1.Anti N-Methyl-D-Aspartate Receptor Encephalitis with Ovarian Teratoma: A Dilemma in Diagnosis
Lee CL ; Zainuddin AA ; Abdul Karim AK ; Yulianty A ; Law ZK ; Md.Isa N ; Nor Azlin MI
Journal of Surgical Academia 2016;6(1):66-69
We report a rare case of altered mental status in a young patient with immature ovarian teratoma. A 22-year-old
woman presented with seizures, hallucination, amnesia and orofacial dyskinesia. Examination and investigation
revealed an ovarian massand asalphing-oophorectomy was performed. The histopathological examination result
showed an immature teratoma grade 2 with thepresence of immature primitive glial tissue. Her CSF N-Methyl-DAspartic
acid receptor (Anti-NMDAR) antibodytest was positive. N-Methyl-D-Aspartic acid receptor antibody
associated limbic encephalitis is an autoimmune antibody-mediated neuropsychiatric disorder. Resection of the
tumour and immunotherapy resulted in full recovery.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
2.Important differential in a patient presenting with neuropsychiatric symptoms: Anti-N-Methyl-D-Aspartate receptor encephalitis
The Medical Journal of Malaysia 2017;72(5):306-307
Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is
an immune mediated condition, which remains relatively
unknown in Malaysia outside tertiary hospitals with
neurology unit. It is often misdiagnosed as a psychiatric
illness before definitive treatment is instituted. We report
here an 18-year-old man who initially presented to the
psychiatry unit before he was subsequently diagnosed as
having anti-NMDAR encephalitis. To our knowledge, this is
the first reported case of anti-NMDAR encephalitis in the
east coast of Peninsular Malaysia.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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Encephalitis
3.Cryptococcal Encephalitis Complicating Anti-N-methyl-D-aspartate Receptor Encephalitis in an Immunosuppressed Patient.
Dongming LIU ; Hongwei XU ; Fang YI
Acta Academiae Medicinae Sinicae 2020;42(3):417-420
Cryptococcal encephalitis is a fatal central nervous system infectious disease,whereas anti-N-methyl-D-aspartate(NMDA)receptor encephalitis(NMDARE)is an autoimmune syndrome associated with psychological symptoms,behavioural abnormalities,seizures,and dyskinesias.Despite their distinct pathologies and pathogenic mechanisms,both of them can lead to cognitive dysfunction and abnormal behaviors,although anti-NMDARE can also have mood and mental disorders as its core manifestations.A patient with nephrotic syndrome accompanied by both cryptococcal encephalitis and anti-NMDARE was treated in our center,which for the first confirmed that these two conditions could coexist in one patient.The underlying mechanism may be similar to that of anti-NMDARE after other infections.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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Antibodies
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Humans
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Receptors, N-Methyl-D-Aspartate
4.Recent research on immunotherapy for anti-N-methyl-D-aspartate receptor encephalitis.
Chinese Journal of Contemporary Pediatrics 2022;24(8):948-953
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a central nervous system disease characterized by neurological and psychiatric symptoms. Immunotherapy is the basic treatment for this disease, including first- and second-line therapies for the acute stage and the long-course therapy for the chronic stage. Anti-NMDAR encephalitis often has a good prognosis, but some patients may still have neurological dysfunction due to poor response to current immunotherapy. In addition, the adverse reactions and economic burden of drugs are practical problems in clinical practice. To solve the above problems, continuous improvements have been made in immunotherapy regimens in terms of dose, route of administration, and course of treatment, and some new immunotherapy drugs have emerged. This article reviews the recent research on immunotherapy for anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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Humans
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Immunotherapy
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Receptors, N-Methyl-D-Aspartate
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Retrospective Studies
5.Clinical presentations of pediatric anti-N-methyl-D-aspartate receptor encephalitis.
Chinese Journal of Contemporary Pediatrics 2014;16(6):575-577
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis in children with characterized clinical features. Here we review clinical presentations of typical and atypical anti-NMDAR encephalitis and characteristics of clinical presentations of pediatric anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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diagnosis
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Child
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Humans
6.A Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated with Glioma of the Pons.
