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Chronic Manganese Toxicity Associated with Voltage-Gated Potassium Channel Complex Antibodies in a Relapsing Neuropsychiatric Disorder.

Heavy metal poisoning is a rare but important cause of encephalopathy. Manganese (Mn) toxicity is especially rare in the modern world, and clinicians’ lack of recognition of its neuropsychiatric manifestations can lead to misdiagnosis and mismanagement. We describe the case of a man who presented with recurrent episodes of confusion, psychosis, dystonic limb movement and cognitive impairment and was initially diagnosed with anti-voltage-gated potassium channel (VGKC) complex limbic encephalitis in view of previous positive autoantibodies. His failure to respond to immunotherapy prompted …

Clinical characteristics and outcomes of patients with autoimmune encephalitis at a tertiary center in Saudi Arabia.

BACKGROUND: Autoimmune encephalitis (AE) is an important cause of subacute encephalopathy and seizure. Regional cohort-level data are limite … creened, 18 met the inclusion criteria, including 12 with antibody-positive AE, 5 with seronegative limbic encephalitis (LE), and 1 with probable AE. Anti-N-methyl-D-aspartate receptor encephalitis (n=9) occurred exclusively in young women (mean 17.2 years), with orofacial dyskinesia (44.4%) and one ovarian teratoma. Two patients with anti-glutamic acid decarboxylase …

Infection of SCID mice with Montana Myotis leukoencephalitis virus as a model for flavivirus encephalitis.

We have established a convenient animal model for flavivirus encephalitis using Montana Myotis leukoencephalitis virus (MMLV), a bat flavivirus. This virus has the same genomic organization, and contains the same conserved motifs in genes that encode potential antiviral targets, as flaviviruses that cause disease in man (N. Charlier et al., accompanying paper), and has a similar particle size (approximately 40 nm). MMLV replicates well in Vero cells and appears to be equally as sensitive as yellow fever virus and dengue fever virus to a selection of experimental antiviral agents. Cells infecte

Overview of therapeutic plasma exchange in pediatric neurology: a single-center experience.

Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspe … es of the central nervous system. Other indications were autoimmune encephalitis and paraneoplastic limbic encephalitis. No mortality was recorded during TPE. The complication rate was 2.2% and consisted of transient events like hypotension and allergic reactions. Therapetic plasma exchange is one of the safe methods of treatment for neuroimmunological disorders in c …

Disrupted AMPA Receptor Function upon Genetic- or Antibody-Mediated Loss of Autism-Associated CASPR2.

Neuropsychiatric disorders share susceptibility genes, suggesting a common origin. One such gene is CNTNAP2 encoding contactin-associated protein 2 (CASPR2), which harbours mutations associated to autism, schizophrenia, and intellectual disability. Antibodies targeting CASPR2 have also been recently described in patients with several neurological disorders, such as neuromyotonia, Morvan's syndrome, and limbic encephalitis. Despite the clear implication of CNTNAP2 and CASPR2 in neuropsychiatric disorders, the pathogenic mechanisms associated with alterations in CASPR2 function are unknown. Here…

Encephalitis with antibodies against the GABAB receptor: seizures as the most common presentation at admission.

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. All patients presented with seizures (N = 11; 100%), and they were given anti-epilepti

Antibody-positive paraneoplastic neurological syndromes associated with immune checkpoint inhibitors: a systematic review.

BACKGROUND AND OBJECTIVES: This study aimed to describe the clinical and prognostic characteristics of antibody-positive paraneoplastic neur … eurological symptoms within 6 months after ICIs treatment. The most common clinical phenotypes were limbic encephalitis (35.2%), rapidly progressive cerebellar syndrome (19.4%), and Lambert-Eaton myasthenic syndrome (13.0%), while the most common autoantibodies were anti-Hu (34.3%), anti-Ma2 (16.7%), and anti-P/Q VGCC (14.8%) antibodies. CSF inflammation was observed …

Cognitive Deficits in Anti-LGI1 Encephalitis Are Linked to Immunotherapy-Resistant White Matter Network Changes.

BACKGROUND AND OBJECTIVES: Cognitive deficits represent a major long-term complication of anti-leucine-rich, glioma-inactivated 1 encephalit … ve functions (r = -0.60, all pFDR = 0.017). DISCUSSION: Although traditionally viewed as a form of limbic encephalitis, our study characterizes LGI1-E as a "network disorder" that affects the whole brain. Structural reorganization of WM networks was linked to long-term and multidomain cognitive impairment, which was not prevented by immunotherapy. These findings hig …

Immune-mediated neurological manifestations of dengue virus- a study of clinico-investigational variability, predictors of neuraxial involvement, and outcome with the role of immunomodulation.

INTRODUCTION: Our aim was to study dengue-related immune-mediated neurological complications (IMNC) during the recent epidemic. MATERIALS A … days) latency period; however, there was no mortality seen. We found Miller Fisher syndrome (MFS), limbic encephalitis, and immune-mediated cerebellar demyelination (IMCD) as the new findings in the IMNC spectrum. Patients with DF were more prone to developing brachial plexus neuritis and polyneuritis cranialis, whereas those patients with a severe syndrome were mor …

Role of IFN-gamma in an experimental murine model of West Nile virus-induced seizures.

