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[A case of anti-Hu antibody- and anti-GluR epsilon2 antibody-positive paraneoplastic neurological syndrome presenting with limbic encephalitis and peripheral neuropathy].

We report a case of paraneoplastic neurological syndrome with anti-neuronal antibodies, namely anti-Hu and anti-GluR epsilon 2 antibodies in … i-Hu and anti-GluR epsilon 2 antibodies were detected in sera but not in CSF. We diagnosed him with limbic encephalitis and peripheral neuropathy due to paraneoplastic neurological syndrome and treated him with two courses of intravenous immunoglobulin (IVIg) (400 mg/kg, 5 days). The consciousness disturbance, and prolonged distal latency revealed by motor nerve cond …

Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report.

Autoantibodies to leucine-rich glioma-inactivated protein 1 (LGI-1) are associated with inflammation of the limbic system. Faciobrachial dystonic seizures are pathognomonic for LGI1-antibiodies and their treatment with immunotherapy is effective in seizure control with a potential to prevent cognitive decline. We report a 57-year-old man who presented to the emergency department with recurrent seizures, visual hallucinations and severe memory impairment over a seven-week period; he reported a background of alcohol excess. Initial investigations revealed hyponatremia, indicating syndrome of ina

Pre- and long-term postoperative courses of hippocampus-associated memory impairment in epilepsy patients with antibody-associated limbic encephalitis and selective amygdalohippocampectomy.

OBJECTIVE: Limbic encephalitis (LE) is defined by mesiotemporal lobe structure abnormalities, seizures, memory, and psychiatric disturbances. This study aimed to identify the long-term clinical and neuropsychological outcome of selective amygdalohippocampectomy (sAH) in drug-resistant patients with temporal lobe epilepsy due to known or later diagnosed subacute LE not responding to immunotherapy associated with neuronal autoantibodies. METHODS: In seven patients with temporal lobe epilepsy due to antibody positive LE (glutamic acid decarboxylase (GAD65): n=5; voltage-gated potassium channel c…

Синдром Вундерлиха, вызванный эноксапарином, у молодого пациента с оксоклоном и лимбическим энцефалитом, связанным с антиGAD 65: редкое осложнение в редкой болезни.

Синдром Ундерлиха является редким заболеванием, характеризующимся острым нетравматическим кровоизлиянием в подкапсулальные и перриналальные пространства. Наш случай аутоиммунного энцефалита (AE) в возрасте 30 лет, связанного с антиGAD65, создал это осложнение после использования эноксапарина и управлялся избирательной эмболизацией субсегментальных ветвей правых корковых артерий клеем. В нашем случае опоколон был одним из клинических проявлений, которые до сих пор были описаны только у двух пациентов этого AE. Пациентка получила все виды индуктивной терапии (стероиды, плазмоферез, иммуноглобули

Characterization of a Subtype of Autoimmune Encephalitis With Anti-Contactin-Associated Protein-like 2 Antibodies in the Cerebrospinal Fluid, Prominent Limbic Symptoms, and Seizures.

IMPORTANCE: Autoantibodies against contactin-associated protein-like 2 (CASPR2) are observed in several neurological syndromes, including neuromyotonia (NMT), Morvan syndrome (MoS), and limbic encephalitis. OBJECTIVE: To characterize the clinical and biological presentations of patients with anti-CASPR2 antibodies in the cerebrospinal fluid (CSF). DESIGN, SETTING, AND PARTICIPANTS: We conducted a retrospective cohort analysis of 18 patients who had anti-CASPR2 antibodies in their CSF between March 2009 and November 2015 at the Centre National de Référence pour les Syndromes Neurologiques Par…

[An autopsied case of paraneoplastic neurologic syndrome (limbic encephalitis, cerebellar degeneration, and pseudohypertrophy in the inferior olivary nuclei) associated with T cell lymphoma].

We describe a patient with paraneoplastic neurologic syndrome confirmed on postmortem examination. This 42-year-old man was admitted due to … system or lymphatic organs. Based on the pathological findings, paraneoplastic neurologic syndrome (limbic encephalitis, cerebellar degeneration and olivary pseudohypertrophy) associated with T-cell type malignant lymphoma was diagnosed. Only three other cases of paraneoplastic neurologic syndrome associated with T-cell lymphoma have been reported. In those cases, de …

Микроглиальный метаморфоз в трёх измерениях в вирусном лимбическом энцефалите: небиозное пикториальное представительство, основанное на стереологическом подходе к отбору проб выживающих и реактивных микроглий.

