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Incidentally Detected Subclinical Limbic Encephalitis on 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Patient with Myasthenia Gravis and Sarcoidosis: A Rare Immunological Triad?

Limbic encephalitis is the inflammation of the medial temporal lobe structures due to direct infectious or indirect autoimmune etiology. Sarcoidosis is a rare cause of limbic encephalitis, has been reported by some authors. Association has also been reported between sarcoidosis, myasthenia gravis, and limbic encephalitis. We report an interesting case of 18F-fluorodeoxyglucose positron emission tomography/computed tomography demonstrating limbic encephalitis and sarcoidosis in a known case of myasthenia gravis.

Should the Traditional Algorithm for Syphilis Screening be Abandoned? Neurosyphilis Presenting as Limbic Encephalitis: A Prozone False-Negative RPR Missed by an RPR-Only Initial Screen.

INTRODUCTION: Neurosyphilis is a treatable mimic of viral and autoimmune limbic encephalitis. The prozone phenomenon-antibody excess preventing the antigen-antibody lattice-can render an undiluted non-treponemal test such as the rapid plasma reagin (RPR) test falsely negative, with reactivity restored on dilution. CASE DESCRIPTION: A 37-year-old man presented with seizures and rapidly progressive limbic encephalitis; Brain magnetic resonance imaging (MRI) with fluid-attenuated inversion recovery sequences showed hyperintensities in the amygdalae, periventricular regions, left insula and left …

[Paraneoplastic anti-gamma aminobutyric acid (GABA)B receptor antibody limbic encephalitis associated with small cell lung cancer presenting as new-onset status epilepticus: a case report].

An 84-year-old man was admitted to our hospital with new-onset refractory status epilepticus of unclear etiology. On the third day, diffusion-weighted brain MRI demonstrated lesions in the right medial temporal and parietal lobes. As a CSF sample showed pleocytosis, paraneoplastic limbic encephalitis (PLE) associated with small cell lung cancer (SCLC) was suspected. The patient was also positive for anti-gamma aminobutyric acid (GABA)B receptor antibody in the CSF, which has recently been reported in elderly patients with SCLC-related PLE. Methylprednisolone pulse therapy ameliorated the sympt…

Loss of Autonoetic Awareness of Recent Autobiographical Episodes and Accelerated Long-Term Forgetting in a Patient with Previously Unrecognized Glutamic Acid Decarboxylase Antibody Related Limbic Encephalitis.

We describe a 35-year-old male patient presenting with depressed mood and emotional instability, who complained about severe anterograde and … ase antibodies. These findings led to the diagnosis of glutamic acid decarboxylase antibody related limbic encephalitis. Monthly steroid pulses over 6 months led to recovery of subjective memory and to intermediate improvement but subsequent worsening of objective memory performance. During the course of treatment, the patient reported de novo paroxysmal non-responsi …

Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia.

Antibodies that immunoprecipitate (125)I-alpha-dendrotoxin-labelled voltage-gated potassium channels extracted from mammalian brain tissue have been identified in patients with neuromyotonia, Morvan's 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 potassiu…

Managing paraneoplastic GABABR-related limbic encephalitis in a patient with a small cell lung cancer receiving an Immune-Checkpoint Inhibitor (ICI) therapy: description of a case report.

INTRODUCTION: GABAB receptor (GABABR)-related paraneoplastic limbic encephalitis (PLE) is frequently associated with cancer, particularly small cell lung carcinoma (SCLC) and shows several clinical presentations often accompanied by distinctive MRI and EEG findings. It can be exacerbated by immune-modulating therapies, such as immune checkpoint inhibitors (ICIs) and needs a multidisciplinary approach. CASE REPORT: This case report describes a 67-year-old male with a diagnosis of SCLC who developed a paraneoplastic LE. The patient's clinical presentation included amnesia, apraxia, and mesial t…

Coexistence of anti-SOX1 and anti-GABAB receptor antibodies with paraneoplastic limbic encephalitis presenting with seizures and memory impairment in small cell lung cancer: A case report.

