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Autoimmune Encephalitis.

Autoimmune encephalitides (AE) constitute a broad group of inflammatory brain disorders characterized by prominent neuropsychiatric symptoms, frequently in association with autoantibodies against neural (neuronal or glial) antigens. The most frequent AE are anti-NMDA receptor encephalitis, acute disseminated encephalomyelitis (associated with MOG antibodies in 60% of patients), and limbic encephalitis (with several immunologic subtypes, anti-LGI1 encephalitis being the most frequent). The first 2 predominantly affect children and young adults, whereas limbic encephalitis usually affects patien…

[Pediatric autoimmune encephalitis associated with anti-glutamic acid decarboxylase 65 antibody: two cases report and literature review].

Objective: To investigate the clinical features and prognosis of pediatric autoimmune encephalitis associated with anti-glutamic acid decarb … tients had short-term improvement. Follow-up for 6 months to 6 years showed that 3 cases with extra limbic encephalitis improved to baseline levels, and 5 limbic encephalitis cases had poor outcomes, including 1 death and 4 cases still had focal epilepsy. Conclusions: Pediatric anti-GAD65 antibody associated autoimmune encephalitis is a rare but treatable disease, in …

Mild Encephalitis/Encephalopathy with reversible splenial lesion syndrome: An unusual presentation of anti-GFAP astrocytopathy.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare recently defined antibody-mediated encephalitis. Meningo-encephalomyelitis presentation is frequent with lymphocytic pleiocytosis in the cerebro-spinal fluid and brain MRI classically demonstrates in 50% of cases, a linear perivascular enhancement extending radially from the ventricles. Here, we describe 2 cases of pediatric autoimmune GFAP astrocytopathy with limbic encephalitis presentation and peculiar MRI characteristics: one with normal MRI and the second suggestive of Mild Encephalitis/Encephalopathy with reversib…

Progressive limbic encephalopathy: Problems and prospects.

BACKGROUND: It was observed that a good number of patients presenting with psychiatric manifestations when investigated later because of unresponsiveness to treatment or late development of organic features turned out to be treatable limbic syndromes. INTRODUCTION: The aim of this study is to assess the patients presenting with new onset neuropsychiatric symptoms satisfying the criteria for probable limbic encephalitis. PATIENTS AND METHODS: Patients referred to neurology department following a period of treatment for neuropsychiatric symptoms, which did not respond to conventional treatment…

Wait and see: a 5 year history of 'recurrent dementia'.

Rapid evolving cognitive impairment can be the onset of either a progressive or a treatable dementia. We describe a case of a man in whom the clinical and laboratory presentation suggested a diagnosis of Creutzfeldt-Jakob disease, which subsequently turned into a diagnosis of voltage-gated potassium channel complex antibodies limbic encephalitis. The natural history and an increased knowledge of limbic encephalitis and the related antibodies led us to the correct diagnosis.

A case of anti-VGKC antibody encephalitis and prolonged encephalopathy despite spontaneous resolution of imaging abnormalities.

UNLABELLED: Anti-voltage-gated potassium channel (anti-VGKC) antibody encephalitis is a common form of autoimmune encephalitis (AE). AE is usually associated with autoimmune diseases or paraneoplastic phenomena such as seen in small cell lung cancer. Clinical presentation can include memory impairment, seizures, and psychiatric symptoms. We report a case of a 72-year-old male with non-small lung cancer in remission who presented with erosive gastritis and acute severe encephalopathy. Anti-VGKC antibody limbic encephalitis was diagnosed. Spontaneous resolution of encephalitis-associated changes…

Anti Ma2-associated myeloradiculopathy: expanding the phenotype of anti-Ma2 associated paraneoplastic syndromes.

Anti-Ma2 associated paraneoplastic syndrome usually presents as limbic encephalitis in association with testicular tumours., Only four patients have been reported with involvement outside the CNS, two of whom also had limbic or brainstem encephalitis., We report a man with anti- Ma2 associated myeloradiculopathy and previous testicular cancer whose neurological syndrome stabilised and anti-Ma2 titres fell following orchidectomy of a microscopically normal testis.

Case report: Overlapping anti-AMPAR encephalitis with anti-IgLON5 disease post herpes simplex virus encephalitis.

Autoimmune encephalitis (AE) is the result of an autoimmune process that occurs as a rapidly advancing encephalopathy. Autoimmune encephalitis was commonly linked to herpes simplex virus 1 (HSV-1) as the most frequently identified virus. The main areas affected by this invasion are the temporal lobe, frontal lobe, and limbic system. Limbic encephalitis is a highly uncommon occurrence involving anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis and anti-IgLON family member 5 (IgLON5) disease, both belonging to the rare category. As far as we know, this i…

[Autoimmune Associated Encephalitis and Dementia].

Antibodies against various neural surface antigens induce cognitive impairments. Anti-VGKC (voltage gated potassium channel) complex antibodies are well known as one of the causative autoantibodies. An anti-VGKC antibody was identified as the autoantibody in acquired neuromyotonia (Isaacs' syndrome), which causes muscle cramps and difficulty in opening the palm of the hands. However, this antibody also tests positive in autoimmune limbic encephalitis, which has a subacute progress and causes poor memory or epilepsy attacks. Typical cases have a distinctive adult-onset, frequent, brief dystonic…

Autoimmune and Paraneoplastic Disorders.

