Movement disorders in autoimmune encephalitis: an update
Abstract: Autoimmune encephalitis (AE) is a form of encephalitis resulting from an immune response targeting central nervous system antigens, which is characterized by cognitive impairment, neuropsychiatric symptoms, seizures, movement disorders (MDs), and other encephalopathy symptoms. MDs frequently manifest throughout the progression of the disease, with recurrent involuntary movements leading to discomfort and, in some cases, necessitating admission to the intensive care unit. Prompt identification and management of MDs can aid in the diagnosis and prognosis of AE. This review synthesizes current knowledge on the characteristics, underlying mechanisms, and treatment options for MDs in the context of AE.
Sleep disorders and polysomnography findings in patients with autoimmune encephalitis
Sleep disorders in patients with autoimmune encephalitis (AE) are increasingly reported. Sleep disorders are frequent and essential components of AEs. Systematic clinical questionnaires and routine polysomnography (PSG) assessments would significantly impact the correct diagnosis and proper treatment of sleep-related breathing disorders (SRBD) and the overall prognosis of AE.
Psychometric deficits in autoimmune encephalitis: A retrospective study from the Australian Autoimmune Encephalitis Consortium
Deficits in psychometric markers of executive dysfunction were the most common finding in this cohort, followed by deficits on tasks sensitive to memory. A total of 54.2% of patients were classified as having psychometric impairments across at least two cognitive domains. Twenty-nine patterns were observed, suggesting outcomes in AE are complex. None of the demographic data, clinical features or auxiliary examination variables were predictors of psychometric outcome. Further detailed and standardized cognitive testing, in combination with magnetic resonance imaging volumetrics and serum/cerebrospinal fluid biomarkers, is required to provide rigorous assessments of disease outcomes.
Relapses of Anti-NMDAR, Anti-GABABR and Anti-LGI1 Encephalitis: A Retrospective Cohort Study
A total of 100 patients were enrolled. Relapse occurred in 26 (26%) patients after a median follow-up of 18 months since the first event. The relapse rates of anti – NMDAR, anti – GABABR and anti – LGI1 encephalitis were 25%, 33.3%, and 28.6%, respectively. The multivariable analysis results suggested that immunotherapy delay at the acute phase was independently associated with an increased risk of relapse in total patients (HR = 2.447, 95% CI = 1.027 – 5.832; P = 0.043). Subgroup analysis results showed that antibody titer was associated with the likelihood of relapse in anti-LGI1 encephalitis. The higher the concentration, the more likely it was for patients to have relapse (p=0.019).
The general relapse rate of anti-NMDAR, anti-GABABR and anti-LGI1 encephalitis was 26%. The risk of subsequent relapse was elevated in those with delayed immunotherapy in the first episode. In subgroup of anti-LGI1 encephalitis, higher antibody titer was the risk factors of relapse. Thus, timely and aggressive immunotherapy may be beneficial for patients to prevent subsequent relapse.
Autoantibody Encephalitis: Presentation, Diagnosis, and Management
Autoantibody encephalitis involves distinct clinical syndromes that can be recognized in many cases. In other patients, cancer screening and rational antibody testing can lead to a precise diagnosis. Our knowledge about the optimal treatments is still hampered by the lack of randomized treatment trials, but new information from retrospective studies has provided important new information on treatment of anti-LGI1 and anti-NMDAR syndromes. In selecting treatments it is important to understand the underlying disease mechanisms and the evidence supporting treatment. There will almost certainly be additional autoantibody syndromes discovered in the coming year. Each disease teaches us about the neuroscience of the target antigens. It is not reasonable to expect a general neurologist to memorize the associations of each new antibody. Rather, the goal should be to read and review the cancer associations and treatment guidance for a specific antibody when encountering a case.
Clinical Features, Treatment, and Prognostic Factors in Neuronal Surface Antibody-Mediated Severe Autoimmune Encephalitis
Patients with severe AE were in critical condition at baseline but could be salvaged after effective rescue immunotherapy. A lower dosage of rituximab could be an optimal option for severe AE. CD19+ B-cell count and NLR may provide prognostic information for predicting treatment response and outcome of severe AE.