PURPOSE: Paraneoplastic neurological syndromes associated with autoantibodies are rare diseases that cause abnormal manifestations of the nervous system. Early diagnosis of paraneoplastic neurological syndromes paves the way for prompt and efficient therapy. CASE REPORT: we reported a 56-year-old man presenting with seizures and rapidly progressive cognitive impairment diagnosed as paraneoplastic limbic encephalitis (PLE) with anti-SRY-like high-mobility group box-1 (SOX-1) and anti-γ-aminobutyric acid B (GABAB) receptor antibodies and finally confirmed by biopsy as small cell lung cancer (SC…

Лимбический энцефалит у пациента с системным волшебным эритематозом успешно лечился высокодозированными глюкокортикоидами и внутривенной циклофосхамидной терапией: потенциальная патогенность антилютаматных рецепторов.

Лимбический энцефалит (LE) — клинически определенный синдром, характеризующийся острым или субакустическим расстройством краткосрочной памяти, припадками и психическими симптомами (т.е. депрессией, тревогой и галлюцинацией). LE может появиться в определенных условиях, когда нейропсихиатрическая системная волчанка эритематоза (NPSLE) мультицентральной нервной системы сломана. В этом докладе мы описываем 46-летнюю японскую женщину с SLE, которая неожиданно попала с припадками, сенсорной афазией и псевдобулкой. У нее был диагностирован сильный ЯИЭ, содержащий клинический LE (LE-SLE), за исключени

Совпадение в аннотации оригинала
Limbic encephalitis (LE) is a clinically defined syndrome characterised by an acute or subacute impairment of short-term memory, seizures and psychiatric symptoms (i.e. depression, anxiety and hallucination). LE could come from certain conditions where the neuropsychiatric systemic lupus erythematosus (NPSLE) of the multiple central nervous system is layered…

Anti-voltage-gated potassium channel complex antibody-mediated limbic encephalitis: a case report of a 53-year-old man admitted to intensive care psychiatric unit with psychotic mania.

Limbic encephalitis represents a cluster of autoimmune disorders, with inflammation in the medial temporal lobe characterised by subacute onset of neuropsychiatric symptoms such as anxiety, affective symptoms, psychosis, short-term memory impairment as well as faciobrachial and grand mal seizures. We aim to present a case of a 53-year-old man with positive anti-voltage-gated potassium channel (VGKC) complex antibodies who initially presented with symptoms of psychotic mania. Six weeks post-psychiatric symptomatology, he presented with neurological symptoms such as faciobrachial jerking and ton…

Severe hyponatremia caused by syndrome of inappropriate secretion of antidiuretic hormone developed as initial manifestation of human herpesvirus-6-associated acute limbic encephalitis after unrelated bone marrow transplantation.

Severe hyponatremia is a critical electrolyte abnormality in allogeneic stem cell transplantation (allo-SCT) recipients and >50% of cases of severe hyponatremia are caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Here, we present a patient with rapidly progressive severe hyponatremia as an initial sign and symptom of human herpesvirus-6-associated post-transplantation acute limbic encephalitis (HHV-6 PALE) after allo-SCT. A 45-year-old woman with acute lymphoblastic leukemia received unrelated bone marrow transplantation from a one locus-mismatched donor at t…

Cholangiocarcinoma associated with limbic encephalitis and early cerebral abnormalities detected by 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography-positron emission tomography: a case report.

BACKGROUND: Limbic encephalitis was originally described as a rare clinical neuropathological entity involving seizures and neuropsychological disturbances. In this report, we describe cerebral patterns visualized by positron emission tomography in a patient with limbic encephalitis and cholangiocarcinoma. To our knowledge, there is no other description in the literature of cerebral positron emission tomography findings in the setting of limbic encephalitis and subsequent diagnosis of cholangiocarcinoma. CASE PRESENTATION: We describe a case of a 77-year-old Caucasian man who exhibited persis…

Creutzfeldt-Jakob disease versus anti-LGI1 limbic encephalitis in a patient with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal electroencephalogram: a case report.

Diagnosis of Creutzfeldt-Jakob disease (CJD) is often challenging in elderly individuals, not only because of its variable clinical features but also because of nonspecific changes on the electroencephalogram (EEG) in the early stages of the disease. Here we report on a patient who presented with progressive cognitive dysfunction, psychiatric symptoms, involuntary facio-brachio-crural movement, and an abnormal EEG. We provide a detailed analysis and differential diagnosis between anti-leucine-rich glioma inactivated 1 (LGI1) limbic encephalitis versus CJD, in the hope of providing a new unders…

An Uncommon Presentation of a Primary Bone Tumor: Anti-AMPA (Anti-α-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic acid) Receptor Limbic/Paraneoplastic Encephalitis as a Presenting Feature of Ewing Sarcoma.