Микроглия влияет на патологическое развитие неврологических заболеваний, реагируя на оскорбления, выражая множественные морфофункциональные фенотипы. Однако полный морфологический спектр реактивной микроглии, обнаруженный трехмерной микроскопической реконструкцией, не был подробно описан в вирусном лимбическом энцефалите. Здесь, используя анатомическую серию мозговых секций, мы расширили ранее проведенное исследование энцефалита Piry arbovirus, включив в него CA1/CA2 и провели оценку морфологической реакции гомеостатического и реактивного микроглии в течение восьми дней после заражения. Иерарх

Совпадение в аннотации оригинала
Microglia influence pathological progression in neurological diseases, reacting to insults by expressing multiple morphofunctional phenotypes. However, the complete morphological spectrum of reactive microglia, as revealed by three-dimensional microscopic reconstruction, has not been detailed in virus limbic encephalitis. Here, using an anatomical series of …

Thymoma-associated Myasthenia Gravis in a Young Adult with Development of Paraneoplastic Limbic Encephalitis and Systemic Lupus Erythematosus Post-thymectomy: A Case Report.

We report a young adult with thymoma-associated myasthenia gravis (MG) who, following thymectomy, developed paraneoplastic limbic encephalitis (LE) and systemic lupus erythematosus (SLE). Although thymomas commonly co-occur with MG, LE is an uncommon autoimmune sequela. Herein, we discuss the pathophysiology of paraneoplastic LE and its management. Our report also highlights an unusual case of a thymoma patient who presented with multiple autoimmune disorders. The treatment of such a patient is therefore challenging and requires care from multiple specialized teams.

Autoimmune Limbic Encephalitis With Leucine-Rich Glioma-Inactivated 1 (LGI1) Antibodies Presenting as Rapidly Progressive Cognitive Decline and Psychiatric Symptoms: A Case Report.

Autoimmune limbic encephalitis (LE) with leucine-rich glioma-inactivated 1 (LGI1) antibodies is rare but treatable, often presenting as rapidly progressive cognitive decline, behavioural changes, and hyponatraemia. These features can resemble psychiatric or neurodegenerative disorders, delaying diagnosis. We report a case of a 63-year-old man with confusion, memory loss, dream enactment, hallucinations, severe hyponatraemia, and specific higher cortical features, including apraxia, anomia, and executive dysfunction. Faciobrachial dystonic seizures (FBDS), a recognised hallmark of LGI1 antibody…

Cerebrospinal Fluid Anti-Neuronal Autoantibodies in COVID-19-Associated Limbic Encephalitis with Acute Cerebellar Ataxia and Myoclonus Syndrome: Case Report and Literature Review.

Since the outbreak of coronavirus (COVID-19) in 2019, various rare movement disorders and cognitive changes have been recognized as potentia … nsive care unit. A diagnosis of COVID-19-associated autoimmune encephalitis with characteristics of limbic encephalitis and immune-mediated acute cerebellar ataxia and myoclonus syndrome was supported by alterations in the limbic system shown in magnetic resonance imaging, lateralized discharges shown in electroencephalography, a slightly elevated protein level in th …

Executive deficits in neuropsychological testing differentiate between autoimmune temporal lobe epilepsy caused by limbic encephalitis and temporal lobe epilepsies with non-autoimmune etiologies.

OBJECTIVE: Patients with temporal lobe epilepsy caused by autoimmune limbic encephalitis (AI-TLE) clinically resemble patients with temporal lobe epilepsy with non-autoimmune etiologies (NAI-TLE) but have a different prognosis and require specific adjusted therapies. The objective of this study was to investigate whether patients with these forms of TLE can be discerned by means of neuropsychological assessment. METHODS: Data from 103 patients with TLE (n = 39 with AI-TLE and n = 64 with NAI-TLE, including n = 39 with hippocampal sclerosis [HS] and n = 25 with low-grade epilepsy-associated tu…

Risk Factor and Long-Term Outcome Analyses for Acute Limbic Encephalitis and Calcineurin Inhibitor-Induced Encephalopathy in Adults following Allogeneic Hematopoietic Cell Transplantation.

