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Neuropsychological Performance in Autoimmune Limbic Encephalitis: Evidence from an Immunotherapy-Naïve Cohort

This study provides a detailed clinical neuropsychological presentation of autoimmune limbic encephalitis (ALE) in immunotherapy-naïve patients. It confirms that memory impairments and symptoms of depression form hallmarks of ALE. It also provides further and new insights: (i) Memory impairment predominantly pertains to a consolidation impairment for new information resulting in long-term memory and recognition deficits. (ii) Short-term and working memory can be impaired, but usually only at a subclinical level. (iii) Recall of retrograde autobiographical episodes can be affected and (iv) ALE-patients suffer from deficits in emotion recognition especially regarding fear. In addition, we found (v) apraxia in pantomimes and imitation of gestures, which has not yet been described in ALE-patients. Further deficits may include impaired visuo-construction, processing speed, and flexibility. However, the performances between patients vary considerably.

Taken together, neuropsychological deficits presented in our cohort indicate possible involvement of brain networks outside the limbic system in ALE.

Neuropsychological assessment for diagnosing ALE should include long-term memory, memory recognition, autobiographical-episodic memory, emotion recognition, and a detailed investigation of symptoms of depression.

Distinct movement disorders in contactin-associated-protein-like-2 antibody associated autoimmune encephalitis

This international, retrospective cohort study included patients with CASPR2 autoimmunity from participating expert centers in Europe. Patients with ataxia and/or movement disorders were analyzed in detail using questionnaires and video recordings. 

We observed distinct movement disorders in CASPR2 autoimmunity (14.6%): episodic ataxia (6.7%), paroxysmal orthostatic segmental myoclonus of the legs (3.7%) and continuous segmental spinal myoclonus (4.3%). These occurred together with further associated symptoms or signs suggestive of CASPR2 autoimmunity. However, 2/164 patients (1.2%) had isolated segmental spinal myoclonus. Movement disorders and ataxia are highly prevalent in CASPR2 autoimmunity. Paroxysmal orthostatic segmental myoclonus of the legs is a novel albeit rare manifestation. Further distinct movement disorders include isolated and combined segmental spinal myoclonus and autoimmune episodic ataxia.

Seizure underreporting in LGI1 and CASPR2 antibody encephalitis

Out of 32 patients with LGI1 (n = 24) and CASPR2 (n = 8) encephalitis, 20 (15 LGI1, 5 CASPR2) fulfilled the inclusion criteria. Twelve patients were excluded because of ongoing seizures (n = 3) or lack of video-EEG in the follow-up (n = 9). The average age was 62.5 ± 11.04 years (mean ± SD; range = 41–82 years) at disease onset, and 13 of the 20 patients were male (Table 1). The average reported seizure-free interval at the last follow-up was 24.2 ± 20.91 months (mean ± SD) and ranged from 3 to 76 months (Figure 1). Seizures persisted for an average of 21.3 ± 22.39 months (±SD; range = 1–96 months) before seizure freedom was achieved. All patients were taking ASM at the time of video-EEG and received first-line immunosuppressive therapy. We recorded seizures in four of 20 eligible patients (20%) during 24–48 h of video-EEG, although they previously reported seizure freedom. The reported seizure-free interval (before the recorded seizure) in these patients ranged between 3 and 27 months. In two patients focal impaired awareness seizures (after 18 and 27 months of patient-reported seizure freedom) were recorded, and in two other patients focal aware seizures (after 3 and 4 months of patient-reported seizure freedom) were recorded. The two patients who had suffered focal impaired awareness seizures had not noticed their seizures. In one of these patients, the seizure was recorded out of sleep. One of the patients with focal aware seizures did not interpret the seizure as such, and the other patient with the focal aware seizure had a habitual aura. The four patients with seizure relapse did not show other clinical signs of relapse of the autoimmune encephalitis, such as decline in the neuropsychological performance, behavior changes, new seizure semiology, or new findings in the cerebral magnetic resonance imaging.

Case Report: Isolated Epileptic Seizures Associated With Anti-LGI1 Antibodies in a 7-Year-Old Girl With Literature ReviewGI1 antibodies

We describe the case of a 7-year-old girl with anti-leucine-rich glioma-inactivated 1 (anti-LGI1) antibodies (Abs) who presented with isolated epileptic seizures. Her refractory focal seizures did not respond to anti-seizure medicines but responded rapidly to immunotherapy. She remained seizure-free at 2 years follow-up. Reviewing the literature, isolated epileptic seizures have not been reported as the phenotype of anti-LGI1 autoimmunity in children. Our study indicated that screening for anti-LGI1 Abs is necessary for children with severe and/or drug-resistant new-onset focal epileptic seizures.

