Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan

Kong, Shu-Sing; Chen, Yun‐Ju; Su, I-Chen; Lin, Jainn-Jim; Chou, I-Jun; Chou, Min-Liang; Hung, Po-Cheng; Hsieh, Meng‐Ying; Wang, Yishan; Chou, Cheng-Che; Wang, Huei-Shyong; Lin, Kuang-Lin

doi:10.1016/j.pedneo.2018.10.006

Cited by 28 publications

(24 citation statements)

References 13 publications

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“…The ratio of patients with good prognosis in our study was 76.6% which similar to other study [30,16,10,14,12]. The predictors of poor outcome in previous study including decrease consciousness, speech disorder, longer hospital day, higher initial mRS, and treatment with RTX were consistent with the outcomes of our study [30,16,10,14,12,31]. Byrne et al found the patients who recovered completely had shorter interval (15 days) between onset and initiation of treatment than that of those who did not recovered completely (21 days) [32].…”

Section: Discussionsupporting

confidence: 92%

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Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Hou

et al. 2020

Preprint

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Background: Research on pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is limited. We studied clinical features of children with anti-NMDAR encephalitis in southern China.Methods: Pediatric anti-NMDAR encephalitis between 2014 and 2019 from one tertiary medical center was analyzed. The Modified Rankin Scale (mRS) score was adopted to evaluate outcomes.Results: 107 children (M/F=50/57; mean onset age=6.3 y) with anti-NMDAR encephalitis were involved. Boys significantly showed earlier onset age, higher ratio of fever, longer hospital day, more courses of steroid treatment, higher mRS score after treatment. 48.6% of patients had prodromal events with infectious events most common. The most common symptom at onset and during whole course was seizures and psychiatric symptoms respectively. Initial median white cell count of cerebral spinal fluid (CSF) was 22.0x106/L. 12.1% of patients had positive herpes simplex virus DNA of CSF. All patients had CSF positive NMDAR antibodies, although 86.9% of patients had serologically positive. 9.3% of patients had overlapped other neuronal antibodies. Electroencephalograph showed abnormalities with slow wave (95.3%) and epileptic activity (41.5%). Delta brush was revealed in 3.8% of patients. 36.4% of patients had lesions in brain MRI, with local lesions most common. No tumor was found in all. 84.9% of patients responded well to the first line therapy (steroid plus immunoglobulin), while 12 patients accepted second-line therapy (Rituximab) with 91.7% of effective rate. 12.1% of patients relapsed. 2 male patients died. The median length of hospital stay was 28 days. The mRS score was significantly improved after treatment. 51.0% of patients had a full recovery. 76.6% of patients had mild neurological disability (mRS≤2). Male, speech disorder, initial score of mRS and administration of second-line therapy were independent risk factors associated with the outcome.Conclusions: Of pediatric anti-NMDAR encephalitis in southern China: the mean onset age was about 6-year-old, earlier in boys; boys remained slower to recover and with a worse prognosis; the most common respective symptom at onset and during whole course was seizures and psychiatric symptoms; combination with tumor was rare; most patients respond well to the immunotherapy; relapse and fatality rate were relatively low; most patients had a good prognosis.

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Section: Discussionsupporting

confidence: 92%

“…Male, lesions in brain MRI and autonomic dysfunction served as predictors of poor outcome in our study, although previous study did not [30,16,10,14,12,31]. We found patients aged 3 years or younger was a risk factor for outcome.…”

Section: Discussionmentioning

confidence: 46%

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Hou

et al. 2020

Preprint

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“…Intravenous methylprednisolone, IVIG, plasmapheresis and tumor surgery, if present are applied as first-line treatments and second-line treatments (rituximab and cyclophosphamide) are started in cases that do not ameliorate (14). In a cohort study conducted on anti-NMDA receptor encephalitis in children regarding the treatment process, the authors have reported that immunosuppressive treatment for more than 12 months was given to 40% of the cases (6).…”

Section: Discussionmentioning

confidence: 99%

Anti-NMDA Receptor Encephalitis Presenting with Acute Psychosis

Çelik¹,

Özdemir²,

Tolunay³

et al. 2019

J Pediatr Inf

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Günümüzde tetkik imkanlarının çoğalması ve klinisyenlerin konuya dair farkındalığının artması ile otoimmün ensefalit hastaları daha sık tanı alabilmektedir. Anti-N-metil-D-aspartat (NMDA) reseptör ensefaliti, çocukluk çağında en sık görülen otoimmün ensefalit tipidir. Hastalığa erken dönemde tanı konulması, prognoza önemli oranda katkı sağlamaktadır. Beyin omurilik sıvısında ve serumda saptanan anti-NMDA reseptörlerine karşı gelişen antikorların saptanması ile tanı konulmakta, santral sinir sistemi görüntülemeleri ve elektroensefalografi (EEG) bulguları hastalığa özgün olmayıp, tanıda destekleyici özellik arz edebilmektedir. Ateş, psikotik semptomlar, bilinç kaybı ile başvuran ve anti-NMDA reseptör ensefaliti tanısı alan 15 yaşındaki kız olgu sunulmuştur.

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“…However, the expense of this natural cure is delay in hospitalization and associated serious complications that may arise due to the delay in hospitalization and lack of proper regular care. After the identification of the patient's symptoms and antibodies, targeted immunotherapy should be initiated as soon as possible [33]. Approximately 48% of patients without tumors had substantial improvements after first-line immunotherapy compared with 80% of patients with tumors.…”

Section: Anti-nmdar Encephalitismentioning

confidence: 99%

“…However, recurrence indicated that tumor activity can also occur alone, in some cases even 15 years after onset [28,36]. Tumor diagnosis can also be considerably delayed (by up to 7 years) after the manifestation of early clinical symptoms of encephalitis [33].…”

Section: Anti-nmdar Encephalitismentioning

confidence: 99%

Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies

Li¹,

Zhang²,

Liu³

et al. 2020

Neuroimmunomodulation

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Several studies have certified that autoantibodies play an important role in the manifestation of neuromuscular diseases. Scientists have discovered specific neuronal tumor antibodies in patients with typical paraneoplastic neurological disorders. But in some clinical cases, it is not useful to cure this disease with common treatments unless the autoantibodies are addressed. In addition, recent studies have shown a close relationship between certain antibodies and neuronal surface proteins in some special cases. These antibodies, which act on the surface of neurons, mainly include voltage-gated calcium channel (VGKC) antibodies. VGKC antibodies are further divided into several types including antileucine-rich glioma inactivated 1 (LGI1), anti-contactin-associated protein-like 2 (Caspr2), anti-N-methyl-D-aspartate receptor (NMDAR), anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), anti-γ-aminobutyric acid receptor (GABAR), and glycine receptor. For the purpose of this review, cases of clinical studies of autoantibody-associated encephalitis were collected, the key points regarding the pathogenesis were summarized, the clinical manifestation was discussed, and all this information was organized as this review in order to introduce the relationship between autoantibodies and autoimmune encephalitis. Furthermore, it is hoped that it can effectively direct the development of diagnostic and therapeutic approach in the future.

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Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan

Cited by 28 publications

References 13 publications

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Pediatric Anti-NMDAR Encephalitis in Southern China: Analysis of 107 Cases

Anti-NMDA Receptor Encephalitis Presenting with Acute Psychosis

Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies

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