Sleep macro- and micro-structure in autoimmune encephalitis: single case report from the subacute phase of the disease to the follow-up
Sleep disorders are frequently described in autoimmune encephalitis (AE); however, data on sleep texture are fragmentary. We analyzed the polysomnography of a woman affected by AE, and we performed cyclic alternating pattern (CAP) scoring during the subacute phase of the disease and at follow-up. The first polysomnography showed deviations both at macro and microstructure levels, with a marked reduction of CAP rate compare to healthy sleepers (20.8% vs 33%). After 6-months sleep macrostructure improved, whilst CAP parameters remained abnormal. This is the first polysomnographic analysis, comprehensive of microstructural data, performed in AE. We briefly discuss the results.
Neuro-Ophthalmic Features of Autoimmune Encephalitides
Several of the antibody-mediated encephalitides, specifically N-methyl-D-aspartate receptor, dipeptidyl-peptidase-like protein 6, glial fibrillary acidic protein, metabotropic glutamate receptor 1 (mGluR1), gamma-aminobutyric acid receptor, glutamic acid decarboxylase 65 (GAD65), collapsing response mediator protein 5 (CRMP5), and kelch-like protein 11 (KLHL11), contain features of neuro-ophthalmic interest.
The novel cell-surface protein-directed autoimmune encephalitis group can present with a wide range of afferent and efferent neuro-ophthalmic manifestations. Neuro-ophthalmologists should be familiar with these antibody-associated syndromes, which are treatable and often require a high index of suspicion for diagnosis.
Clinical features and outcome of patients with autoimmune cerebellar ataxia evaluated with the Scale for Assessment and Rating of Ataxias
Patients with idiopathic ACA significantly improved after immunotherapy. SARA score reflects well patients’ clinical status and may be a suitable outcome measure for patients with autoimmune cerebellar ataxia.
Autoimmune Encephalitis Resembling Dementia Syndromes
This nationwide observational cohort study evaluated cognitive characteristics in middle-aged or older patients with anti-LGI1, anti-NMDAR, anti-GABABR, and anti-CASPR2 encephalitis. We show that AIE can resemble dementia frequently, especially as rapidly progressive dementia (RPD). Ancillary testing can be misleading, lacking an inflammatory signature (in the CSF or on brain MRI), whereas the CSF biomarker profile that is often requested for dementia workup might mimic a neurodegenerative syndrome. Seizures are often present both early and late in the disease course. These can be very subtle and therefore easily overlooked.
Red flags for AE in patients with suspected dementia are: (1) rapidly progressive cognitive decline, (2) subtle seizures, and (3) abnormalities in ancillary testing atypical for neurodegeneration. Physicians should be aware that inflammatory changes are not always present in AIE, and that biomarkers often requested when dementia was suspected (including 14-3-3) can show abnormal results. Diagnosis is essential as most patients profit from immunotherapy.
Autoimmune Neurology The Need for Comprehensive Care
Autoimmune neurology is a rapidly developing subspecialty. Familiarity and awareness of different antibody-associated phenotypes can help to ensure prompt diagnosis and treatment. In cases that are less clear, a sound diagnostic approach anchored on objective clinical findings is important for optimizing outcomes. Clinical monitoring and treatment must expand beyond the acute care setting. A growing body of literature illustrates prolonged periods of recovery complicated by behavioral dysfunction and neuropsychiatric symptoms in the months to years after the diagnosis. These findings highlight the importance of comprehensive teams with expertise in autoimmune neurologic disorders. The ultimate goal is to provide optimal, long-term outpatient care coordinated among clinicians while anticipating the needs of the patients and caregivers.
Prospective Quantification of CSF Biomarkers in Antibody-Mediated Encephalitis
Objectives: To determine whether neuronal and neuroaxonal injury, neuroinflammation and synaptic dysfunction associate with clinical course and outcomes in antibody-mediated encephalitis (AME), we measured biomarkers of these processes in CSF from patients presenting with AME and cognitively normal individuals.
Conclusions: CSF biomarkers suggest that neuronal integrity is acutely maintained in AME patients, despite neuroaxonal compromise. Low-levels of biomarkers of synaptic function may reflect antibody-mediated internalization of cell-surface receptors, and may represent an acute correlate of antibody-mediated synaptic dysfunction, with the potential to inform disease severity and outcomes.