Anti-AMPA (anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor limbic encephalitis is a rare autoimmune syndrome. It can be associated with an underlying malignancy, such as lung, breast, or thymus. We are reporting a case of a 19-year-old patient who presented with a 2-week history of confusion and short-term memory loss. A magnetic resonance imaging of brain showed increased T2 hyperintensity within the hippocampi bilaterally. Cerebrospinal fluid analysis was positive for anti-AMPA receptor antibodies. A computed tomography revealed multiple pulmonary metastases as well as an…

[Anti-voltage-gated potassium channel (VGKC) complex/leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis mimicking dementia with Lewy bodies in a patient with subacute REM sleep behavior disorder].

A 73-year-old woman was admitted to our hospital owing to abnormal diurnal behavior, sudden brief episodes of impaired awareness, and loud nocturnal sleep talking. Her symptoms had developed gradually over several months and had been treated as dementia with Lewy bodies (DLB) at another clinic. Video-polysomnography revealed brief sleep talking and gross movements associated with REM sleep without atonia. 18F-FDG PET revealed increased glucose metabolism in both medial temporal lobes. These findings led to a diagnosis of limbic encephalitis (LE) comorbid with REM sleep behavior disorder (RBD).…

Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: a case series.

Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients including stiff person syndrome, cerebellar ataxia, refractory epilepsy, limbic and extralimbic encephalitis. GAD antibodies-related limbic encephalitis cases are well described; reports of extralimbic involvement are limited. We describe four cases of GAD antibody-related autoimmune encephalitis. Three of them had extralimbic involvement and only one had limbic encephalitis.

Survey of herpes simplex virus infections of the central nervous system, including acute disseminated encephalomyelitis, in the Kyushu and Okinawa regions of Japan.

We analysed data from 27 patients with herpes simplex virus (HSV) infections of the central nervous system (CNS) found in a 1990-1992 survey in Kyushu and Okinawa, Japan. Patients ranged in age from one year to 70 years, with peaks seen in the 20s and 50s. Temporal lobe-limbic encephalitis was the most common HSV infection (13 patients), followed by meningitis (5), diffuse encephalitis (4), disseminated encephalomyelitis (ADEM) (3) and brain stem encephalitis (2). Another three patients with non-herpetic, non-paraneoplastic acute limbic encephalitis were presented. Our study indicates that HSV…

Neuropathology of the limbic system.

The history and development of the fanciful terminology concerning the structures of the limbic system are discussed. The diseases involving the limbic system are divided into three groups; (1) diseases in which the limbic system is more or less selectively involved, such as limbic encephalitis, herpes simplex encephalitis, cerebral confusions by the falx and tentorium, and internal herniations through the falx and tentorium; (2) diseases in which the limbic system is predominantly involved, such as arhinencephalia, holoprosencephaly, cyst of cavum septi pellucidi, Pick's disease, Alzheimer's …

Uncommon epiloptogenic lesions affecting the temporal lobe.

There are several processes implicated as uncommon causes of temporal lobe epilepsy. Trauma is the leading cause of epilepsy in young adults … racteristics of the affectation associated with high risk of early and late seizures. Noninfectious limbic encephalitis is a syndrome characterized by seizures, memory loss, and confusion. It includes paraneoplasic and non-paraneoplasic limbic encephalitis, both presenting as hyperintense lesion affecting temporobasal regions more evident with fluid-attenuated invers …

Anti-LGI1 Encephalitis Developing Immunoglobulin Responsive Orthostatic Hypotension after Remission.

Anti-leucine-rich glioma-inactivated 1 (LGI1) antibody is associated with limbic encephalitis. We herein report a patient with anti-LGI1 encephalitis who developed severe orthostatic hypotension (OH) responsive to immunoglobulin therapy five years after developing symptoms of encephalitis. A 71-year-old man presented with amnesia caused by limbic encephalitis. The symptoms of encephalitis improved partially without any immunotherapy. Five years later, he developed severe OH, and anti-LGI1 antibody was positive. The catecholamine dynamics indicated that the central autonomic nervous system was …