Anti-N-Methyl-D-Aspartate Receptor Encephalitis in a 3-Year-Old Toddler with the Involvement of Severe Autonomic Dysfunctions in Gastrointestinal System: A Case Report

2018 ◽  
Vol 17 (01) ◽  
pp. 041-044 ◽  
Author(s):  
Li-Ying Liu ◽  
Yang-Yang Wang ◽  
Ling-Yu Pang ◽  
Gui-Xia Zhang ◽  
Li-Ping Zou
Keyword(s):  
Gastrointestinal System ◽  
Immunomodulatory Treatment ◽  
Gastrointestinal Dysmotility ◽  
System A ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Rare Manifestation ◽  
Autonomic Dysfunctions ◽  
First Onset ◽  
Receptor Antibodies

AbstractAnti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is under-recognized in children and adults. It is characterized by prominent neurologic and psychiatric features at first onset. Autonomic manifestations are rarer in children than in adults. We report a 3-year-old male patient with rare manifestation of severe autonomic dysfunctions of the gastrointestinal system and atypical prodromal symptoms. The serum and CSF of the patient were positive for anti-NMDA receptor antibodies, and his electroencephalogram results showed an “extreme delta brush” pattern. The patient had completely recovered by the sixth-month assessment after receiving first-line immunomodulatory treatment. Thus, anti-NMDAR encephalitis should be considered in the differential diagnosis of children with mental status changes and widespread autonomic dysfunction, particularly if symptoms are accompanied by gastrointestinal dysmotility and anhidrosis.

scholarly journals Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Decrease in Blood Flow in Cerebellum

2021 ◽  
pp. 17-23
Author(s):  
Koji Obara ◽  
Tomoko Ono ◽  
Itaru Toyoshima
Keyword(s):  
Working Memory ◽  
Blood Flow ◽  
Ovarian Teratoma ◽  
Emission Computed Tomography ◽  
High Dose ◽  
Promising Alternative ◽  
Flow Monitoring ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Consciousness Disturbance

In anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, progressive cerebellar atrophy potentially leads to severe sequelae. We encountered a patient with anti-NMDAR antibody encephalitis who showed a decrease of blood flow in the cerebellum. A 15-year-old girl presented with consciousness disturbance. Influenza encephalopathy was suspected, and she was treated with glucocorticoid pulse therapy, high-dose intravenous immunoglobulins, and plasma exchange sequentially. She subsequently underwent left oophorectomy due to the presence of anti-NMDAR antibodies and a left ovarian teratoma. In spite of the surgery, her neuropsychiatric symptoms persisted, and she recovered slowly after the introduction of oral methotrexate (MTX). Sequential cerebral blood flow monitoring with single-photon emission computed tomography showed marked cerebellar hypoperfusion. Although mild impairments including working memory and verbal fluency persisted, she eventually returned to high school 3 years after onset. Profound cerebellar hypoperfusion including lobules VI and VII may be the reason for her working memory impairment and speaking problems. Oral MTX may be a promising alternative treatment for some refractory cases of anti-NMDAR encephalitis.

Catatonia secondary to anti-N-methyl-D-aspartate receptor (NMDAr) encephalitis: a review

CNS Spectrums ◽  
2019 ◽  
Vol 25 (4) ◽  
pp. 475-492 ◽  
Author(s):  
Inês Marques Macedo ◽  
João Gama Marques
Keyword(s):  
Disease Management ◽  
Literature Review ◽  
Clinical Setting ◽  
Neuropsychiatric Symptoms ◽  
Initial Symptom ◽  
Know How ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Psychiatric Conditions ◽  
Possible Cause

AbstractAnti-N-methyl-D-aspartate receptor (NMDAr) encephalitis is a relatively recent autoimmune entity, as it was first described in 2007. Given that it is a condition with neuropsychiatric symptoms, its initial symptom is frequently psychiatric in nature. Hence, psychiatrists are often the first physicians to assess these patients and, as so, must recognize this type of encephalitis as a possible cause. Catatonia may be inaugural or develop throughout the course of the disease. Management of patients with anti-NMDAr encephalitis is based on etiologic treatment with immunotherapy and removal of the associated tumor, if any. However, these catatonic patients may have variable responses to etiologic treatment, sometimes with refractory catatonic symptoms, which attests to the necessary urgency to know how to manage these patients. In the clinical setting, physicians appear to be using guidelines originally created to the management of catatonia due to primary psychiatric conditions. In this literature review, catatonia was historically contextualized and anti-NMDAr encephalitis overall described. Finally, catatonia secondary to this type of encephalitis was discussed.

Pure ataxia associated with N-methyl-d-aspartate receptor antibodies

2014 ◽  
Vol 20 (5) ◽  
pp. 568-569 ◽  
Author(s):  
Diana Aguiar de Sousa ◽  
Patrícia Pita Lobo ◽  
Ana Castro Caldas ◽  
Miguel Coelho ◽  
Luísa Albuquerque
Keyword(s):  
Aspartate Receptor ◽  
Receptor Antibodies

scholarly journals Expression of Various Glutamate Receptors Including N-Methyl-D-Aspartate Receptor (NMDAR) in an Ovarian Teratoma Removed from a Young Woman with Anti-NMDAR Encephalitis

2010 ◽  
Vol 49 (19) ◽  
pp. 2167-2173 ◽  
Author(s):  
Naoko Tachibana ◽  
Takashi Shirakawa ◽  
Keiko Ishii ◽  
Yukitoshi Takahashi ◽  
Keiko Tanaka ◽  
...  
Keyword(s):  
Glutamate Receptors ◽  
Young Woman ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

scholarly journals Anti-n-Methyl-d-Aspartate-Receptor (NMDAR) Encephalitis in Association with Ovarian Teratoma

Cureus ◽  
2017 ◽  
Author(s):  
Javaad Ahmad ◽  
Muhammad Saad Sohail ◽  
Amina Khan ◽  
Ahmed H Qavi ◽  
Pramod Gaudel ◽  
...  
Keyword(s):  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

scholarly journals Overlapping anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis With Neuromyelitis Optica Spectrum Disorders: A Case Report

2021 ◽  
Author(s):  
Jialin Pan ◽  
Begench Ovlyakulov ◽  
Lili zhou
Keyword(s):  
Neuromyelitis Optica ◽  
Brain Magnetic Resonance Imaging ◽  
Chinese Patient ◽  
Cerebellar Hemisphere ◽  
Double Positive ◽  
Blurred Vision ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Abstract BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.Case presentationThis report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD is presented. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments. ConclusionsThis case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double positive antibodies remain elusive.

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