ECT in autoimmune encephalitis
In this case report, a 27-year-old male presented at a department of neurology with postherpetic anti-N-methyl-d-aspartate (NMDA) receptor autoimmune encephalitis. The patient was psychotic and exhibited symptoms of akinetic and excited catatonia. He was mechanical restrained for a total of 46 days due to violent behaviour. He was treated with olanzapine and lorazepam up to 15 mg/day without effect on catatonic and behavioural symptoms. ECT was initiated, and the patient received a total of 16 treatments. He responded well to the treatment, and the violent behaviour resolved completely after the first treatment. ECT should be considered for catatonia in anti-NMDA-receptor autoimmune encephalitis.
Antibody Negative Autoimmune Encephalitis: A Case Report
Gender issues of antibody-mediated diseases in neurology: (NMOSD/autoimmune encephalitis/MG)
Neuromyelitis optica spectrum disorder (NMOSD), autoimmune encephalitis (AE), myasthenia gravis (MG), and Lambert-Eaton myasthenic syndrome (LEMS) are antibody-mediated neurological diseases. They have mostly female predominance, affecting many women during childbearing age. Interactions between the underlying disease (or necessary treatment) and pregnancy can occur in every one of these illnesses. Herein, we present the characteristics of NMOSD, AE, MG, and LEMS in general, and review published data regarding the influence of the different diseases on fertility, pregnancy, puerperium, treatment strategy during pregnancy and post-partum period, and menopause but also male factors. We summarise key elements that should be borne in mind when confronted with such cases.
Search for Viral Infections in Cerebrospinal Fluid From Patients With Autoimmune Encephalitis
Autoantibodies against neuronal cells were detected in CSF from 8 individuals (4% of all encephalitis patients): 7 (3.5%) had anti-NMDAR and 1 (0.5%) had anti-GABA B. RT-PCR/PCR identified human herpes virus type 1 (HSV-1; 300 copies/mL) and the representative of Enterovirus genus (550 copies/mL) in 1 patient each. Torque teno virus (TTV) was found in another patient using metagenomic analysis, and its presence was confirmed by specific PCR.
We detected the presence of HSV, TTV, and Enterovirus genus in CSF samples from 3 out of 8 AE patients. These findings support the concept of viral involvement in the pathogenesis of this disease.
Imaging Review of Paraneoplastic Neurologic Syndromes
Paraneoplastic neurologic syndromes can cause a wide range of imaging abnormalities throughout the central and peripheral nervous systems. We have described the imaging findings of paraneoplastic limbic encephalitis, cerebellar degeneration, brain stem encephalitis, cranial neuropathy, myelitis, and polyneuropathy. Additionally, we have highlighted the fact that these entities can have variable imaging findings resulting in many potential diagnostic pitfalls. Because imaging features of each entity are sometimes nonspecific, consideration of paraneoplastic syndromes based on imaging findings combined with review of patient history are important.
Autoantibody-Mediated Encephalitis Differential diagnosis in patients with impaired consciousness of unclear origin
Results: The incidence of autoimmune encephalitis in Germany is estimated at 8–15 cases per million persons per year. In some patients with psychotic manifestations or impaired consciousness of acute or subacute onset, an autoimmune pathogenesis can be demonstrated by the laboratory detection of autoantibodies against neuronal target antigens (e.g., glutamate receptors). Testing of this type should be performed in patients with inflammatory changes in the cerebrospinal fluid or on magnetic resonance imaging (MRI), or those who have had an otherwise unexplained first epileptic seizure or status epilepticus. The cumulative sensitivity of testing for all potentially causative antineuronal antibodies in patients with clinically defined autoimmune encephalitis is estimated at 60–80 %. Figures on cumulative specificity are currently unavailable.
Autoimmune encephalitis and gastrointestinal dysmotility: achalasia, gastroparesis, and slow transit constipation
Neurological autoimmune disorders (NAD) are caused by autoimmune inflammation triggered by specific antibody subtypes. NAD may disturb the gut-brain axis at several levels including brain, spinal cord, peripheral, or enteric nervous system. NAD may disturb gastrointestinal motility by altering various levels of the gut-brain axis.
Neuronal Surface Antibody Syndrome: A Review of the Characteristics of the Disease and Its Association with Autoantibodies
This article reviews the pathogenesis, clinical manifestations, therapeutic approach, and other aspects of antibody-mediated autoimmune encephalitis in order to spread awareness about this disease.