Long-term evolution and prognostic factors of epilepsy in limbic encephalitis with LGI1 antibodies

Significance: In patients with LGI1 encephalitis, rapid initiation of immunomodulatory treatment favors long-term epilepsy remission. Evolution to drug-resistant epilepsy drug-resistant epilepsy (DRE). DRE might primarily reflect the anatomical lesion of limbic structures. Determining what modalities of immune treatment may alter these outcomes requires prospective studies with long-term follow-up. 

Faciobrachial Dystonic Seizures as a Sign of Relapse in a Child with LGI-1 Encephalitis

LGI1-antibody encephalitis is rare in children. To the best of our knowledge, we describe the first pediatric case with FBDS and highlight the importance of early recognition. Like in adults, relapse can occur in children, but is very rare. Prompt treatment with rituximab can accelerate recovery and prevent relapse. Larger studies are needed in children to better define the clinical spectrum of this condition.

Whole-brain metabolic pattern analysis in patients with anti-LGI1 encephalitis

Faciobrachial dystonic seizures (FBDS) and hyponatraemia are the distinct clinical features of LGI1 autoimmune encephalitis.
Whole-brain metabolic pattern analysis was performed to assess the pathological network of anti-LGI1 AE, as well as the symptomatic networks of FBDS and to investigate the hyponatraemia-associated brain network and its effect on serum sodium levels.
 
Results: The pathological network of anti-LGI1 AE involved a hypermetabolism in cerebellum, subcortical structures, and Rolandic area, as well as a hypometabolism in the medial prefrontal cortex. The symptomatic network of FBDS shown a hypometabolism in cerebellum and Rolandic area (PFDR < 0.05). Hypometabolism in the cerebellum was an independent predictor of FBDS (P < 0.001). Hyponatraemia-associated network highlighted a negative effect on caudate nucleus, frontal and temporal white matter. Serum sodium level had the negative trend with metabolism of hypothalamus (Pearson’s R = -0.180, P = 0.342) but the mediation was not detected (path c’ = -7.238, 95% CI = -30.947 to 16.472).

Contemporary advances in antibody-mediated encephalitis: anti-LGI1 and anti-Caspr2 antibody (Ab)-mediated encephalitides

Anti-LGI1-, and anti-CASPR2 Ab-mediated encephalitis have benefited from a surge in knowledge with respect to pathobiology, diagnosis and management. Incipient characterisation of disease molecular and neuropathology, and genetic underpinnings represent recent advances in the field. Questions remain however, such as the respective role of T-cells, given the varying HLA class-II associations, and the significance thereof in light of clinical similarities. Both diseases are commonly characterised

Human CASPR2 Antibodies Reversibly Alter Memory and the CASPR2 Protein Complex

IgG from patients with anti-CASPR2 encephalitis causes reversible memory impairment, inhibits the interaction of CASPR2/TAG1, and decreases the levels of CASPR2 and related proteins (VGKC, AMPAR). These findings fulfill the postulates of antibody-mediated disease and provide a biological basis for antibody-removing treatment approaches.

LGI1 antibody encephalitis: acute treatment comparisons and outcome

Retrospective case series of 118 patients with LGI1 antibody encephalitis evaluated at Mayo Clinic across all US sites from 1 May 2008 to 31 March 2019. Corticosteroids appeared more effective acutely than IVIg in improving LGI1 antibody encephalitis in this retrospective comparison of immunotherapies. While improvement with immunotherapy is typical and long-term outcome is favourable, short-term memory deficits are noted in approximately a third of the patients.

Neuropsychological Performance in Autoimmune Limbic Encephalitis: Evidence from an Immunotherapy-Naïve Cohort

Comprehensive presentation of neuropsychological performance of Autoimmune limbic encephalitis (ALE) in an immune therapy-naïve sample. 

ALE shows a wide spectrum of neuropsychological impairments, which might exceed the limbic system, with no evidence of differences between autoantibodies (AABs) groups. Neuropsychological assessment for diagnosing ALE should include long-term memory, memory recognition, autobiographical-episodic memory, emotion recognition, and a detailed investigation of depression.

