pairs) from a cohort of 623 patients who were tested for CNS autoantibodies by the University of Pennsylvania clinical laboratory over a 24-month period with cell-based assays (CBAs) on commercially obtained slides of fixed cells for antibodies to NMDA receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-B receptor (GABABR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (Caspr2), and glutamic acid decarboxylase (GAD65 ...
Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AE-specific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare. This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses. Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided
OBJECTIVE: We tested whether antibody screening samples of patients with suspected autoimmune encephalitis with additional research assays would improve the detection of autoimmune encephalitis compared with standard clinical testing alone. METHODS: We examined 731 samples (333 CSF, 182 sera, and 108 pairs) from a cohort of 623 patients who were tested for CNS autoantibodies by the University of Pennsylvania clinical laboratory over a 24-month period with cell-based assays (CBAs) on commercially obtained slides of fixed cells for antibodies to NMDA receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-B receptor (GABABR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (Caspr2), and glutamic acid decarboxylase (GAD65 ...
Research Areas: Behavioural neuroscience: Associative learning, its neural substrates, and neural network models, including adaptive timing, learning-to-learn, extinction, savings, configural learning, and selective attention. Instructional design: Working memory, schema formation, computer learning.. ...
Depending on the number and character of their functional groups, steroid molecules may show diverse reactivities. Moreover, the reactivity of a functional group varies according to its location within the molecule (for example, esters are formed
The journal focuses on neuroimmunology and neuroinflammation, and the coverage extends to other basic and clinical studies related to neuroscience including molecular biology, psychology, pathology, physiology, endocrinology, pharmacology, oncology, etc.
The journal focuses on neuroimmunology and neuroinflammation, and the coverage extends to other basic and clinical studies related to neuroscience including molecular biology, psychology, pathology, physiology, endocrinology, pharmacology, oncology, etc.
If you have a question about this talk, please contact Dr Julia Deakin.. Abstract not available. This talk is part of the Department of Psychiatry & CPFT Thursday Lunchtime Seminar series.. ...
Limbic encephalitis with antibodies to the voltage-gated potassium channel-complex (VGKC-LE) is now a well-established neurological syndrome. Recent work demonstrates many patients suffer from persisting anterograde amnesia following the resolution of VGKC-LE. However, no detailed assessment of retrograde memory, episodic or otherwise, has yet been undertaken. We assessed 14 VGKC-LE patients and 14 age-matched controls were assessed on a detailed measure of retrograde episodic memory (the Autobiographical Interview). One episodic memory was obtained from each decade of life (six in total). The results show that VGKC-LE patients had significant deficits in episodic recall across the most recent 40 years of their life, in the context of preserved personal semantic memory (as measured by the Autobiographical Memory Interview). Moreover, ultra-high field magnetic resonance imaging suggests that VGKC-LE is associated with focal hippocampal atrophy. These data demonstrate that retrograde amnesia is ...
We describe a patient presenting with a combination of muscle fasciculations, paresthesias, hyperhidrosis, as well as insomnia, agitation and confusion. He went on to develop psychosis and respiratory failure requiring intensive care. Electromyography confirmed the presence of neuromyotonia and CSF showed mild pleocytosis. Routine testing for voltage-gated potassium channel complex (VGKC-complex) antibodies was highly positive, confirming the clinical diagnosis of Morvans syndrome. The patient improved after treatment with intravenous immunoglobulin and methylprednisolone. Further investigation of the antigenic targets using immunohistochemistry and cell-based assays revealed that he had autoantibodies targeting Lgi1, Caspr2 and Contactin-2/Tag-1, all proteins known to be complexed with VGKC in peripheral nerves and CNS. This is the first case of Morvans syndrome from Cyprus and illustrates the clinical features as well as the emerging complexity of antigenic targets involved in the pathogenesis.
BACKGROUND: GQ1b antibody (GQ1b-Ab) is detected in approximately two-thirds of sera of patients with Bickerstaffs encephalitis (BE). Whilst some of the remaining patients have antibodies to other gangliosides, many patients with BE are reported to be seronegative. METHODS AND RESULTS: Voltage-gated potassium channel antibody (VGKC-Ab) at high titer was detected during the diagnostic work-up of one patient with BE. Sera of an additional patient with BE and nine patients with Miller Fisher syndrome (MF) (all GQ1b-Ab positive) were investigated for VGKC-Ab and other anti-neuronal antibodies by radioimmunoprecipitation using 125I-dendrotoxin-VGKC and immunohistochemistry, respectively. Two patients with MF exhibited moderate titer VGKC-Abs. Regardless of positivity for VGKC or GQ1b antibodies, serum IgG of all patients with BE and MF reacted with the molecular layer and Purkinje cells of the cerebellum in a distinctive pattern. CONCLUSION: Voltage-gated potassium channel antibodies might be involved in some
Polyclonal antibody for KV1.1/KCNA1 detection. Host: Rabbit.Size: 100μg/vial. Tested applications: WB. Reactive species: Human. KV1.1/KCNA1 information: Molecular Weight: 56466 MW; Subcellular Localization: Cell membrane ; Multi- pass membrane protein . M
Background Patients with intracellular onconeural antibodies may present with neuro-psychiatric syndromes. We aimed to evaluate the evidence for an association between well-characterized onconeural...
The cyclists route will cover 100 miles of beautiful Wiltshire countryside over the two days, passing landmarks such as the Avebury Stone Circle, Stonehenge and the Wiltshire White Horses.. The VEKA UK Group chose to support The Encephalitis Society after Mark Barsby, Head of Technical was diagnosed with an autoimmune form of Limbic Encephalitis last year. He - in discussion with Head of Commercial Sales, Richard Garland and Head of Product Management, Steve Bakewell - planned the challenge as part of the companys year-long fundraising programme for the charity.. Mark explained: "I wanted to do my bit and put something back for the help The Encephalitis Society had given me. Steve gave us some ideas as he had already taken part in similar events to raise money for charity.. "I decided to make some changes to my diet and exercise more when I came out of hospital. As part of my goal, however, Ive promised myself an ice cold beer and a hot sausage roll on completion of the second day as theyre ...
A budget friendly kit of Kir channel antibodies from Alomone Labs, ideal for screening purposes. An economical way to sample Abs. Control antigens included. Lyophilized. Worldwide shipping at room temperature. Top supplier for inward rectifier K+ channel research! Join the thousands of researchers using our products.
An extensive body of literature has accrued on the imaging of the human hippocampus since it was first visualized clearly in a living person using magnetic resonance imaging (MRI) in the late 1980s. Clinicians now routinely perform high-resolution MRI to inspect the structure of the hippocampus and surrounding cortices in detail in patients suspected of having Alzheimers disease (AD) and related disorders, temporal lobe epilepsy and related conditions, or limbic encephalitis, transient global amnesia, or other forms of amnesia. Researchers have developed protocols for measuring the size of the hippocampus and are now working toward visualization and measurement of hippocampus subfields in vivo. Investigators are also using advanced imaging techniques to measure hippocampal activity during memory and other tasks as well as its perfusion, metabolism, and molecular constituents. New technical advances in imaging continue to be applied to the investigation of the hippocampus, medial temporal ...