scholarly journals Pathological Findings in Male Patients With Anti-N-methyl-d-Aspartate Receptor Encephalitis

2019 ◽  
Vol 78 (8) ◽  
pp. 735-741 ◽  
Author(s):  
Makito Hirano ◽  
Tatsuki Itoh ◽  
Harutoshi Fujimura ◽  
Kimiko Inoue ◽  
Makoto Samukawa ◽  
...  
Keyword(s):  
Microglial Activation ◽  
Cell Function ◽  
Neuronal Loss ◽  
Autoimmune Encephalitis ◽  
Brain Pathology ◽  
Testicular Damage ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Male Patients ◽  
Leydig Cell Function

Abstract Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune encephalitis. The disease predominantly affects women (1:5–1:10), with only 3 reports of autopsy findings in women being published to date. The present study reports findings from the first autopsy performed on a man with anti-NMDAR encephalitis. The patient had some scattered lesions in the limbic system with neuronal loss, gliosis, and microglial activation. The temporal and frontal cortices showed additional patchy demyelination. T-lymphocyte infiltration was detectable in the fusiform gyrus lesion. These findings were partly similar to those reported in female patients. Although clinical differences based on the sex of the patient are reported for this disease, the observed pathological similarities potentially help to establish common therapeutic strategies for all patients. Severe testicular damage was additionally observed in the male patient in this study. Biopsy-proven severe testicular damage was also confirmed in another, previously fertile man who became azoospermic. Moreover, serum follicle-stimulating hormone levels, which often increased in response to disturbed spermatogenesis, were elevated, and testosterone/luteinizing hormone ratio reflecting Leydig cell function was low in all 5 male patients in this study. Overall, these findings suggest similar brain pathology in patients of both sexes and severe testicular damage in male patients.

Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

2020 ◽  
Vol 19 (5) ◽  
pp. 124-131
Author(s):  
N.M. Podzolkova ◽  
◽  
V.V. Korennaya ◽  
O.S. Levin ◽  
E.E. Vasenina ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Rare Complication ◽  
Autoimmune Encephalitis ◽  
Neurological Deficits ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Life Threatening ◽  
Causal Treatment ◽  
Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

scholarly journals The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

scholarly journals The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

2019 ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. The anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special anti-NMDAR encephalitis patient who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, and the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions The anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. The recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

scholarly journals Current Progress on Assessing the Prognosis for Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Hao Wang ◽  
Zheng Xiao
Keyword(s):  
Positive Impact ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Antibody Testing ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Behavioral Abnormalities ◽  
Positron Emission ◽  
The Impact

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common antineuronal antibody encephalitis in autoimmune encephalitis found at present. It has complex clinical manifestations such as psychiatric and behavioral abnormalities, seizures, movement disorders, consciousness, and autonomic dysfunction. The relationship between those manifestations and prognosis is unclear. Electroencephalography (EEG) is gradually becoming useful in the evaluation of the prognosis of anti-NMDAR encephalitis patients, while imaging and antibody testing have a limited prognostic value. Starting the patients on adequate treatments (such as immunotherapy) in a timely fashion has a positive impact on their prognosis. Nevertheless, research on the prognosis of anti-NMDAR encephalitis remains scarce. Here, we review the current advances of prognosis-related research from the clinical manifestations of the disease and auxiliary examinations such as EEG, magnetic resonance imaging (MRI), 18F fluorodeoxyglucose positron emission tomography (FDG-PET), and antibody measurement. In addition, we also discuss the impact of different treatment options on prognosis. In-depth research on the prognosis of patients with anti-NMDAR encephalitis will contribute to a better understanding of this disease, leading to better treatments options and, ultimately, a better prognosis.

scholarly journals Anti-N-Methyl-D-Aspartate Receptor Encephalitis in HIV Infection

2016 ◽  
Vol 8 (3) ◽  
pp. 251-257 ◽  
Author(s):  
Eunice Patarata ◽  
Vera Bernardino ◽  
Ana Martins ◽  
Rui Pereira ◽  
Conceição Loureiro ◽  
...  
Keyword(s):  
Infected Individual ◽  
Autoimmune Encephalitis ◽  
Rare Condition ◽  
Reproductive Age ◽  
Neoplastic Disease ◽  
Acute Confusional State ◽  
Confusional State ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Extrapyramidal Signs

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Her mental state progressively deteriorated. Without evidence of infection, the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid clinched the diagnosis of autoimmune encephalitis. No evidence of neoplastic disease was found, and the beneficial response to immunosuppressive therapy was exceptional. This is the first report of anti-NMDAR encephalitis in an HIV-infected individual, reminding us that autoimmune encephalitis should be included in the differential diagnosis of a young patient presenting in an acute confusional state.

