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Anti-Ma-associated encephalitis due to dysgerminoma in a woman with Swyer syndrome.

Paraneoplastic limbic encephalitis (PLE)/diencephalitis associated with anti-Ma2 antibodies was linked to testicular cancer and non-small-cell lung cancer (non-SCLC).(1,2) We report a case of anti-Ma-associated PLE/diencephalitis due to dysgerminoma in a woman with gonadal dysgenesis, or Swyer syndrome.

[Anti-Ma2 paraneoplastic autoimmune encephalitis].

Paraneoplastic syndromes are a heterogeneous group of conditions affecting cancer patients, where the symptoms are not owing to the local effects of the tumor but instead owing to humoral or immunologic effects. Paraneoplastic neurological syndromes (PNS) develop in less than 1% of cancer patients and can affect any part of the nervous system. A variety of PNS phenotypes are associated with anti-Ma2 antibody, primarily encephalitis with a predominant involvement of brainstem, limbic and diencephalic structures. We describe a case of anti-Ma2 autoimmune encephalitis with a recurrent course in a

Acute viral encephalitis: the recent progress.

In the new Japanese control law for infectious diseases, most varieties of acute viral encephalitis belong to Category IV requiring report o … , and the data are reviewed here. Trends include an increase in a new subtype of non-herpetic acute limbic encephalitis. In contrast, the incidence of Japanese encephalitis (JE) in Japan has dramatically decreased to a few patients per year; however, JE remains a threat for those with decreased or absent immunity to the JE virus. Imported emerging and reemerging CNS …

Clinic-radiological classification of herpesviral encephalitis in humans (systematic review).

The development of a comprehensive classification for herpesvirus encephalitis remains an urgent task. Distinct clinic-radiological forms of herpesvirus cerebral lesions have been characterized, including findings from histopathological studies. Differences among these forms have been demonstrated concerning key clinical and paraclinical parameters. The presented classification identifies several distinct forms of herpesvirus encephalitis: temporal, brainstem, limbic, diencephalic encephalitis, rhombencephalitis, leukoencephalitis, ventriculoencephalitis, diffuse glial micronodular encephaliti

Prevalence and outcome of late-onset seizures due to autoimmune etiology: A prospective observational population-based cohort study.

OBJECTIVE: The increasing incidence of new-onset seizures with age is well known. Often, the etiology cannot be clarified. In the present st … n-associated protein-like 2) Abs in serum and CSF and fulfilled the diagnostic criteria of definite limbic encephalitis. Another two patients had bilateral encephalitic temporal MRI abnormalities. They also satisfied the criteria of definite limbic encephalitis, even though they had no Abs in serum or CSF. All four were in the age range of 55-70 years. They received …

Viral infections of the brain.

Brain infections are generally well visualized by contrast-enhanced computed tomography or magnetic resonance imaging. However, these modalities are often unrevealing in the encephalopathy produced by the human immunodeficiency virus and in the early stages of herpes simplex encephalitis. Several studies have documented the greater sensitivity of perfusion single-photon emission computed tomography (SPECT) in human immunodeficiency virus encephalopathy. In herpes simplex encephalitis, a few case reports have documented that SPECT may depict increased perfusion in the characteristically involve

Anti-AMPA-Receptor Encephalitis Presenting as a Rapid-Cycling Bipolar Disorder in a Young Woman with Turner Syndrome.

Background. Autoimmune encephalitis is a disorder characterised by the subacute onset of seizures, short-term memory loss, and psychiatric a … id. Conclusion. This case description emphasises the importance of timely recognition of autoimmune limbic encephalitis in patients with psychiatric manifestations and a possible predisposition to autoimmune conditions, in order to rule out malignancy and to quickly initiate treatment.

Efficacy of immunotherapy and prognosis in anti-LGI1 encephalitis patients: A meta-analysis.

OBJECTIVE: To assess the efficacy and safety of immunotherapy for LGI1 antibody encephalitis, and consider the predictors of poor outcomes following immunotherapy. METHODS: We searched PubMed and Embase for articles reporting the immunotherapy data of anti-LGI1 encephalitis patients. The proportions of patients with poor outcomes (modified Rankin Scale [mRS] score > 2) at 3 months, 12 months, and the last follow-up, as well as the odds ratio [OR] of predictors were pooled. RESULTS: The review included 162 articles with 1066 patients. The proportion of patients with poor functional outcomes w

When should we test patients with epilepsy for autoimmune antibodies? Results from a French retrospective single center study.

BACKGROUND AND PURPOSE: Seizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additional autoimmune antibodies serum and cerebrospinal fluid (CSF) detection. METHODS: In this retrospective single center study from the French Salpêtrière Hospital, data from 286 adult patients with epilepsy who received an autoantibody assay for th

Autoimmune encephalitis with CASPR2 antibody: A case series and updated literature review.

OBJECTIVE: Contactin-associated protein-like 2 (CASPR2) encephalitis is an under-recognized and relatively rare type of autoimmune encephalitis (AE). Here, we investigated the clinical manifestations and outcomes in patients with CASPR2 encephalitis. METHODS: The data of patients with CASPR2 encephalitis were collected retrospectively, and their clinical features were analyzed. Additionally, clinical features and prognoses of CASPR2 encephalitis in previously reported studies were summarized and analyzed. RESULTS: Fifteen patients (median age: 52 years; age range: 10-81 years, nine were male

PET-positive extralimbic presentation of anti-glutamic acid decarboxylase antibody-associated encephalitis.