Francesca BERETTA ; Angelo ALIPRANDI ; Claudio DI LEO ; Andrea SALMAGGI
Journal of Clinical Neurology 2019;15(1):125-127
No abstract available.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis*
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Glioma*
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Pons*
7.Anti-NMDA-receptor encephalitis in Filipino adults: Case series and outcomes in a tertiary government hospital in the Philippines.
Rodelia C. Pascua ; Debbie Co Liquete
Philippine Journal of Neurology 2022;25(1):28-35
INTRODUCTION:
We performed a case series of all five (5) confirmed adult Filipino cases of Anti-N-Methyl-D-Aspartate
receptor (anti-NMDA-R) encephalitis in a tertiary government hospital in the Philippines admitted in the
past three years. Two cases were identified with unique features: (1) a 23-year old female who presented
with combined refractory seizures and persistent chorea and orofacial dyskinesias; and (2) a 22-year old
male who presented with refractory epilepsia partialis continuua. The rest of the patients were hereby
presented.
BACKGROUND:
In the past years, anti-NMDA-R encephalitis has been considered a diagnosis of exclusion in lieu of other
infectious causes of encephalitis. It is rare and an emerging disease with an incidence estimated at
approximately 2-3 cases per million. Recent literature recorded severe cases of anti-NMDA-R encephalitis
that presented as intractable first onset seizures, combined with hyperkinetic movement disorders, acute
psychosis without a premorbid condition, and dysautonomia.
OBJECTIVES:
To present the clinicodemographic profile and to discuss the management and outcomes of patients with
anti-NMDAR encephalitis in a tertiary hospital in the Philippines.
RESULTS:
Here, we report five confirmed cases of anti-NMDA-R encephalitis admitted in 2019-2021. The mean age is
23 years old, with 4:1 female to male ratio with a median length of hospitalization of 58 days. All patients
presented with acute psychiatric symptoms without premorbid condition, focal and generalized seizures,
decreased consciousness, dyskinesias, and autonomic instability. Four patients needed airway support for
central hypoventilation, one had first onset seizure that developed into refractory epilepsia partialis
continuua, one had persistent chorea and orofacial dyskinesia. Imaging studies of the brain included
contrast-enhanced CT Scan and MRI with unremarkable findings. No female patients had an ovarian
teratoma as revealed in the whole abdominal ultrasound. All CSF analysis for anti-NMDA-receptor was
done in the same laboratory outside the hospital which revealed positive for NMDA-receptor antibodies,
while CSF lymphocytic pleocytosis was only seen in 1/5 and protein elevation in 4/5. All of the patients
underwent electroencephalogram (EEG) studies which revealed diffuse delta-theta slowing without
epileptiform discharges. The patient who had persistent chorea and orofacial dyskinesias showed extreme
delta brush, while one had normal EEG findings. They all received high-dose steroid and intravenous
Immunoglobulin (IVIg); three patients were able to undergo Rituximab infusion. Only one female patient
had mild deficits, one female was discharged fully functional and ambulatory from being weaned off from
the mechanical ventilator, one female had aborted cardiac arrest and was discharged bedridden at GCS 10,
and two died due to the other concomitant medical conditions. The Modified Rankin Scale (MRS) and
Mini-mental Status Examination (MMSE) were used to assess the neurological and functional outcomes of
our patients.
CONCLUSION
Anti-NMDA-R encephalitis is an emerging neurological disorder that warrants early identification as it
impacts timeliness of management and long-term outcomes.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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Status Epilepticus
8.A rare disease with a unique feature: Anti-NMDA receptor encephalitis and mesenteric teratoma.
Carpio Iris Sylvan L ; Reyes Jose Paciano Baltazar T ; Reyes Heizel M
Philippine Journal of Neurology 2012;16(1):49-
BACKGROUND: Antibodies to neuronal extracellular membrane antigens, including the NR1 subunit of the N-methyl-D-aspartate receptor, have been associated with neuropsychiatric symptoms. In 2003, the first case of anti-NMDA receptor encephalitis was identified in a young woman with subacute onset of ehavioural changes and seizures.
OBJECTIVES: The aim of this case report is to describe the clinical features, disease course and management of a documented case of anti-NMDA receptor encephalitis in the Philippines. It also aims to highlight a unique feature of the disease as seen in the patient.