scholarly journals Case Report: Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Elderly Patient With Primary Sjögren's Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Xia Li ◽  
Rui Kong ◽  
Qiuju Liao ◽  
Jing Ye ◽  
Yi Zhao
Keyword(s):  
Elderly Patient ◽  
Autoimmune Encephalitis ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Sjögren‘S Syndrome

Neurological manifestations of primary Sjögren's syndrome (SS) are diverse involving the peripheral and central nervous system. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, as the most prevalent autoimmune encephalitis, was rarely reported to be complicated with primary SS. Herein, we present an elderly patient with a 15-year history of primary SS presenting with progressive cognitive dysfunction due to anti-NMDAR encephalitis that was once misdiagnosed as primary degenerative dementia. Early recognition of anti-NMDAR encephalitis and initiation of treatment with steroids and immunosuppressant gained a favorable outcome. Our findings enhance the awareness that autoimmune encephalitis should be taken into account in the patients with primary SS presenting with progressive cognitive impairment.

Anti-N-Methyl-D-Aspartate receptor Encephalitis as a potential cause of worsening Herpes Simplex Virus Encephalitis.

2020 ◽  
pp. 1-3
Author(s):  
Saeed Arif ◽  
Shaheer Arif ◽  
Admin
Keyword(s):  
Herpes Simplex Virus ◽  
Herpes Simplex ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Herpes Simplex Virus Encephalitis ◽  
Virus Encephalitis ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Intravenous Acyclovir ◽  
Simplex Virus

Madam,Herpes Simplex Virus Encephalitis (HSVE) though rare tends to be lethal if acquired without treatment. The mortality of untreated HSE is up to 70% (1) and most patients don’t return to normal function (1). That said with prompt recognition and treatment with antiviral therapy the prognosis is considerably better (1). HSVE may become complicated by the presence of autoimmune encephalitis particularly Anti-N-Methyl-D-Aspartate receptor Encephalitis (NMDAR) (2). When this occurs the differentiation between two is not possible clinically and radiologically. In such situations the understanding and awareness of autoimmune encephalitis profile which also includes test for NMDAR antibodies becomes very important. Although such expensive tests have become available in Pakistan but the facility is present at only a hand full of centers along with unawareness of this possible condition making the situation worse. We encountered such a situation with our 60-years-old patient who initially presented with altered behaviour and recurrent episodes of focal seizures of left arm with secondary tonic clonic generalization. He was subsequently diagnosed with HSVE on cerebrospinal fluid (CSF) HSV polymerase chain reaction (PCR) and was treated with intravenous acyclovir. Magnetic resonance imaging of the patient showed lesions in the insular cortex, hippocampus, medial frontal lobes and temporal lobes bilaterally (Figure 1). The patient had resolution of symptoms with relapse of altered behaviour eight weeks later. On CSF PCR for HSV this time was negative, with positive serum Anti-N-Methyl-D-Aspartate receptor (NMDAR) antibodies. This condition was treated with plasmapharesis and intravenous methylprednisolone without acyclovir.HSV has been postulated to induce autoimmunity against neuronal cell proteins (3, 4). Moreover, NMDAR Encephalitis has also been described in the literature as an etiology of “Relapsing Post-HSVE”, which is amenable to immune modulation if diagnosed and treated timely. Although one of the considered differential diagnosis in cases of relapse of HSVE, at the same time, NMDAR Encephalitis can also be a reason of worsening or lack of improvement of patients suffering from HSVE (1,2). These clinical situations may be missed by clinicians. So, it is important to differentiate among these clinical situations timely, which can be done easily by the antibody tests. This is important because the direction of therapy changes with initiation of immunotherapy if anti-NMDAR antibodies are tested positive. This may result in improvement in a large subset of patients (5).Continuous...

N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl

2019 ◽  
Vol 50 (04) ◽  
pp. 253-256
Author(s):  
A. Barth ◽  
I. Nassenstein ◽  
R.B. Tröbs ◽  
A. Tannapfel ◽  
H. Dercks ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Ovarian Tumor ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Adolescent Females ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

AbstractN-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare antibody-mediated autoimmune encephalitis often associated with an ovarian teratoma in adolescent females. Here we present a 17-year-old girl with only and unusual psychiatric symptoms as part of her NMDAR encephalitis in combination with a very small ovarian teratoma suspected by magnetic resonance (MR) imaging and finally histologically confirmed. We further review the literature of NMDAR encephalitis in combination with an ovarian teratoma and discuss the recommended radiological workup in children with a suspected ovarian tumor.

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