Spectrum of anti-NMDA receptor antibody encephalitis: Clinical profile, management and outcomes

Datta, Amlan Kusum; Pandit, Alak; Biswas, Samar; Biswas, Atanu; Roy, Biman Kanti; Gangopaddhyay, Goutam

doi:10.4103/aian.aian_817_20

Cited by 7 publications

(12 citation statements)

References 28 publications

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“…The chronic TBI might also cause a disruption in the blood-brain barrier leading to brain inflammation and dementia, as proposed by Abrahamson and Ikonomovic [26] Other types of brain injury such as ischemia are known to be accompanied by antibodies against the NMDAR subunit NR1 that are closely associated with the outcome of neuropsychiatric symptoms [27]. The NMDAR, in particular, might play an important role in TBI, as it has an important function in regulating synaptic plasticity and memory formation [28] explaining predominant learning and memory deficits in patients with NMDAR antibodies, as a study by Datta et al showed [29]. The role of AP3B2 antibodies is less clear than those of the NMDAR antibodies.…”

Section: Discussionmentioning

confidence: 97%

Mild Cognitive Impairment in Chronic Brain Injury Associated with Serum Anti-AP3B2 Autoantibodies: Report and Literature Review

et al. 2021

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Background: Chronic traumatic brain injury is a condition that predisposes the brain to activate B-cells and produce neural autoantibodies. Anti-adaptor protein 3, subunit B2 (AP3B2) autoantibodies have thus far been associated with diseases affecting the cerebellum or vestibulocerebellum. Through this case report, we aim to broaden the spectrum of anti-AP3B2-associated disease. Case description: We report on a 51-year-old woman with a brain injury approximately 28 years ago who recently underwent neuropsychological testing, magnetic resonance imaging of the brain (cMRI), and cerebrospinal fluid (CSF) analysis. Neural autoantibodies were determined in serum and CSF. Our patient suffered from mild cognitive impairment (amnestic MCI, multiple domains) with stable memory deficits and a decline in verbal fluency and processing speed within a two-year interval after the first presentation in our memory clinic. Brain MRI showed brain damage in the right temporoparietal, frontolateral region and thalamus, as well as in the left posterior border of the capsula interna and white matter in the frontal region. Since the brain damage, she suffered paresis of the upper extremities on the left side and lower extremities on the right side as well as gait disturbance. Our search for autoantibodies revealed anti-AP3B2 autoantibodies in serum. Conclusions: Our report expands the spectrum of symptoms to mild cognitive impairment in addition to a gait disturbance associated with anti-AP3B2 autoantibodies. Furthermore, it is conceivable that a prior traumatic brain injury could initiate the development of anti-AP3B2-antibody-associated brain autoimmunity, reported here for the first time.

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Section: Discussionmentioning

confidence: 97%

Mild Cognitive Impairment in Chronic Brain Injury Associated with Serum Anti-AP3B2 Autoantibodies: Report and Literature Review

et al. 2021

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Section: Introductionmentioning

confidence: 99%

“…Anti-N-methyl-d-aspartate (Anti-NMDA) encephalitis is an autoimmune disorder with neurological and psychiatric features [ 1 ]. The incidence of the disease is 0.15 per 100,000 persons per year [ 1 ]. The condition affects both sexes, with a 4:1 ratio in favor of females, and it has a bimodal age distribution, with children (as young as eight months) and adults (20 to 40 years) being the most affected.…”

Section: Introductionmentioning

confidence: 99%

“…The condition affects both sexes, with a 4:1 ratio in favor of females, and it has a bimodal age distribution, with children (as young as eight months) and adults (20 to 40 years) being the most affected. [ 1 ]. Although patients tend to have a longer hospital stay, ICU admission, and hospital costs than in other encephalitides, they are more likely to have a full recovery or recovery with minor sequelae (75%) than severe CNS deficits or death (25%) [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…There are several phases in ANMDARE. It begins with a prodromal phase characterized by headache, fever, or a week-long viral-like process in 70% of patients [ 1 , 5 ]. The illness phase is characterized by psychiatric symptoms such as psychosis, agitation, hallucinations, mania, and seizures (the latter can appear at any time during the disease) [ 1 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Understanding Seizures and Prognosis of the Extreme Delta Brush Pattern in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Systematic Review