Commentary: Epidemiology of Antibody-Positive Autoimmune Encephalitis in Southwest China: A Multicenter Study
This is a meaningful report providing epidemiological data about the antibody distribution in AE in the Chongqing area, along with revealing the factors associated with poor prognosis of AE. Despite some of the above-mentioned concerns, this study would be helpful to researchers and clinicians alike to gain more insight into AE.
It is crucial that clinicians have an awareness of the common patterns of presentation and investigation fi ndings to enable early treatment. Immunological testing strategies need to keep evolving to improve diagnosis and patient outcome. Where possible near-site testing of the more common antibodies is preferable. Prospective registries should inform clinicians on the incidence of false-positive results with different antibodies and assays
Open issues in antibody-mediated encephalitis
Once the diagnosis of NSAE is established, the choice of the best treatment option and its timing remains the most important open issue. The therapeutic approach is based on retrospective studies and expert opinion. Clinical trials are lacking or are very difficult to be completed. The advice provided is addressed to the clinician faced with a patient with NSAE, who must manage different issues in different phases of the disease.
Another major issue is represented by antibody-negative cases, considering that in world reference laboratories at least 7% of clinically definite LE are antibody negative. It is not surprising that antibody-negative AE, which are mainly diagnosed after exclusion of other disorders, tend to have a poorer prognosis because of delayed treatments
Searching for Autoimmune Encephalitis: Beware of Normal CSF
CSF WBC count and protein were within normal for 27% (CI 95%: 19-37) of patients with early active autoimmune encephalitis.
Sleep Disturbances in Patients With Autoimmune Encephalitis
Autoimmune encephalitis is an inflammatory brain disorder characterized by the subacute onset of psychiatric symptoms, cognitive impairment, and focal neurologic deficits or seizures. Sleep disturbances are detected in a majority of patients systematically screened for sleep complaints, may be the presenting symptom in patients with autoimmune encephalitis, and may compromise recovery in patients with autoimmune encephalitis. Early recognition of specific sleep disturbances in patients with subacute changes in behavior or cognition may support the diagnosis of autoimmune encephalitis. Similarly, recognition and treatment of sleep dysfunction in patients with known autoimmune encephalitis may speed recovery and improve long-term outcomes.
Utility of Brain Fluorodeoxyglucose PET in Children With Possible Autoimmune Encephalitis
We aimed to explore the utility and additional clinical contribution of brain fluorodeoxyglucose (FDG) PET imaging for the assessment of children with possible autoimmune encephalitis in comparison to brain MRI.
Conclusions: Our findings support the usage of fluorine-18-FDG PET/computed tomography (CT)/MRI with quantitative analysis early in the diagnostic work-up of possible autoimmune encephalitis, particularly in those with normal or nonspecific MRI findings. However, it remains a purpose of further studies, if and to what extent FDG PET/CT or integrated FDG PET/MRI with quantitative analysis can improve the diagnostic workup of children with possible autoimmune encephalitis.
Predisposing factors and prognosis of status epilepticus in patients with autoimmune encephalitis
The aim of this study was to study the predisposing factors and prognosis of status epilepticus (SE) in patients with autoimmune encephalitis (AE).A total of 227 cases of AE were collected. SE is a common complication in patients with AE. EEG and MRI abnormalities may be predisposing factors for SE. Glasgow scores <8 points, abnormal EEG, delayed immunotherapy, and SE duration lasting >30 minutes at admission are risk factors for a poor prognosis in patients with SE.
Th17 Lymphocytes Drive Vascular and Neuronal Deficits in a Mouse Model of Postinfectious Autoimmune Encephalitis
Basal ganglia encephalitis (BGE), representing a subset of AE syndromes, is triggered in children by repeated group A Streptococcus (GAS) infections that lead to neuropsychiatric symptoms.
Th17 lymphocytes have reduced BBB leakage, microglial activation, and antibody infiltration into the CNS, and have their olfactory function partially restored. Th17 lymphocytes are therefore critical for selective CNS entry of autoantibodies, microglial activation, and neural circuit impairment during postinfectious BGE.
Clinical Symptoms of Patients With Autoimmune Encephalitis: A Guide to Timely Recognition and Treatment
Autoimmune encephalitis is often treatable, and fast recognition and treatment are essential to prevent irreversible damage. Identification of patients with autoimmune encephalitis is challenging because patients display various symptoms and consequently present to different medical specialists. We describe 3 cases of autoimmune encephalitis due to different antibodies.