Neuropsychological implication in possible antibody-negative limbic encephalitis: a clinical case report

This current case report describes a patient with seronegative LE that presented 2 months after the onset of the disease with symptoms of severe autobiographical amnesia and a deficit in emotional face recognition.

In conclusion, the seronegative variant of LE still represents a significant challenge for achieving a correct diagnosis. New and more sensitive techniques to find antibodies other than the ones previously described could improve the knowledge and prognosis of this clinical condition. In addition, early treatment of symptoms could minimize cognitive dysfunction in the long term.

Clinical characteristics, long-term functional outcomes and relapse of anti-LGI1/Caspr2 encephalitis: a prospective cohort study in Western China

The clinical characteristics such as age, gender, and tumor occurrence rates of anti-LGI1 encephalitis and anti-Caspr2 encephalitis in western China are different from those in the Western countries. Most patients in our study had favorable long-term functional outcomes. The relapse rates are still high in both types of encephalitis, which warrants caution.

Clinical Features, Immunotherapy, and Outcomes of Anti-Leucine-Rich Glioma-Inactivated-1 Encephalitis

Results: The main clinical manifestations included epilepsy (100%), faciobrachial dystonic seizures (FBDS; 44.2%), cognitive dysfunction (95.3%), neuropsychiatric disturbances (76.7%), sleep disorders (58.1%), and disturbance of consciousness (48.8%). Twenty-two patients (51.2%) had hyponatremia, 31 (72.1%) had abnormal EEG results, and 30 (69.8%) had abnormal brain MRI scans, mainly involving the hippocampus (76.7%) or temporal lobe (40%). Twenty of 34 patients (58.8%) in a follow-up MRI examination exhibited hippocampal atrophy. Twenty-five patients (58.2%) were administered corticosteroids and intravenous immunoglobulin, whereas 17 patients were treated only with corticosteroids. Forty-one patients (95.3%) had favorable outcomes after a median of 21.5 months (IQR: 7-43) of follow-up. Serum sodium level was a factor associated with a disabled status (odds ratio=0.81, 95% CI=0.66, 0.98, p=0.03). Anti-LGI1 encephalitis patients were characterized by seizures, FBDS, cognitive deficits, neuropsychiatric disturbances, and hyponatremia.

Conclusions: Most patients with anti-LGI1 encephalitis are nonparaneoplastic, have low recurrence rates, and have favorable prognostic outcomes. Rapid evaluation, prompt immunotherapy, and long-term follow-up are essential in the care of anti-LGI1 encephalitis patients.

Role of LGI1 protein in synaptic transmission: From physiology to pathology

In this review, we will focus on LGI1 binding partners, “A Disintegrin And Metalloprotease (ADAM) 22 and 23”, the complex they form at the synapse, and will discuss the effects of LGI1 on neuronal excitability and synaptic transmission in physiological and pathological conditions. Finally, we will highlight new insights regarding N-terminal Leucine-Rich Repeat (LRR) domain and C-terminal Epitempin repeat (EPTP) domain and their potentially distinct role in LGI1 function.

Subcortical Hypermetabolism Associated With Cortical Hypometabolism Is a Common Metabolic Pattern in Patients With Anti-Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis

Brain 18F-fluorodeoxyglucose positron emission tomography (FDG PET) is a sensitive technique for assisting in the diagnosis of patients with anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis. The purpose of this study aimed to explore the glucose metabolic patterns of this disorder based on PET voxel analysis.This retrospective study enrolled 25 patients with anti-LGI1 encephalitis.

Conclusion

Subcortical hypermetabolism associated with cortical hypometabolism presented with a common metabolic pattern in patients with anti-LGI1 encephalitis in the present study. The resolution of the metabolic gradient of the hippocampal hypermetabolism and neocortical hypometabolism may bring about improved clinical neurologic disability.

Autoimmune Limbic Encephalitis: A Review of Clinicoradiological Features and the Challenges of Diagnosis

Autoimmune limbic encephalitis is a challenging diagnosis for several reasons. The clinical presentation can mimic various other diseases and, therefore, the differential diagnosis is large. Additionally, radiological features are frequently absent or non-specific in the more common subtypes. Being aware of the ways in which LE can present allows it to be considered as a diagnostic possibility in undifferentiated neuro-psychiatric presentations, which in turn improves treatment outcomes. Ongoing research into the pathogenesis and application of novel radiological techniques will assist in further characterizing these conditions in the future.