Anti-glutamic acid decarboxylase (GAD) antibody-associated autoimmune encephalitis has been reported mostly as limbic encephalitis. Only few cases with extralimbic involvement are reported with limited investigation. Here, we report an extensive investigation with MRI, PET, and pathological examination. A 66-year-old Japanese female with a history of hypothyroidism, colon cancer, pheochromocytoma, and thymoma-associated myasthenia gravis presented with generalised tonic-clonic seizures. MRI showed multiple hyperintense lesions and PET showed hypermetabolic lesions in the brain. Biopsy showed n…

Paraneoplastic LGI1 Encephalitis Associated with Lung Adenocarcinoma: A Case Report.

Limbic encephalitis (LE) associated with anti-LGI1 antibodies is an autoimmune disorder characterized by memory decline, behavioral changes, and temporal lobe epilepsy. Faciobrachial dystonic seizures (FBDS) are a hallmark symptom, often preceding cognitive and psychiatric issues. This report presents an 80-year-old male with LGI1 encephalitis, initially manifesting as FBDS. A lung adenocarcinoma was diagnosed two months after the onset of neurological symptoms. Clinical and paraclinical data, including MRI and [18]FDG PET imaging, are described. The patient responded to immunotherapy, includi…

Autoimmune encephalitis as a possible reason for psychiatric hospitalisation in the teenage population.

Autoimmune encephalitis (AE) is a rare disease manifested by rapidly progressive short-term memory loss and other cognitive impairment accompanied by multiple disorders related to the limbic system involvement. The initial symptoms of autoimmune encephalitis may imitate other psychiatric disorders and delay the implementation of an appropriate treatment. The case description of a 15-year-old patient with an initial diagnosis of psychotic disorder has been presented. Because of atypical course of an illness and an ineffective treatment with psychotropic drugs, additional tests were made includi

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis.

PURPOSE OF REVIEW: The aim of this study was to assess the seizure manifestations and risk of epilepsy in encephalitis associated with antibodies against neuronal cell-surface (autoimmune encephalitis) or myelin-associated antigens, and to review several chronic epileptic disorders, including Rasmussen's encephalitis, fever-induced refractory epileptic syndromes (FIRES) and new-onset refractory status epilepticus (NORSE). RECENT FINDINGS: Seizures are a frequent manifestation of autoimmune encephalitis. Some autoimmune encephalitis may associate with characteristic features: faciobrachial dys

Neurobehavioral Disorders: The Corticolimbic System in Health and Disease.

The corticolimbic system (prefrontal cortices, amygdala, and hippocampus) integrates emotion with cognition and produces a behavioral output … aling with feline orofacial pain syndrome or feline hyperesthesia syndrome. By contrast, autoimmune limbic encephalitis requires prompt diagnosis and management with immunosuppression and seizure control.

Включает лимбическую систему, Spinal Cord и Peripheral Nerves.

Здоровая 28-летняя женщина неожиданно попала в неразрешимую эпилептику: припадок мозга превратился в общий припадок, которому предшествует высокая температура. Мозговые магнитные резонансные снимки указывают на двустороннюю гиперинтенсию в гиппокампе на Т2 взвешенной снимке. Электроэнцефалограммы постоянно демонстрируют диффузные острые волны и полипики. Комплексная иммунодибилизационная терапия и антиэпилептические препараты не привели к каким-либо улучшениям. Поэтому мы диагностировали у нее криптогенный лимфатический энцефалит и новообразованный рефрактический эпилептик (NORSE). Мы обнаружи

Совпадение в аннотации оригинала
A healthy 28-year-old woman presented suddenly with intractable status epilepticus: a focal seizure evolved into a generalized seizure prece … anti-epileptic drugs did not lead to any improvements. We therefore diagnosed her with cryptogenic limbic encephalitis and new-onset refractory status epilepticus (NORSE). We detected positive anti-ganglioside antibodies, IgG-GQ1b, GD1a, and GT1b, which were negative at six months after the onset. We emphasize the heterogeneous pathogenesis and intractable condition …

Autoimmune Encephalitis and Other Neurological Syndromes With Rare Neuronal Surface Antibodies in Children: A Systematic Literature Review.

Neuronal surface antibody syndromes (NSAS) are an expanding group of autoimmune neurological diseases, whose most frequent clinical manifest … in adult anti-GlyR and anti-amphiphysin autoimmunity, in children isolated epileptic syndromes and limbic encephalitis appear predominant, respectively. To our knowledge, this is the first systematic review on rare pediatric NSAS. An improved characterization may aid their recognition in children.

Spontaneous partial resolution of autoimmune-mediated brain MRI abnormalities before immunotherapy in anti-metabotropic glutamate receptor 5 encephalitis: a case report.

Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare autoimmune disorder characterized by varied neurological symptoms, often associated with limbic syndromes. We report the case of a 57-year-old female who presented with obvious ataxia, and then cognitive impairment and behavioral changes. MRI revealed cerebellar dentate nucleus and basal ganglia lesions with enhancement, which spontaneously regressed on follow-up imaging-a rare phenomenon in mGluR5 encephalitis. Diagnosis was confirmed by the presence of serum mGluR5 antibodies. Extensive evaluations excluded underlying mal