CASE DESCRIPTION: The patient is a 36-year-old female with no known co-morbidities presenting with subacute onset of ehavioural change and generalized seizures. She was initially worked-up for viral encephalitis but the diagnostic tests were negative. With a suspicion of anti-NMDA receptor encephalitis, a search was made for the presence of a teratoma. This was confirmed by imaging studies and subsequent surgical resection of an immature mesenteric teratoma was done. Immunohistochemical assay of both blood and CSF were positive for NMDAR antibodies.
CONCLUSION: This is the first confirmed case of anti-NMDA receptor encephalitis reported in the Philippines. It has the unique feature of being associated with an immature mesenteric teratoma, a finding that has not been reported in literature. The possibility of anti-NMDAR encephalitis need to be considered among patients with subacute onset of ehavioural changes and seizures when usual diagnostic tests for the more common etiologies prove negative.
Human ; Female ; Adult ; Anti-n-methyl-d-aspartate Receptor Encephalitis ; Antibodies ; Encephalitis, Viral ; Mesentery ; Receptors, N-methyl-d-aspartate ; Teratoma
9.Anti-NMDA Receptor Encephalitis Which Has Shown Clinical Symptoms of Schizophrenia.
Hyung Seok LEE ; Seung Woo KIM ; Seok Jong CHUNG ; Han Soo YOO ; Phil Hyu LEE ; Sun Ah CHOI
Journal of the Korean Neurological Association 2013;31(2):115-117
Encephalitis associated with antibodies to the N-methyl-D-aspartate (NMDA) receptor has variable clinical manifestations and treatment responses. Anti-NMDAR encephalitis is often associated with ovarian teratoma, but some cases without tumor have been reported. Here, we describe a patient who has shown psychiatric symptoms, memory impairment and been diagnosed as schizophrenia for one year and had anti-NMDA receptor antibody. The patient showed atypical clinical course compared to previous cases with anti-NMDA receptor encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis
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Antibodies
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Encephalitis
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Humans
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Memory
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N-Methylaspartate
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Schizophrenia
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Teratoma
10.Electroencephalographic features of anti-N-methyl-D-aspartate receptor encephalitis in children.
Xin GAO ; Zhixian YANG ; Jiao XUE ; Taoyun JI ; Yao ZHANG ; Xiaoyan LIU ; Ye WU ; Yuehua ZHANG ; Xinhua BAO ; Yuwn JIANG
Chinese Journal of Pediatrics 2016;54(3):192-196
OBJECTIVETo investigate electroencephalographic (EEG) characteristics of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in children.
METHODClinical data of 28 children diagnosed as anti-NMDAR encephalitis were retrospectively analyzed for EEG characteristics in different periods and severity of disease and outcome.
RESULTAmong the 28 patients with anti-NMDAR encephalitis, 15 were males and 13 were females. Their age at disease onset ranged from 1 year 3 months to 12 years 4 months. Patients were divided into mild group (5 cases) and severe group (23 cases). In the different stage of the disease, occipital background activity of the EEG was preserved in more than half of patients. Accompanied by the evolution of disease course, the occipital background activity and slow waves gradually recovered to normal. In the peak phase of disease, occipital background activity in the awake state was preserved in 4/5 patients of the mild group and 9/17 patients of the severe group. Alpha and theta band rhythms in non-rapid eye movement (NREM) sleep existed in 77% (17/22) patients. EEG monitoring showed delta brushes in 2 cases, and the delta brushes were mixed with background fast waves in one case; 71% (20/28) patients had epileptiform discharges in EEG during the course, and among them, 6 patients had secondary epilepsy.
CONCLUSIONThe background activity in the awake state and abnormal diffuse slow waves of EEG were evolved and gradually recovered during the course of the disease. Regardless milder or severe illness condition, occipital background activity was still preserved during different stages in most patients. Alpha and theta rhythms in NREM sleep might represent a relatively overt EEG characteristic. The presence of delta brush in EEG was rare, and sometimes they were difficult to be distinguished from fast wave activities caused by drugs. The presence of epileptiform discharges in EEG suggested the possibility of secondary epilepsy.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Child ; Electroencephalography ; Female ; Humans ; Male ; Receptors, N-Methyl-D-Aspartate ; Retrospective Studies ; Sleep