Autoimmune Limbic Encephalitis: A Review of Clinicoradiological Features and the Challenges of Diagnosis

Autoimmune limbic encephalitis is a challenging diagnosis for several reasons. The clinical presentation can mimic various other diseases and, therefore, the differential diagnosis is large. Additionally, radiological features are frequently absent or non-specific in the more common subtypes. Being aware of the ways in which LE can present allows it to be considered as a diagnostic possibility in undifferentiated neuro-psychiatric presentations, which in turn improves treatment outcomes. Ongoing research into the pathogenesis and application of novel radiological techniques will assist in further characterizing these conditions in the future.

Leucine-Rich Glioma-Inactivated 1 versus Contactin-Associated Protein-like 2 Antibody Neuropathic Pain: Clinical and Biological Comparisons

This study shows neuropathic pain (NP) as a frequent and often rapidly immunotherapy-responsive feature in a large cohort of patients with LGI1 and CASPR2 antibodies, including ~10% with relatively isolated pain syndromes.
7 main points:
1. Study inspired by a patient with LGI1-Abs, HLA-DRB1*07:01 & isolated episodic NP who responded remarkably rapidly to steroids
2. From the patients, NP is more common with CASPR2- than LGI1-Abs
3. In both, pattern=length dependent but trunk  only involved with LGI1-Abs
4. LGI1-Ab patientss responded far quicker to immunotherapy vs conventional analgesia.
5. The CASPR2-Ab pts showed limited response with persistent QOL in multiple domains.
6. The CASPR2-Abs bind human sensory nerve iPSCs & rodent DRGs. LGI1-Abs don’t.
 7. Both show decreased skin IENFD
Conclusions: NP is common in these patients; sometimes an isolated feature; some NP responds well to immunotherapy but the CASPR2-Ab patients have unmet needs. The pathophysiology appears to be a small fibre neuropathy but where do the LGI1-Abs bind? Is a question researchers are asking.

LGI1- versus CASPR2-antibody neuropathic pain: clinical and biological comparisons

Pain is a recognized association with LGI1-/ CASPR2-antibodies. Of 147 patients, pain was experienced by 17/33 (52%) CASPR2- versus 20/108 (19%) LGI1-antibody patients (p=0.0005), and identified as neuropathic in 89% versus 58% of these, respectively. Typically, normal nerve conduction studies and reduced intraepidermal nerve fibre densities were observed in subsets of both cohorts. In LGI1-antibody patients, pain responded to immunotherapy (p=0.008), often rapidly, with greater residual patient-rated impairment observed in CASPR2-antibody patients (p=0.019). Serum CASPR2-antibodies, but not LGI1-antibodies, bound in vitro to unmyelinated human sensory neurons and rodent dorsal root ganglia, suggesting pathophysiological differences which may underlie clinical observations.

Neurofunctional outcomes in patients with anti-leucine-rich glioma inactivated 1 encephalitis

Objective: To evaluate the cognitive and neurofunctional outcomes in patients with (LGI1) encephalitis.

Results: The results showed that 81 of 86 (94.2%) patients with anti-LGI1 encephalitis were successfully followed up, while eight (9.9%) died after discharge. Among the 73 survivors, clinical relapses occurred in 18 (24.7%) patients, and those with relapses were at a higher risk of developing remote symptomatic seizure (p = .019). Although 85.2% of the patients became functionally independent (mRS ≤2), the sequelae of symptomatic seizure, neuropsychiatric symptoms, and cognitive deficits were found in 11.0%, 21.9%, and 39.7% of the patients, respectively. Residual cognitive deficits primarily occurred in the elderly subjects as well as those with symptoms of memory deficit, psychiatric disorders, sleep disturbance, disturbance of consciousness at diagnosis, and higher CSF protein levels.

Conclusions: Although most patients survived and became functionally independent, a subset of patients could not return to all premorbid activities. They may have clinical relapses or suffer from remote symptomatic seizure, neuropsychiatric symptoms, and cognitive impairment.

Clinical Profile and Treatment Response in Patients with CASPR2 Antibody-Associated Neurological Disease

Clinical, radiological, electrophysiological, treatment, follow-up, and outcome data were collected by retrospective chart review.