Minimal breast milk transfer of rituximab, a monoclonal antibody used in neurological conditions
In women with serious autoimmune neurologic conditions, monoclonal antibody therapy may afford an acceptable benefit to risk ratio, supporting both maternal treatment and breastfeeding.
Olfactory and Gustatory Dysfunction in patients with Autoimmune Encephalitis
To test the hypothesis that olfactory (OF) and gustatory function (GF) is disturbed in patients with autoimmune encephalitides (AE)
An evolving redefinition of autoimmune encephalitis
Atypical Autoimmune Encephalitis With Neuropil Antibodies Against a Yet Unknown Epitope
Autoimmune encephalitis often causes acute psychiatric symptoms and epileptic seizures. However, it is becoming increasingly clear that depending on the target antigen both symptoms and disease severity may vary.
Neuroimmunology – the past, present and future
Neuroimmunology encompasses fundamental and applied biology, immunology, chemistry, neurology, pathology, psychiatry and virology of the central nervous system (CNS). Scientists in the field study the interactions of the immune and nervous system during development, homeostasis and response to injuries with the major aim of developing approaches to treat or prevent neuroimmunological diseases.
Prognosticating autoimmune encephalitis: A systematic review
Clinical Features and Inflammatory Markers in Autoimmune Encephalitis Associated With Antibodies Against Neuronal Surface in Brazilian Patients
To the best of our knowledge, this is the largest cohort of patients with AE reported in Brazil.
Regulatory T cells in autoimmune disease
The prevalence and treatment outcomes of antineuronal antibody-positive patients admitted with first episode of psychosis
When the body attacks the brain: Immune system often to blame for encephalitis, study finds
AE - Neuroimmunology 2017: making progress over 20 years abstract
Medical Encyclopedia → Tardive dyskinesia
Clinical Signs for Identifying Autoimmune Encephalitis in Psychiatric Patients
The Emerging Field of Autoimmune Neurology
Psychosis: an autoimmune disease?
Postviral autoimmune encephalitis: manifestations in children and adults
Untangling Apparent Inconsistencies for Clinical Practice
A Survey of Treatment Progress and Prospects From Pediatric Neurologists
If you want to know about OCD: Neuroinflammation and psychiatric illness By: Dr. Najjar
Stem Cell - MS breakthrough: Replacing diseased immune system halts progression and allows repair
Autoimmune Encephalopathies and Dementias McKeon, Andrew MD
Discovery- Brain is directly connected to the immune system by vessels previously thought not to exist
CSF markers of neuronal and glial cell damage to monitor disease activity and predict long-term outcome in patients with AE
IRB Barcelona scientists on Promising development of new Drugs that cross the Blood Brain Barrier (BBB) to reach their Targets
Headache as a predictor for dementia: The HUNT Study
Scientists discover atomic-resolution details of brain signaling
This stunning discovery about the brain will have scientists rewriting textbooks
Neuroimmunology: an expanding frontier in autoimmunity
Woman Falls Suddenly Ill After Developing Autoimmune Encephalitis
Treating seizures and preventing amnesia in LGI1-antibody encephalitis A new MRI signature?
Scientists discover how to 'switch off' autoimmune diseases
Ovarian teratoma (dermoid cyst) and encephalitis: A link to keep on your radar
Myoclonus Fact Sheet
The Emerging Link Between Autoimmune Disorders and Neuropsychiatric Disease
Left shift: (meaning that there are more immature precursors present than you would normaly see)
Autoimmune and paraneoplastic neurological disorders: A review of relevant neuroimaging findings
• Overall, high-risk neuronal antibodies were associated with higher mortality and deep brain structures.
• Intermediate/low-risk neuronal antibodies were associated with lower mortality and more cortical involvement.
• Proper imaging evaluation is crucial to avoid delayed or missed diagnoses of paraneoplastic and autoimmune encephalitis.
Case Report: High-dose steroid and IVIG successful treatment in a case of COVID-19-associated autoimmune encephalitis: a literature review
Conclusion: despite limited data and lack of guidelines regarding the use of high-dose steroids and IVIG, this therapeutic approach may still prove beneficial for patients with COVID-19-associated autoimmune encephalitis. Consequently, we propose that the incorporation of a combined regimen of IVIG and high-dose corticosteroids could potentially prove advantageous for patients diagnosed with autoimmune encephalitis, particularly in instances where there is a concomitant COVID-19.