Seizures are a major complication of viral encephalitis. However, the mechanisms of seizure-associated neuronal dysfunction remain poorly understood. We report that intranasal inoculation with West Nile virus (WNV) (Sarafend) causes limbic seizures in C57BL/6 mice, but not in interferon (IFN)-gamma-deficient (IFN-gamma-/-) mice. Both strains showed similar levels of virus in the brain, as well as similar concentrations of the cytokines, tumor necrosis factor and interleukin-6, both of which can alter neuronal excitability. Experiments in chimeric IFN-gamma-/- mice reconstituted with IFN-gamma-

[An adult patient of Reye's syndrome--the possible background mechanism of lesions in claustrum, striatum and limbic system and limbic dementia].

We reported a 35-year-old male patient with Reye's syndrome, who showed temporarily lesions of claustrum, striatum, hippocampus and amygdala on CT, MRI and PET scan. He was previously healthy and affected upper respiratory infection. After taking 4 tablets of aspirin (335 mg x 4), he developed severe headache and status epileptics. The cerebrospinal fluid showed normal cell count and protein. The serum GOT and GPT elevated to 499 IU/l and 221 IU/l respectively. The parainfluenza virus type I titer was 32 times on admission and increased up to 128 times two weeks later. In spite of the anticonv

Глутаматные антитела в аутоиммунной центральной нервной системе: основные механизмы, клинические особенности и обнаружение антител.

На протяжении более 50 лет было признано, что воспаление головного мозга при помощи иммунной смеси является вторичным по отношению к лежащему в основе новообразованию. Некоторые из этих паранеопластических энцефалитидов выражаются в антителах сыворотки, но они не считаются патогенными, а вместо этого имеют патофизиологию с Т-клеток. За последние два десятилетия несколько патогенных антител против антигенов нейрональной поверхности были описаны в аутоиммунном энцефалите, поддающемся иммунной терапии. Некоторые из этих антител направлены против глютаматных рецепторов (ГлюР). Наиболее распростран

Совпадение в аннотации оригинала
Immune-mediated inflammation of the brain has been recognized for more than 50 years, although the initial descriptions were mainly thought … vely less common than NMDARE but is more likely to paraneoplastic. AMPAR antibodies typically cause limbic encephalitis, with patients presenting with confusion, disorientation, memory loss, and often seizures. The syndromes associated with the metabotropic receptor antibodies are much rarer and often can be paraneoplastic-mGluR1 (cerebellar degeneration) and mGluR5 …

BK virus encephalitis: case report, review of the literature, and description of a novel treatment modality.

OBJECTIVE: To describe a case of BK virus encephalitis with attempted direct antiviral therapy, review the reported cases of BK virus in the … ter transplantation, he developed fever and confusion. Magnetic resonance imaging was suggestive of limbic encephalitis and cerebrospinal fluid tested positive for BK virus. Therapy with intravenous cidofovir was thought to be indicated. Although probenecid is commonly used to minimize the toxicities of cidofovir, the patient's severe graft-versus-host disease raised …

Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center.

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, … litis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS co …

Autoantibody-mediated disorders of the central nervous system.

Autoimmunity has been demonstrated in a diverse range of peripheral neurological disorders, such as myasthenia gravis and acquired neuromyotonia. Serum antibodies found in these conditions are directed against ion channels and receptors situated on the cell surface and have been shown to produce pathogenic effects. The symptoms of these peripheral disorders have been transferred to animals by passive or active immunisation and, in humans, treated successfully with immunomodulatory therapy. Recently, a number of central nervous system disorders (CNS), such as limbic encephalitis, certain forms …

Serial proton MR spectroscopy and diffusion imaging findings in HIV-related herpes simplex encephalitis.

We report the case of pathologically proved atypical herpes simplex encephalitis (HSE) in a 40-year-old male patient with AIDS who was followed up by MR imaging, which included diffusion-weighted imaging and proton MR spectroscopy ((1)H-MRS). MR revealed sparing of hippocampi and limbic cortices, necrosis of both cingulate gyri, and cerebellar involvement. Increased diffusivity and severe metabolic alterations were compatible with biopsy findings of necrotizing inflammation. Clinical recovery corresponded with partial metabolite and diffusion normalization and a myo-inositol increase that indi

[Amnesia and stiff-man syndrome. Manifestations disclosing paraneoplastic encephalomyelitis].

The authors report a case of hypertonia, the clinical and electrophysiological features of which were identical to those of the Stiff-man syndrome first described by Moersch and Woltman in 1956. This and the five other previously reported cases can be attributed to encephalomyelitis of the anterior horns of the spinal cord. All of these cases had severe but partial involvement of the anterior horns and the probable physiopathological mechanism of the resulting contractures was elective destruction of the small inhibitory neurones. In the present case, the association of a limbic encephalitis a…

Local Use of Apisan Gel, A New Oral Care Product in the Treatment of Experimental Periodontitis Against the Background of Hyperacid Gastritis and Intoxication with Tobacco Smoke.

Hashimoto's encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antith … Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. In the context of the typical clinical picture, high titres of antithyroid antibodies, in particular antithyroid peroxidase antibodies, are diagnostic. These antibodies, however, can be detected in elevated titres in the healthy general population. …

A Rare Case of Human Herpesvirus 6 Meningitis in an Immunocompetent Asian Male Presented With a Severe Intractable Headache.

Human herpesvirus 6 (HHV-6) manifesting as a central nervous system (CNS) infection (especially meningoencephalitis) is reported as a primary infection in children and from reactivation in immunocompromised patients; however, it has rarely been reported in immunocompetent adults. Latent infections of the CNS can cause a myriad of clinical presentations ranging from a benign, febrile, self-resolving illness to limbic encephalitis, temporal lobe seizures, and neuropsychiatric symptoms such as behavioral disturbances and psychosis. No standard diagnostic criteria or management guidelines exist fo…