The other seven patients were followed up for a mean duration of 19.71 months. All of them improved completely. Relapse occurred in one patient after 13 months but responded favorably to steroids.

Conclusion: CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.

Residual Fatigue and Cognitive Deficits in Patients After Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis

Fatigue is a major residual feature in LGI1 antibody patients. Inspired by seeing >100 of these patients, and hearing their stories.
 
Leucine-rich glioma-inactivated 1 antibody encephalitis (LGI1-Ab-E) typically affects older men who present with prominent amnesia and frequent seizures1-3 and often shows a marked short-term improvement with immunotherapies.
In particular, seizure cessation occurs within just a few weeks.
However, only traditional cognitive domains have been investigated as longer-term outcomes, with improvements in cognition described as “not good enough.” Here, motivated by
patient feedback and our clinical observations, we aimed to
quantify the residual deficits observed after LGI1-Ab-E across
several functional domains.
The data here indicate that despite a “good” mRS, several long-term residual deficits remain: across domains of cognition,
mood, and fatigue, with a significant effect on employment status. Our cohort’s mean mRS was comparable with other
LGI1-Ab-E studies, suggesting this traditional outcome measure captures only limited long-term morbidity in multiple
studies. Fatigue was the most commonly impaired domain in
our cohort, a novel finding in LGI1-Ab-E. This observation is closely reflected by the many patients in our clinic who volunteer fatigue as a major residual symptom. Also, it parallels findings in pediatric N-methyl-D-aspartate receptor antibody encephalitis, where fatigue is associated with quality of life.

Flortaucipir (tau) PET in LGI1 antibody encephalitis

Increased [18F]flortaucipir PET retention was observed in the two LGI1 antibody encephalitis patients with hippocampal atrophy and persistent cognitive impairment, including one patient with autopsy‐proven AD and moderate‐stage neurofibrillary pathology. Inflammation associated with autoimmune encephalitis may accelerate the accrual of tau neuropathology in susceptible individuals and may associate with hippocampal atrophy and persistent cognitive complaints.

LGI1 Antibody Encephalitis: Treatments and Long-term Outcome

Corticosteroids appeared to be more effective acutely than IVIG in improving LGI1 antibody encephalitis in this retrospective comparison of immunotherapies. While improvement with immunotherapy is frequent, most patients have residual long-term memory deficits.

Specific B- and T-cell populations are associated with cognition in patients with epilepsy and antibody positive and negative suspected limbic encephalitis

Objective: Neuropsychological impairments are major symptoms of autoimmune limbic encephalitis (LE) epilepsy patients. Here, we evaluated whether T- and B-cells are linked to cognitive impairment in these groups.

Conclusion: Our results indicate a link between T- and B-cell activity and cognition in epilepsy patients with suspected limbic encephalitis, thus suggesting that flow cytometry results can provide an understanding of cognitive impairment in LE patients with autoantibodies against intracellular antigens.

Sleep Disorders in Leucine-Rich Glioma-Inactivated Protein 1 and Contactin Protein-Like 2 Antibody-Associated Diseases

Objective: Sleep disorders are common in voltage-gated potassium channel complex antibody (VGKC-Ab) diseases. The aim was to investigate the sleep disturbances and polysomnography (PSG) characteristics in patients with VGKC-Ab-associated diseases.

Methods: Twenty-seven patients with leucine-rich glioma-inactivated protein 1 antibody (LGI1-Ab) encephalitis, seven patients with contactin protein-like 2 antibody (Caspr2-Ab)-associated diseases, and 14 healthy controls.

Conclusion: Sleep disorders in patients with VGKC-Ab-associated diseases include decreased total sleep time (TST)and poor sleep efficiency. Our studies provide evidence of status dissociatus (SD) in patients with LGI1-Ab encephalitis.

Limbic encephalitis and Post-Acute neuropsychology rehabilitation: A review and case examples

Rehabilitation research on limbic encephalitis (LE), a rare disorder characterized by inflammation of the brain caused by autoimmunity or infection, has focused on acute rather than post-acute management of symptoms. The frequency of clinical relapse in encephalitis ranges from 12% to 35%. Commonly, individuals diagnosed with LE experience relapses or breakthrough seizures during their post-acute treatment. The treating neuropsychologist will often need to do family and staff education regarding the disorder, a possible unusual pattern of recovery, and discuss the risk of relapse. Additionally, staff may need help with behavior management and the potential need for a behavior management plan.

