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Encephalitis in Kikuchi-Fujimoto disease being immune-mediated.

INTRODUCTION: Histiocytic necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a rare benign self-limiting inflammatory disease often seen in young adults. The main clinical features are fever with cervical lymphadenopathy. Neurological complications of Kikuchi-Fujimoto disease were occasionally reported although the specific pathogenesis was not clear. The condition could be severe when encephalitis coexists. METHODS: Here we reported a young case of Kikuchi-Fujimoto disease with subsequent severe autoimmune encephalitis. RESULTS: The symmetric striatal and limbic MRI lesio

The clinical approach to managing herpes simplex virus encephalitis.

This article explains the approach to managing a patient with herpes simplex virus encephalitis. Acute encephalopathy is a common and often … mportant because the disease is potentially treatable with immunosuppressive agents. Paraneoplastic limbic encephalitis may present months or years before the detection of a tumour.

Clinical features of 28 cases of anti-leucine-rich glioma-inactivated protein 1 encephalitis and anti-contactin-associated protein-like 2 encephalitis.

OBJECTIVES: Autoimmune encephalitis arising from autoantibodies against leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-assoc … e of 11 patients was 17-68 (47.18±16.20) years old, and the ratio of male to female was 5꞉6, with 7 limbic encephalitis, 1 Morvan syndrome, and 3 acquired neuromyotonia (NMT). Three patients had increased slow waves and 2 had epileptic discharge in EEG. Brain MRI showed T2WI and FLAIR hyperintense signal in the temporal lobe, hippocampus in 2 patients. Steroids, intr …

[Autoimmune Encephalitis/Meningitis].

Less than half of the cases of autoimmune encephalitis have brain MRI abnormalities; however, some patterns of MRI findings help diagnosis. Usually, DWI and FLAIR images reveal hyperintensity lesions in the cortical or subcortical regions or the cerebellum and/or the brainstem. Hyperintensity lesions in the limbic cortex on DWI suggest NMDAR encephalitis. RA or polychondritis-related meningitis show bright dot or linear signals on the convexities on DWI. Area postrema syndrome is a typical form of neuromyelitis optica. These conditions need to be diagnosed promptly for effective treatment.

Autoimmune epilepsy in children: case series and proposed guidelines for identification.

PURPOSE: Antibodies against neuronal surface proteins are increasingly recognized in autoimmune central nervous system (CNS) disorders in which seizures are the main or an important feature. The disorders include antibody-associated limbic encephalitis and N-methyl-D-aspartate receptor (NMDAR) encephalitis; however, seizures of autoimmune etiology may exist beyond the spectrum of these recognized syndromes. Because these seizures are potentially treatable with immune therapy, guidelines are needed to help in their early recognition. METHODS: We describe 13 representative children seen at our …

18F-FDG PET/CT in Anti-Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis: Typical Pattern and Follow-up.

We report the case of a 55-year-old man presenting pseudopsychiatric behavior disorders of subacute-onset. MRI showed a FLAIR (fluid-attenuated inversion recovery) hyperintensity in the left hippocampus. The diagnosis of limbic encephalitis was raised, and the patient was referred for an 18F-FDG PET/CT. PET/CT depicted an increased uptake of the left mesiotemporal structures and also an increased uptake of both cerebellum and striatal areas. This pattern was compatible with an anti-leucine-rich glioma-inactivated 1 antibody encephalitis that was later confirmed.

Differential diagnosis of temporal lobe lesions with hyperintense signal on T2-weighted and FLAIR sequences: pictorial essay.

Various neuropathologies produce hyperintense signals on T2-weighted or fluid-attenuated inversion recovery sequences of the temporal lobes. Recognition of the distribution pattern and associated findings may narrow the spectrum of differential diagnoses or suggest a specific disease. This pictorial essay aims to illustrate the relatively common diseases that affect the temporal lobe, such as herpes simplex encephalitis, neurosyphilis, limbic encephalitis, postictal edema, neoplasia, and multiple sclerosis, as well as those that are less common, such as myotonic dystrophy type 1, CADASIL, and …

Limbic system symptoms of rabies infection.

Rabies is a fatal aggressive disease of the nervous system which predominantly causes motor and autonomic dysfunction. Limbic system involvement has been reported rarely, with limited data on its prevalence. The diagnosis becomes challenging when a patient presents with limbic system involvement in the absence of a clear history of an animal bite. We herein illustrate a case of a young man who presented with recurrent episodes of inappropriate ejaculation. He eventually developed hydrophobia and aerophobia, leading to a diagnosis of rabies. This case emphasises the importance of considering th

Case Report: Guillain-Barré Syndrome Characterized by Severe Headache Associated With Metabotropic Glutamate Receptor 5 Antibody.

Autoantibodies to metabotropic glutamate receptor 5 (mGluR5) are known to be the cause of autoimmune encephalitis, particularly limbic encephalitis, closely related to Hodgkin's lymphoma (HL). The involvement of peripheral neuropathy is rarely reported. In our case, mGluR5 antibody was found in a Guillain-Barré syndrome (GBS) patient accompanied by severe headache but without neuropsychiatric manifestations or HL. Presenting with severe headache, the patient developed progressive bilateral limb weakness, areflexia, and cranial nerve involvement consisting of eye movement disorder, restricted m…

Case Studies Illustrating Focal Alzheimer's, Fluent Aphasia, Late-Onset Memory Loss, and Rapid Dementia.

Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis). Recognizing the different syndromes can help the clinician to i…

Выявление аденилатной киназы 5 антител во время обычной диагностики в тканевом анализе: три новых случая и обзор литературы.

Антитела против аденилатной киназы 5 (АК5) были описаны у пациентов с непаранопластическим лимбовым энцефалитом, главным образом у мужчин в возрасте около 70 лет. Обычные испытания с использованием конкретных клеточных тестов пока отсутствуют. У трёх пациентов с эпизотическими проблемами передней памяти и депрессией были значительные лимфатические повреждения и развилась сильная атрофия, главным образом из медиальных височных долей. Антитела идентифицировались в сыворотке и CSF на основе типичной модели АК5 антител на основе тканевого анализа (здесь, нефиксированный мозг мыши). Впоследствии он

Совпадение в аннотации оригинала
Antibodies against adenylate kinase 5 (AK5) have been described in patients with non-paraneoplastic limbic encephalitis, mainly in men around 70 years of age. Routine testing with specific cell-based assays is not yet available. Three patients with episodic anterograde memory problems and depression had extensive limbic lesions and developed severe atrophy, …

Neurologic Presentation of Probable Seronegative Paraneoplastic Encephalitis in a Woman With an Ovarian Teratoma.

We describe a case of probable autoimmune encephalitis developed as a result of paraneoplastic syndrome in a woman with an ovarian teratoma. Patients may present with psychiatric and neurological symptoms, which are caused by anti-N-methyl-D-aspartate (anti-NMDA) receptor antibodies produced in response to a teratoma that crosses the blood-brain barrier and damages brain tissue in the limbic area, causing encephalitis. Our patient presented with seizures, myoclonus, and memory problems. This is a relatively newly discovered and rare problem; however, it can be quite debilitating if left untrea

Caspr2 antibodies in patients with thymomas.

Myasthenia gravis is the best known autoimmune disease associated with thymomas, but other conditions can be found in patients with thymic tumors, including some that affect the central nervous system (CNS). We have become particularly interested in patients who have acquired neuromyotonia, the rare Morvan disease, or limbic encephalitis. Neuromyotonia mainly involves the peripheral nerves, Morvan disease affects both the peripheral nervous system and CNS, and limbic encephalitis is specific to the CNS. Many of these patients have voltage-gated potassium channel autoantibodies. All three condi…

Diagnosing autoimmune encephalitis in a real-world single-centre setting.

BACKGROUND: Early recognition and treatment of autoimmune encephalitis (AE) are crucial for patients, but diagnosis is often difficult and time-consuming. For this purpose, a syndrome-based diagnostic approach was published by Graus et al. (Lancet Neurol 15:391-404, 2016), but very little is known in the literature about its application in clinical practice. AIM: Our aims are to test the feasibility of such approach in a real-world single-centre setting and to analyse the most relevant factors in criteria fulfilment. METHODS: We retrospectively applied these criteria to our cohort of patient

Autoimmune neuromyotonia.

PURPOSE OF REVIEW: Autoimmune neuromyotonia encompasses a group of rare immune-mediated neurological disorders frequently associated with an … ntibodies, including cramp-fasciculation syndrome, Isaacs syndrome, Morvan syndrome, and autoimmune limbic encephalitis. Diagnosis relies on the identification of motor, sensory, and autonomic signs of PNH along with other neurological symptoms, anti-CASPR2 antibody-positivity, and of characteristic electroneuromyographic abnormalities. Paraneoplastic associations wi …

Autoimmune Encephalitis: Pathophysiology and Imaging Review of an Overlooked Diagnosis.

Autoimmune encephalitis is a relatively new category of immune-mediated disease involving the central nervous system that demonstrates a widely variable spectrum of clinical presentations, ranging from the relatively mild or insidious onset of cognitive impairment to more complex forms of encephalopathy with refractory seizure. Due to its diverse clinical features, which can mimic a variety of other pathologic processes, autoimmune encephalitis presents a diagnostic challenge to clinicians. Imaging findings in patients with these disorders can also be quite variable, but recognizing characteri

Subsequent Bilateral Hippocampal Diffusion Restriction and Atrophy in Repeated Status Epilepticus.

BACKGROUND: Cortical lesions in status epilepticus have been reported but the underlying mechanisms are poorly elucidated. CASE SUMMARY: We report on afemale patient (75 years) with a history of alcohol abuse who presented with complex partial status epilepticus and lateralized epileptiform discharges in the left frontal and temporal regions in EEG. While cranial magnetic resonance imaging (MRI) showed left hippocampal T2-hyperintensity and diffusion restriction, cerebrospinal fluid was normal and revealed no limbic encephalitis-related antibodies. Following treatment with levitiracetam, seiz…

[Successful use of methylprednisolone therapy in a case of non-herpetic acute encephalitis with antibodies against ionotropic glutamate receptor epsilon2 and delta2].

This report concerns a 32-year old man with non-herpetic acute limbic encephalitis. He was admitted to our hospital because of high fever and consciousness disturbance. Cranial MRI revealed abnormal signal intensities in the bilateral mesial temporal lobes. On the second hospitalization day, he developed status epilepticus, which necessitated general anesthesia. Following administration of intravenous (IV) methylprednisolone pulse therapy (1 g/day, 3 days), his consciousness disturbance began to improve. Treatment with high-dose IV methylprednisolone was continued for about 2 weeks, but on the…