Autoimmune encephalitis (AE) is a disorder in which the immune system targets the central nervous system. AE can be paraneoplastic or postinfectious, though many cases lack a clear trigger. AE typically presents acutely or subacutely with altered mental status and psychiatric symptoms, with clinical features varying by the targeted autoantigen. MR imaging is the imaging modality of choice and may show T2 and fluid attenuation inversion recovery (FLAIR) hyperintensities in the medial temporal lobes, characteristic of limbic encephalitis, though imaging can be normal or may extend beyond the lim…

Uncommon Bilateral Frontoparietal Cortex Hypermetabolism Revealed by 18 F-FDG PET/CT in a Patient With Anti-LGI1 Encephalitis.

Limbic hypermetabolism is a frequently observed manifestation of anti-LGI1 encephalitis on 18 F-FDG PET/CT imaging. In this case report, we present an atypical finding on 18 F-FDG PET/CT in a patient with anti-LGI1 encephalitis and negative MRI results. Apart from the expected hypermetabolism in the bilateral limbic system, there was also an uncommon intense uptake of 18 F-FDG noted in the bilateral frontoparietal cortex. Subsequent follow-up scans demonstrated normalized metabolism following treatment. The case highlights that neocortical hypermetabolism can be a characteristic feature of act

Долгосрочные результаты в аутоиммунном энцефалите: общенациональное, клиническое исследование в Дании.

Аутоиммунный энцефалит является редким заболеванием, связанным со значительной заболеваемостью в острой фазе заболевания. Общенациональные когортные исследования, посвященные изучению долгосрочных когнитивных результатов, встречаются редко. Кроме того, хотя когнитивные оценки на уровне кровати часто используются в клинической практике для оценки пациентов с ПВ, в настоящее время не существует консенсуса в отношении оптимального теста для этой цели. С помощью уникальной датской централизованной системы тестирования антител мы определили неотобранную общенациональную группу AE. Затем мы провели

Совпадение в аннотации оригинала
BACKGROUND AND OBJECTIVES: Autoimmune encephalitis is a rare disease associated with considerable morbidity in the acute phase of the diseas … anti-N-methyl-D-Aspartate receptor (NMDAR) encephalitis (NMDARE) (n = 33) and 86% of patients with limbic encephalitis (n = 37) obtained modified Rankin Scale (mRS) 0-2 at the time of testing, while 45% of patients with NMDARE and 43% of limbic encephalitis had multidomain cognitive deficits on formal neuropsychological testing. Memory, visuoconstruction, and execut …

Autoimmune encephalitis: what the radiologist needs to know.

Autoimmune encephalitis is a relatively novel nosological entity characterized by an immune-mediated damage of the central nervous system. While originally described as a paraneoplastic inflammatory phenomenon affecting limbic structures, numerous instances of non-paraneoplastic pathogenesis, as well as extra-limbic involvement, have been characterized. Given the wide spectrum of insidious clinical presentations ranging from cognitive impairment to psychiatric symptoms or seizures, it is crucial to raise awareness about this disease category. In fact, an early diagnosis can be dramatically ben

The neurobiology and immunology of CASPR2-associated neurological disorders.

CASPR2-associated neurological disorders encompass a wide clinical spectrum broadly divided into overlapping three autoimmune syndromes: CASPR2 limbic encephalitis, Morvan syndrome, and Isaacs syndrome. CASPR2 is a neuronal protein expressed at different sites in the central and peripheral nervous system and has a variety of roles and functions regarding neuronal excitability, synaptic plasticity, and homeostasis of inhibitory networks, most of which are only partially understood. CASPR2 antibodies have various pathogenic effects including internalization of CASPR2, disruption of protein-prote…

Encephalopathy with upper body hypertonia and myoclonus in patient with systemic lupus erythematosus and anti-CASPR2.

Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET).Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorde…

Onset of narcolepsy type 1 in a paraneoplastic encephalitis associated with a thymic seminoma.

UNLABELLED: Narcolepsy type 1 results from probable autoimmune disruption of hypothalamic hypocretinergic neurons. Secondary narcolepsy can occur as a result of other conditions affecting the central nervous system, including limbic paraneoplastic encephalitis. We report the case of a 19-year-old patient presenting with acute-onset diurnal hypersomnolence, hyperphagia, sexual dysfunction, and psychiatric disturbances. Further investigations revealed a limbic paraneoplastic encephalitis associated with mediastinal thymic seminoma. Tumor removal and immunosuppressive treatment resulted in a part

Cytomegalovirus ventriculoencephalitis in a reduced- intensity conditioning cord blood transplant recipient.

Cytomegalovirus (CMV) encephalitis most commonly occurs in patients with advanced human immunodeficiency virus infection and profound CD4 cell depletion and is rare in transplant recipients. We describe a patient with pathologically proven CMV ventriculoencephalitis that occurred after human herpesvirus-6 limbic encephalitis, following reduced-intensity conditioning cord blood transplantation (CBT). At approximately day 150 after CBT, the patient became acutely confused after steroid therapy for grade III acute graft-versus-host disease. Fluid-attenuated inversion recovery magnetic resonance i…

Elevated expression of extracellular matrix metalloproteinase inducer (CD147) and membrane-type matrix metalloproteinases in venous leg ulcers.

BACKGROUND: Autoimmune limbic encephalitis (ALE) is a rare inflammatory disorder characterised by a subacute onset, usually within weeks. The presence of multiple neuropsychiatric symptoms such as seizures, short-term memory deficits, anxiety and depression often leads to misdiagnosis as another medical condition, contributing to poor prognosis and reduced long-term survival. CASE DESCRIPTION: A 60-year-old man, with no chronic illnesses, presented at the emergency department with daily episodes of palpitations, shivering, piloerection and a sense of impending doom lasting two months. Initial…