Autoimmune encephalitis in COVID-19 patients: a systematic review of case reports and case series
This systematic review comprehensively describes the characteristics of COVID-19-associated AE. The main type of COVID-19-associated AE identified in this study is an unknown Ab type of COVID-19-associated AE. Despite the potentially life-threatening risks of COVID-19-associated AE, the majority of patients survived, with some patients reporting residual neurological symptoms.
Autoimmune Encephalitis with Antibodies: Anti-NMDAR, Anti-AMPAR, Anti-GQ1b, Anti-DPPX, Anti-CASPR2, Anti-LGI1, Anti-RI, Anti-Yo, Anti-Hu, Anti-CV2 and Anti-GABAAR, in the Course of Psychoses, Neoplastic Diseases, and Paraneoplastic Syndromes
Encephalitis is a condition with a variety of etiologies, clinical presentations, and degrees of severity. The causes of these disorders include both neuroinfections and autoimmune diseases in which host antibodies are pathologically directed against self-antigens. In autoimmune encephalitis, autoantibodies are expressed in the central nervous system. The incidence of this disease is approximately 4% of all reported cases of encephalitis. Autoimmune encephalitis can be induced by antibodies against neuronal surface antigens such as N-methyl-D-aspartate-activated glutamate receptors (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazole propionate receptors (AMPAR) or gangliosides GQ1b, DPPX, CASPR2, LGI1, as well as by antibodies against neuronal intracellular antigens. The paper presents a number of both mental and neurological symptoms of autoimmune encephalitis. Moreover, the coexistence of psychoses, neoplastic diseases, and the methods of diagnosing autoimmune encephalitis are discussed. Attention was also drawn to the fact that early diagnosis, as well as early initiation of targeted treatment, increases the chance of a successful course of the therapeutic process.
Movement disorders in cell surface antibody mediated autoimmune encephalitis: a meta-analysis
Conclusion: Movement disorders are common in AE and their identification, in conjunction with other clinical and paraclinical features, may facilitate earlier diagnosis. The prognostic implications of movement disorders in AE warrant further dedicated study.
Ophthalmologic Manifestations of Paraneoplastic Syndromes
Families’ Experiences Living with Acquired Brain Injury: “Thinking Family”—A Nursing Pathway for Family-Centered Care
A narrative inquiry qualitative approach was utilized in this research project to capture family group stories about their experiences living with ABI. Narrative inquiry is a useful methodology for examining families affected by an ABI because of its ability to encapsulate how families make sense of their experiences living with ABI through the characteristics of meaning, relatedness, identity, and time. This is the first known study to incorporate narrative inquiry with family research and utilize the life-stage approach of Lieblich et al.
The master narrative themes were structured to replicate the chronological boundaries of the three life-stage chapters of the narrative interview process: (1) Before the ABI Event—Families: a grounding force, (2) Now Living with the ABI Event—a. losses individual and family, b. family adaptive capacities and, c. experiences with the healthcare system: hospital and home, and, (3) The Future—A patchwork future, entering the unknown.
Determining an infectious or autoimmune etiology in encephalitis
Objectives: Early presentation and workup for acute infectious (IE) and autoimmune encephalitis (AE) are similar. This study aims to identify routine laboratory markers at presentation that are associated with IE or AE.
From these findings, the association of presenting with fever, CSF WBC ≥50 cells/μL, and CSF protein ≥75 mg/dL was explored in ruling-out AE. When all three criteria are present, an AE was found to be highly unlikely (sensitivity 92%, specificity 75%, negative predictive value 95%, and positive predictive value 64%).
Seronegative limbic encephalitis in association with Sjögren's syndrome: a rare case report
Cases of autoimmune limbic encephalitis with no identifiable cause, serological screening for rheumatologic disorders is warranted. Sjögren syndrome though a rare aetiology for autoimmune limbic encephalitis, should not be missed out. It can make the difference between treatable and hopeless.
Development of a translational inflammation panel for the quantification of cerebrospinal fluid Pterin, Tryptophan-Kynurenine and Nitric oxide pathway metabolites
Recognising Inflammatory brain disorders provide a window to protect and prevent neuro disability- this will be a game changer. – Inflammation of the brain is increasingly recognised in acute neuroinflammatory diseases (such as encephalitis and multiple sclerosis), but also neurodevelopmental, neuropsychiatric and neurodegenerative processes. The immune response upon inflammation induces the initiation and alteration of a wide range of neuroactive metabolites. The analysis of cerebrospinal fluid (CSF) specimens holds promise in the diagnosis of neurological pathologies.