Relapse involving need for re-admission to an acute setting occurred in four cases and medication side effects (sedation) in three. As a whole, these cases highlight the complex and potentially unusual course of recovery in individuals with LE.

Stop testing for autoantibodies to the VGKC-complex: only request LGI1 and CASPR2

(VGKC) antibodies that lack LGI1 or CASPR2 reactivities (‘double-negative’) are common in healthy controls and have no consistent associations with distinct syndromes. These antibodies target intracellular epitopes and lack pathogenic potential. Moreover, the clinically important LGI1 and CASPR2 antibodies comprise only ~15% of VGKC-positive results, meaning that most VGKC-antibody positive results mislead rather than help. Further, initial VGKC testing misses some cases that have LGI1 and CASPR2 antibodies. These collective observations confirm that laboratories should stop testing for VGKC antibodies and instead, test only for LGI1 and CASPR2 antibodies. This change in practice will lead to significant patient benefit.

Double-negative VGKC antibodies usually target intracellular epitopes and lack pathogenic potential. The VGKC antibody titre is not a clinically reliable measure. The concept of a ‘clinically relevant’ VGKC antibody titre has created many misdiagnoses, often where finding the antibody has overruled the clinical diagnosis. The evidence from multiple international groups suggests there are no longer clinical reasons to test for VGKC antibodies. Stopping this test will reduce clinically irrelevant results, improve diagnostic accuracy and limit the use of unnecessary, potentially toxic, immunotherapies in patients.

The Clinical Value of 18 F-FDG-PET in Autoimmune Encephalitis Associated With LGI1 Antibody

The initial 18F-FDG-PET scan indicated a significant abnormal metabolic pattern in 31 LGI1 AE patients (91%), whereas only 20 patients (59%) showed an abnormal MRI. The 18F-FDG-PET metabolic pattern was reversible after treatment; most of the patients showed an almost normal uptake of 18F-FDG-PET after discharge.

Regarding the abnormal metabolic pattern in LGI1 AE subjects exhibiting hypermetabolism was specifically located in the basal ganglia (BG) and medial temporal lobe (MTL). BG hypermetabolism was observed in 28 subjects (82%), and 68% of patients showed MTL hypermetabolism. A total of 17 patients (50%) exhibited faciobrachial dystonic seizures (FBDS), and the remaining subjects showed non-FBDS symptoms (50 and 50%). BG-only hypermetabolism was detected in seven subjects in the FBDS subgroup (7/16) but in only one subject in the non-FBDS subgroup

Psychiatric Manifestation of Anti-LGI1 Encephalitis

Conclusion: This case study describes a patient with anti-LGI1 encephalitis in the chronified stage and a predominant long-lasting psychiatric course with atypical symptoms of psychosis and typical neurocognitive deficits. The patient’s poor response to anti-inflammatory drugs was probably due to the delayed start of treatment. This delay in diagnosis and treatment may also have led to the FDG-PET findings, which were compatible with frontotemporal dementia (“state of damage”). In similar future cases, newly occurring epileptic seizures associated with psychiatric symptoms should trigger investigations for possible autoimmune encephalitis, even in patients with addiction or other pre-existing psychiatric conditions. This should in turn result in rapid organic clarification and-in positive cases-to anti-inflammatory treatment. Early treatment of anti-LGI1 encephalitis during the “inflammatory activity state” is crucial for overall prognosis and may avoid the development of dementia in some cases.

Distinctive binding properties of human monoclonal LGI1 autoantibodies determine pathogenic mechanisms

Overall, in this study, focused molecular hypotheses have been accurately dissected via the generation of patient-derived mAbs. The findings have highlighted intriguing aspects of the immunology and neuroscience, which inform multiple mechanisms underlying this highly amnesic and epileptogenic disease. While several mechanisms remain to be explored, these findings provide insights into the potential of mAbs to inform many aspects of the underlying disease biology which could not easily be highlighted by the study of polyclonal sera and CSFs.

IVIG Shows Efficacy for Autoimmune Encephalitis and Epilepsy

Intravenous immunoglobulin (IVIG) is an effective acute treatment strategy for reducing seizures in patients with seizures tied to two rare types of antibody-associated encephalitis, LGI1, and CASPR2, according to results from a small, double-blinded randomized, placebo-controlled trial featured as part of the 2020 AAN Science Advances.