Parwani

Ortíz

Alli

et al. 2021

Cureus

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Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis (ANMDARE) is an autoimmune disorder with neurological and psychiatric features. The disease presents with a viral prodrome, followed by psychiatric manifestations. In the next phase, movement disorders or/and seizures occur. Finally, in the last phase, there is a decrease in the level of consciousness. Central hypoventilation and autonomic dysfunction can occur. Recently a unique EEG (electroencephalogram) pattern has been associated with anti-NMDA receptor encephalitis, the extreme delta brush (EDB). Although the association of the EDB with ANMDARE is known by the medical community, its significance is mainly unknown. A systematic review on NMDARE is also scarce. We decided to conduct a systematic review on this topic to consolidate the knowledge and establish the importance of the EDB as a prognostic factor. To conduct this systematic review, we used only studies conducted in humans, written in English, and published in the last 20 years. We used PubMed as a database and searched the following search terms: ("NMDA encephalitis"[Title/Abstract] AND "Epilepsy"[Title/Abstract]) OR (NMDA encephalitis"[Title/Abstract] AND "seizures" [Title/Abstract]) OR ("NMDA encephalitis"[Title/Abstract] AND "extreme delta brush"[Title/Abstract]). The protocol used for this systematic review was the Meta-analyses Of Observational Studies in Epidemiology (MOOSE) protocol, and to analyze the bias of the studies, we used the ROBINS-1 tool. Eight studies were collected from our search strategy. Our data pulling showed that seizures were present in 178/249 (71.48%) patients. Status Epilepticus was reported in 29/96 (30.20%), and the EBD was seen in 30.89% (55/178) patients with seizures. The range of EDB was 5.9%-33% among the studies. Because the sample size was small, the statistical power was decreased. We had a low overall risk of bias. The wide range in the results could be related to the timing of the EEG recording. EDB was associated overall with increased length of hospital stay, increased ICU admission, and incidence of status epilepticus. The etiology of the EDB remains mainly unknown. However, it has been postulated that in NMDAR encephalitis, there is a disruption of the rhythmic neuronal activity. When antibodies block/target the NMDAR, the rhythmic neuronal activity is disrupted, leading to the unique EDB pattern. Another theory suggests that delta activity is caused because of focal abnormalities in the brain, and the superimposition of the beta waves is related to the alterations of the NMDA receptors.

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Autoimmune encephalitis in a resource-limited public health setting: a case series analysis

Morillos,

Borelli,

Noll

et al. 2024

Arq Neuropsiquiatr

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Background Autoimmune encephalitis (AE) consists of a group of acquired diseases that affect the central nervous system. A myriad of phenotypes may be present at the onset. Due to the heterogeneity of clinical presentations, it is difficult to achieve uniformity for the diagnostic and therapeutic processes and follow-up strategies. Objective To describe a series of patients diagnosed with AE in a resource-limited public hospital in southern Brazil and to analyze therapeutics and outcomes. Methods We retrospectively reviewed the electronic medical records of patients diagnosed with AE at the Hospital de Clínicas de Porto Alegre from 2014 to 2022. Data collected included clinical presentation, neuroimaging, cerebrospinal fluid testings, electroencephalogram, autoantibodies, treatments, outcomes, follow-up time, degree of neurological impairment, and mortality. Results Data from 17 patients were retrieved. Eleven cases were classified as definite AE and 6 as possible AE. Autoantibodies were identified in 9 patients. Timing for diagnosis was impacted by the high costs associated with autoantibody testing. Most patients became functionally dependent (82.4%) and most survivors remained with autoimmune-associated epilepsy (75%). Five patients died during hospitalization, and one after a 26-month of follow-up. Conclusion In this resource-limited hospital, patients with AE had a worse clinical outcome than that previously described in the literature. Development of epilepsy during follow-up and mortality were greater, whilst functional outcome was inferior. Autoantibody testing was initially denied in most patients, which impacted the definitive diagnosis and the use of second-line therapies.

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Spectrum of anti-NMDA receptor antibody encephalitis: Clinical profile, management and outcomes

Cited by 7 publications

References 28 publications

Mild Cognitive Impairment in Chronic Brain Injury Associated with Serum Anti-AP3B2 Autoantibodies: Report and Literature Review

Mild Cognitive Impairment in Chronic Brain Injury Associated with Serum Anti-AP3B2 Autoantibodies: Report and Literature Review

Understanding Seizures and Prognosis of the Extreme Delta Brush Pattern in Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis: A Systematic Review

Autoimmune encephalitis in a resource-limited public health setting: a case series analysis

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