Paraneoplastic and Other Autoimmune Encephalitides: Antineuronal Antibodies, T Lymphocytes, and Questions of Pathogenesis
Paper discusses the emerging area of syndromes of autoimmune neuronal injury associated with immune checkpoint inhibitors and the implications current research may have on the treatment of affected patients.
Catatonia: Clinical Overview of the Diagnosis, Treatment, and Clinical Challenges
Catatonia is a complex condition with varying presentations and that is associated with multiple disorders, which can make recognition, diagnosis, and treatment a challenging process for healthcare professionals. Catatonic symptoms are often associated with various psychological and neurological disorders, including schizophrenia, neuroleptic malignant syndrome, and nodding syndrome. Patients with any of the symptoms of catatonia should be further evaluated for a catatonic syndrome secondary to their medical condition or independently of a psychiatric diagnosis. Although the etiology of catatonia is still unknown, diminished GABA signaling and abnormal OFC stimulation have been implicated in its pathophysiology.
The onset of catatonic symptoms and the presence of certain risk factors, such as elevated serum HVA, may help to predict whether a patient will respond to lorazepam. Benzodiazepines, specifically lorazepam, and ECT have been observed to be effective in treating acute catatonic symptoms associated with various mental disorders in both pediatric and adult populations. Early treatment of catatonia can reduce the risk of patients developing complications.
Antibody-mediated autoimmune encephalitis: A practical approach
AE is an umbrella term for a group of inflammatory central nervous system disorders associated with neuronal autoantibodies or other biomarkers of central nervous system autoimmunity.
Common clinical presentations include progressive neurocognitive symptoms with concomitant movement disorders, seizures, and autonomic dysfunction that worsens over weeks to months.
Objective clinical findings are needed to make the diagnosis of AE, including changes on magnetic resonance imaging, electroencephalography, and cerebrospinal fluid analysis.
- Paper contains an excellent review of the broad differential diagnosis
Seasonal variation in autoimmune encephalitis: A multi-center retrospective study
This study suggested summer-autumn predominance of the clinical onsets in patients with AE, especially anti-NMDAR and anti-LGI1 encephalitis. Therefore, clinicians should have a high index of suspicion for AE in encephalopathy patients in summer and autumn period.
Autoantibodies in neurological disease
Includes nice section of genetic contribution to antibody-mediated diseases
Antibody-mediated neurological diseases are a rapidly growing group of variable clinical entities with multifaceted manifestation and often profound response to treatment. The underlying autoantibodies directly confer pathogenicity by targeting single ion channels or receptors responsible for brain function. The diverse mechanisms of disease include antibody-mediated receptor internalization, complement activation, disrupted protein–protein interaction and signalling. The far-reaching clinical and scientific implications relate to emerging evidence that humoral autoimmunity participates in a much larger spectrum of neurological diseases than previously thought, ranging from encephalitis and psychosis to movement disorders, neurodegenerative diseases and neurodevelopmental abnormalities during pregnancy. Future research should now clarify the underlying molecular mechanisms of both neurological pathology and immunological dysfunction, including the role of incomplete B cell checkpoints, altered BCR repertoires, B cell homing, T cell help, affinity maturation and the homeostatic antibody repertoire in the brain.
Autoimmune movement disorders with neuronal antibodies – an update
Phosphodiesterase 10A antibodies are a new marker of paraneoplastic chorea. Seizure-related 6 homolog like 2 antibodies are a differential diagnosis in atypical parkinsonism with cerebellar ataxia and cognitive impairment. mGluR5-antibodies cause various hyperkinetic movement disorders with Ophelia syndrome. Most new antibodies were described in the context of cerebellar ataxia: Kelch-like protein 11 antibodies are a comparatively frequent marker of paraneoplastic cerebellar ataxia with germ cell tumours. Nonparaneoplastic cerebellar ataxia occurs with Septin-5 and neurochondrin antibodies. Studies into the mechanisms of neuronal surface antibodies have shown that there is much pathophysiological heterogeneity, ranging from immediate antagonistic effect to induction of neurodegeneration after weeks.