Limbic encephalitis in a neuroscientist: CASPR 2 antibody-associated disease after antigen exposure

Autoimmune limbic encephalitis is part of CASPR 2 antibody-associated disease. A man with this rare disorder and a very high antibody titre had a unique history of laboratory exposure to the antigen. Together with earlier observations this case calls for caution in laboratory handling of nerve tissue.

Pilomotor Seizures in a Patient With LGI1 Encephalitis

The majority of these patients present faciobrachial dystonic seizures (FBDS), which are regarded as a characteristic symptom. A few cases have reported pilomotor seizures as the main manifestation of anti-LGI1 encephalitis. Here, we described a Chinese woman with frequent pilomotor seizures who was finally diagnosed as having anti-LGI1 encephalitis. Our report emphasizes the possible significance of pilomotor seizures in anti-LGI1 encephalitis.

Intrathecal B-cell activation in LGI1 antibody encephalitis

Our findings suggest that plasmablasts and plasma cells (which do not express CD20) are active in the CSF, and therefore, novel drugs that actively target these prolific protein synthesizers in the CNS compartment might be more effective than selective anti-CD20 treatments. Conversely, given that affinity maturation is an ongoing process in LGI1 antibody encephalitis and could result in increasingly efficient pathogenic antibodies, therapeutic depletion of CD20-expressing B-cell populations early in this disease might prevent development of chronic or progressive clinical phenotypes. Accordingly, early diagnosis of intrathecal immune activation in patients with mild clinical signs would be highly desirable.

 

 

Psychosis associated to CASPR2 autoantibodies and ovarian teratoma: A case report

 

First report of CASPR2 associated psychosis related to an ovarian teratoma. Prompt reduction of psychotic symptoms after excision of teratoma. The diagnosis of autoimmune psychosis can be made in the presence of autoantibodies in the serum while CSF is unremarkable.

Patients with depressive and psychotic symptoms non-responding to antidepressant and antipsychotic treatment should be screened for neuronal autoantibodies including CASPR2.

 

 

Daratumumab treatment for therapy-refractory anti-CASPR2 encephalitis

The anti-CD38 antibody daratumumab is approved for treatment of refractory multiple myeloma and acts by depletion of plasma cells and modification of various T-cell functions. Its safety, immunological effects and therapeutic potential was evaluated in a 60-year old patient with life-threatening and treatment-refractory anti-CASPR2 encephalitis requiring medical care and artificial ventilation in an intensive care unit.

Predictors of neural-specific autoantibodies and immunotherapy response in patients with cognitive dysfunction.

Recent studies have validated the use of Antibody-Prevalence-in-Epilepsy (APE) and Responsive-to-immunotherapy-in-Epilepsy (RITE) scores in the evaluation and management of autoimmune-epilepsy.

Factors underlying the development of chronic temporal lobe epilepsy in autoimmune encephalitis

LGI1 expression and human brain asymmetry

Distinction between anti-VGKC-complex seropositive patients with and without anti-LGI1/CASPR2 antibodies

Non-inheritable predisposition to LGI1 and Caspr2 antibody diseases

Neuroimmunology 2017: making progress over 20 years

Psychiatric symptoms delay the diagnosis of anti-LGI1 encephalitis

Mechanisms of Caspr2 antibodies in autoimmune encephalitis and neuromyotonia

The importance of early immunotherapy in patients with faciobrachial dystonic seizures (LGI1 Limbic encephalitis)

LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes

IVIG treatment for repeated hypothermic attacks associated with LGI1 antibody encephalitis

Focal CA3 hippocampal subfield atrophy following LGI1 VGKC-complex antibody limbic encephalitis

Autoimmune episodic ataxia in patients with anti-CASPR2 antibody-associated encephalitis

The value of LGI1, Caspr2 and voltage-gated potassium channel antibodies in encephalitis

Anti-LGI1 encephalitis is strongly associated with HLA-DR7 and HLA-DRB4

From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time

Unexplicated neuropsychiatric disorders a case report LGI1

Clinical spectrum and diagnostic value of antibodies against the potassium channel-related protein complex

A rare case of antileucine-rich glioma-inactivated 1 encephalitis in a 14-year-old girl

The condition usually has a
good prognosis, with almost 70% of patients doing well after two years of follow-up. Disease recurrence, which can occur mostly within the first six months, accounts for 30% of patients. The major remaining symptoms are amnesia,
spatial disorientation, and insomnia.
However, no clear data are available on the prognosis in childhood. In our patient, we used pulse methylprednisolone and IVIG therapy. Her symptoms completely improved, but short-term brachial dystonia recurred intermittently. Follow-up was continued with oral steroid therapy.
Consequently, though a rare disease in
childhood, anti-LGI-1 encephalitis should
be considered in the differential diagnosis in patients with new-onset psychotic symptoms or altered mental state, concomitant hyponatremia, and unique types of seizures.