Post-transplantation acute limbic encephalitis (PALE) is a rare, severe inflammatory disorder in the bilateral limbic system, including the hippocampus. To date, only a few studies have reported details, including risk factors for PALE; however, further clinical evidence of PALE, especially in cerebrospinal fluid human herpesvirus 6-negative cases, is warranted. In addition, data are sparse regarding the risk factors for calcineurin inhibitor (CNI)-induced encephalopathy (CNIE) following allogeneic hematopoietic cell transplantation (allo-HCT) in adults. Therefore, we examined the risk factors…

Case Report/Case Series: Rare case of anti-LGI1 limbic encephalitis with rapidly progressive dementia, psychiatric symptoms, and frequently seizures: A case report.

RATIONALE: Anti leucine-rich glioma inactivated 1 (LGI1) limbic encephalitis (LE) is rare autoimmune encephalitis, characterized by acute or subacute cognitive impairment, faciobrachial dystonic seizures, mental disorders, and refractory hyponatremia. As a type of treatable rapidly progressive dementia with a good prognosis, early, and accurate diagnosis is essential. We present a case of anti-LGI1 LE who was initially misdiagnosed with Alzheimer disease because his clinical manifestations were similar to Alzheimer disease. PATIENT CONCERNS: A male patient presenting with rapidly progressive …

Limbic encephalitis associated with relapsing polychondritis responded to infliximab and maintained its condition without recurrence after discontinuation: a case report and review of the literature.

Central nervous system (CNS) manifestations are rare complications of relapsing polychondritis (RP). The majority of patients respond well to glucocorticoid therapy, but need to maintain it. Some patients are refractory to initial glucocorticoid therapy and to additional immunosuppressants, and end up with an outcome worse than at therapy initiation. The standardized therapeutic protocol for this condition has not been established. The effects of anti-tumor necrosis factor (TNF) -α agents have been reported recently. We experienced a patient with RP and limbic encephalitis who was refractory t…

Neuropsychiatric lupus erythematosus with neurogenic pulmonary edema and anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor limbic encephalitis: a case report.

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disease predominantly found in women of child-bearing age. Neurogenic pulmonary edema (NPE) is a recalcitrant complication that occurs after injury to the central nervous system and has an acute onset and rapid progression. Limbic encephalitis is an inflammatory encephalopathy caused by viruses, immune responses, or other factors involving the limbic system. NPE caused by SLE is rare. CASE PRESENTATION: Here, we report a case of a 21-year-old woman with SLE who experienced five episodes of generali…

Fatal X-linked lymphoproliferative disease type 1-associated limbic encephalitis with positive anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibody.

BACKGROUND: X-linked lymphoproliferative disease type 1 (XLP1) is a rare monogenic immune dysregulation disorder caused by a deficiency of a signaling lymphocyte activation molecule-associated protein (SAP). While many patients with XLP1 present with fatal hemophagocytic lymphohistiocytosis upon Epstein Barr virus (EBV) infection, a small fraction present with limbic encephalitis in the absence of EBV infection. It is poorly understood why SAP deficiency may cause limbic encephalitis in XLP1. CASE: A 12-year-old boy presented with seizures, changes in personality, memory loss, and cognitive d…

Efficacy of rituximab on seizure control and cognitive symptoms in leucine-rich, glioma-inactivated 1 (LGI1) limbic encephalitis: a high-density electroencephalography case study.

LGI1 encephalitis is an autoimmune disorder characterized by cognitive symptoms and seizures, which rarely respond to common antiepileptic d … ty EEG could represent a novel, safe and reproducible method to study epileptogenesis in autoimmune limbic encephalitis.

A Patient with Limbic Encephalitis Associated with Anti-leucine-rich Glioma-inactivated 1 (LGI1) Antibody Presenting with Slowly Progressive Cognitive Impairment and Fluctuating Striatal Lesions.

We herein report the case of a 59-year-old man with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis who presented with slowly progressive cognitive impairment mimicking dementia for over 3 years and then developed seizures. Unique brain magnetic resonance imaging (MRI) findings of fluctuating striatal lesions were observed during the disease course. He was treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone, which dramatically improved his neurological function. Taken together, these findings indicate that anti-LGI1 encephalitis may prese