Myelitis and Other Autoimmune Myelopathies
Identifying the clinical and radiographic features of immune-mediated myelitis and recognizing mimics and pitfalls will help clinicians treat confirmed autoimmune myelitis appropriately.
Tics in patients with encephalitis
In addition to the established association between tics and encephalitis lethargica, our literature search identified reports of tics in patients with immune-mediated pathologies (including autoimmune encephalitides affecting the N-methyl-D-aspartate receptor, voltage-gated potassium channels, and glycine receptors) and infective processes (ranging from relatively common viral pathogens, such as herpes simplex, to prions, as in Creutzfeldt-Jakob disease). Tics were most commonly reported in the post-encephalitic period and involvement of the basal ganglia was frequently observed.
Sleep disorders in autoimmune encephalitis
Sleep disorders in people with autoimmune encephalitis have received little attention, probably overshadowed by the presence of other neurological and psychiatric symptoms in this group of conditions. However, sleep disorders are frequent, often severe, and usually persist beyond the acute disease stage, interfering with patients’ recovery and quality of life. Because autoimmune encephalitis can affect any brain network involved in sleep initiation and regulation, all types of sleep disorders can occur, with varying distinct associations, frequency, and intensity. Anti-IgLON5 and anti-NMDA receptor encephalitis exemplify two diseases in which sleep disorders are prominent
Comparisons Between Infectious and Autoimmune Encephalitis: Clinical Signs, Biochemistry, Blood Counts, and Imaging Findings
Objective: Infectious encephalitis (IE) and autoimmune encephalitis (AE) are symptomatically similar in clinic, however essentially different in pathogenesis. Therefore, the objective of this study was to identify specific features to distinguish the two types of encephalitis
for early effective diagnosis and treatments through a comparative analysis.
HLA-B27 association of autoimmune encephalitis induced by PD-L1 inhibitor
Significance of Autoantibodies in Autoimmune Encephalitis in Relation to Antigen Localization: An Outline of Frequently Reported Autoantibodies with a Non-Systematic Review
The recent discoveries of several autoantibodies produced in patients with autoimmune encephalomyelitis have expanded new clinical entities, such as autoimmune psychosis and autoimmune
epilepsy, and also provide a deep understanding of the background of neurological symptoms in these disorders, together with new insights into the basic neuroscience. For patients with psychotic disorders or intractable epilepsy previously treated in psychiatric wards, there is a
possibility for effective treatment through immunotherapy, such as intravenous methylprednisolone infusion, high-dose immunoglobulin administration, plasmapheresis, or other immunosuppressants.
Synaptic receptor dysfunction due to antibody binding causes various neurological symptoms of encephalopathy-associated pathologies.
Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France
We report on the epidemiologic features of PNS and AE diagnosed on the French territory and registered with the French National Reference Center between 2016 and 2018.
Conclusions There was a significant widespread year-to-year increase in the incidence of diagnoses registered with the Reference Center for all subgroups of PNS and AE studied. The national observed incidence rate is likely underestimated due to underdiagnosis and underreporting.
Cognitive impact of neuronal antibodies: encephalitis and beyond
In this review, we describe the cognitive impairment associated with each antibody-mediated syndrome and, using evidence from imaging and animal studies, examine how the nature of the impairment relates to the underlying neuroimmunological and receptor-based mechanisms.
We discuss evidence highlighting their prevalence, and association with cognitive outcomes, in a number of common disorders including cancer and schizophrenia. We consider mechanisms, including blood-brain barrier dysfunction, which could determine the impact of these antibodies outside encephalitis and account for much of the clinical heterogeneity observed.
18F-FDG-PET Imaging Patterns in Autoimmune Encephalitis: Impact of Image Analysis on the Results
Open Access link to .pdf
Conclusions: For the evaluation of patients with suspected AE, standard analysis of FDG-PET images benefits from voxel-based analysis, as it may lead to more comparable and accurate results. This study provides new evidence of the utility of FDG-PET for AE beyond the approach based on MRI, CSF sampling and EEG. Patients with AE may benefit from prompt diagnosis when brain FDG-PET is added to the traditional complementary tests.
Antibody-related movement disorders – a comprehensive review of phenotype-autoantibody correlations and a guide to testing
Over the past decade increasing scientific progress in the field of autoantibody–mediated neurological diseases was achieved. Movement disorders are a frequent and often prominent feature in such diseases which are potentially treatable.