Anti-N-Methyl-D-Aspartate Receptor Encephalitis in a Patient with Systemic Lupus Erythematosus

Wu, Ying; Feng, Yan; Huang, Yue; Zhang, Jie Wen

doi:10.3988/jcn.2016.12.4.502

Cited by 10 publications

(9 citation statements)

References 10 publications

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“…Anti-NMDAR encephalitis may also develop with autoimmune disorders, such as autoimmune thyroid diseases, systemic lupus erythematosus, acute disseminated encephalomyelitis, and NMOSD [5,19,20]. HE is an autoimmune encephalopathy which is characterized by high titers of anti-thyroid antibodies [21].…”

Section: Discussionmentioning

confidence: 99%

Thyroid Function and Autoimmune Indications in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Ma¹,

Yin²,

Zeng³

et al. 2018

Neuroimmunomodulation

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Objective: Previous studies have shown that functional abnormalities of the thyroid are associated with the pathogenesis of several neurological diseases. However, their relationship in patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis remains to be defined. Methods: Forty-three patients with anti-NMDAR encephalitis were examined for thyroid function and autoimmune indications, in comparison with 225 healthy controls (CTL). Patients were further classified into 2 subgroups based on their free tri-iodothyronine (fT3) levels. Moreover, fT3 levels were also investigated after at least three months of follow-up. The clinical characteristics of the patients and CTL were described in detail. Results: Serum levels of fT3 and thyroid-stimulating hormone (TSH) were found to be relatively lower in patients with anti-NMDAR encephalitis than in CTL (both p < 0.001). Low T3 syndrome also occurred more frequently in anti- NMDAR encephalitis (25.6 vs. 0.4%, p < 0.001). However, no statistical differences were detected between patients and CTL in terms of the positive rate of thyroid antibodies and other types of thyroid dysfunction. Patients with low T3 levels tended to have a longer hospital stay (p = 0.006), a higher rate of abnormal brain magnetic resonance imaging (MRI) findings (p = 0.033), a higher frequency of consciousness declination (p = 0.029), and a higher modified Rankin scale (mRS) score during hospitalization. Low fT3 levels were also associated with abnormal MRI findings, a decline in consciousness, and the mRS score on admission. In addition, fT3 seemed to gradually return to normal levels upon improvement of the mRS score (r = –0.649, p = 0.002). Conclusions: Low T3 syndrome often copresents in anti-NMDAR encephalitis and indicates a longer hospitalization, abnormal MRI findings, consciousness declination, and a higher clinical severity. However, fT3 levels do not seem to influence the prognosis of anti-NMDAR encephalitis.

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Section: Discussionmentioning

confidence: 99%

Thyroid Function and Autoimmune Indications in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Ma¹,

Yin²,

Zeng³

et al. 2018

Neuroimmunomodulation

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“…Infectious causes such as herpes simplex encephalitis, malignancies such as carcinoma lung, thymoma etc., or any autoimmune disease, such as systemic lupus erythematous can predispose to the development of autoantibodies against cell-surface, synaptic or intracellular antigens. [123] Sometimes, it becomes difficult to ascertain whether the clinical presentations are due to the premorbid disease, it's relapse or due to the subsequently triggered auto-antibodies. Moreover, the clinical presentation of different causes of AE are more or less overlapping, leaving very few of them to be called as specific for a particular disease.…”

Section: Introductionmentioning

confidence: 99%

Antibody negative autoimmune encephalitis- Does it differ from definite one?

Pradhan¹,

Das²,

Das³

et al. 2019

Ann Indian Acad Neurol

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Context:Autoimmune encephalitis (AE) is an emerging cause of non-infective encephalitis, presentations of which vary widely. Traditionally the diagnosis of AE is based on detection of antibodies in a patient with clinical picture suggestive of AE.Aim:To evaluate the clinical characteristics and response to immunotherapy in patients with antibody negative autoimmune encephalitis and to compare them with definite cases.Settings and Design:A prospective follow-up study was done in patients presenting with presumptive symptoms of AE from January 2017 to January 2019. The study was done in a tertiary care institute of Northern India.Patients and Methods:Demographic and clinical parameters were noted and relevant investigations for management were done according to well-defined protocol. The patients were treated with immunomodulatory therapy in the form of steroids and/or intravenous immunoglobulins (IVIg). They were followed up for treatment response and relapse at 2 monthly intervals.Statistical Analysis Used:The data was expressed as either proportions or mean/median. Chi-square test/Independent T test was used to compare antibody positive and antibody negative group.Results:Out of 31 patients with presumptive AE, 16 patients tested positive for autoimmune antibodies (definite AE). Incidences of seizure, behavioral abnormalities, dementia and altered sensorium were similar between the 2 groups (p > 0.05). Complete or partial response was seen in all treated patients in both groups with no significant difference (p 0.716). CSF protein concentration and cellularity were higher in the definite group although only high protein concentration could reach statistical significance (p 0.002). Malignancy could be confirmed after extensive search in 2 out of 16 patients with definite AE and in 1 out of 15 antibody negative AE patients.Conclusions:Clinical presentation of antibody negative cases does not differ significantly from definite ones. Since treatment response is also similar in both the groups, starting immunotherapy in a patient presenting with presumptive symptoms of AE, while ruling out other common mimickers, seems to be the need of the hour in the management of this evolving entity.

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“…Our patient was ultimately diagnosed with a primary autoimmune RE associated with ACA after excluding potential causes, such as autoimmune disease or paraneoplastic syndromes [2, 7–9, 14, 15]. MRI revealed a form of RE specifically affecting the midbrain of the brainstem.…”

Section: Discussionmentioning

confidence: 95%

Recurrent post-partum rhombencephalitis associated with anti-centromere antibody: a case report

et al. 2019

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Background Rhombencephalitis (RE) is a serious condition of the brain with multiple etiologies. We report a unique case of recurrent, postpartum RE that is associated with positive anti-centromere antibody (ACA). A discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed. Case presentation A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods. Signs and symptoms included right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon of the brainstem. Extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid. Given the negative infectious work up with the prior episode and the nearly identical clinical presentations, the second episode was treated with corticosteroid only. This led to complete resolution of her symptoms and reversal of the brain magnetic resonance imaging (MRI) lesions. Conclusion To the author's knowledge, this is the first report of a primary autoimmune RE during postpartum period that is associated with ACA. Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age. Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE.

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Anti-N-Methyl-D-Aspartate Receptor Encephalitis in a Patient with Systemic Lupus Erythematosus

Cited by 10 publications

References 10 publications

Thyroid Function and Autoimmune Indications in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Thyroid Function and Autoimmune Indications in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

Antibody negative autoimmune encephalitis- Does it differ from definite one?

Recurrent post-partum rhombencephalitis associated with anti-centromere antibody: a case report

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