Guillain–Barré-like syndrome: an uncommon feature of CASPR2 and LGI1 autoimmunity

Autoimmune CASPR2 and LGI1 disorders commonly present with Morvan syndrome (Mos) and/or limbic encephalitis, but whether Guillain-Barré syndrome (GBS) is a specific clinical phenotype is unknown. Here, we first reported an adult patient with dual CASPR2 and LGI1 antibodies in both serum and cerebrospinal fluid, who initially presented with a GBS-like syndrome and developed a typical MoS and respiratory paralysis, with a rapid resolution of his neurological symptoms and disappearance of autoantibodies after treatment with plasma exchange. Additionally, we also provided an overview of the previously reported GBS cases associated with CASPR2 or LGI1 antibodies. These cases expand the phenotypic spectrum of CASPR2 and LGI1 autoimmune syndromes, implying that these two antigens, especially CASPR2, are likely to participate in the etiology of GBS as a potential new target antigen, which deserves further exploration.

Seizure underreporting in LGI1 and CASPR2 antibody encephalitis

Our results question the favorable seizure outcome in patients with CASPR2 and LGI1 antibody encephalitis and suggest that the proportion of patients who have persistent seizures may be greater. Our findings underline the importance of prolonged video-EEG telemetry in this population.

Patient-derived antibodies reveal the subcellular distribution and heterogeneous interactome of LGI1

To investigate whether the cellular and subcellular distribution of LGI1 may explain the localisation of these features, and hence gain broader insights into LGI1’s neurobiology, we analysed the detailed localisation of LGI1, and the diversity of its protein interactome, in mouse brains using patients-derived recombinant monoclonal LGI1-antibodies. Combined immunofluorescence and mass spectrometry analyses showed that LGI1 is enriched in excitatory and inhibitory synaptic contact sites, most densely within CA3 regions of the hippocampus. LGI1 is secreted in both neuronal somatodendritic and axonal compartments, and occurs in oligodendrocytic, neuro-oligodendrocytic and astro-microglial protein complexes. Proteomic data support the presence of LGI1/Kv1/MAGUK complexes, but did not reveal LGI1 complexes with postsynaptic glutamate receptors. Our results extend our understanding of regional, cellular and subcellular LGI1 expression profiles and reveal novel LGI1-associated complexes, thus providing insights into the complex biology of LGI1 and its relationship to seizures and memory loss.

[Morvan syndrome with positive anti LGI1/CASPR2 antibodies in serum/cerebrospinal fluid:a case report and literature review]

A typical case of Morvan syndrome, a 39 year old woman, with positive anti-leucine rich glioma-inactivated 1(LGI1) and contactin-associated protein 2 (CASPR2) antibodies in serum and cerebrospinal fluid.

After treated with corticosteroids, IVIG and mycophenolate mofetil, the patient completely improved. Cognitive function scores recovered from MoCA/MMSE (16/24) to MoCA/MMSE (26/29). Positivity of LGI1/CASPR2 antibodies both in serum/cerebrospinal fluid are rarely seen in patients with Morvan syndrome. Steroids and immunosuppressants are suggested for treatment as early as possible.

Activated microglia by 18 F-DPA714 PET in a case of anti-LGI1 autoimmune encephalitis

We reported a female patient with severe memory loss and faciobrachial dystonic seizures. Anti-LGI1 antibodies were detected in both serum and cerebrospinal fluid. Brain MRI only showed subtle abnormality. She received immunotherapy but unfortunately underwent two relapses after the first attack, resulting in gradual memory decline. Intriguingly, we found higher distribution of 18F-DPA714 in her left medial temporal lobe by PET/CT scan. A novel non-invasive molecular imaging technology indicated potential microglial activation, which might be a promising biomarker for diagnosing and monitoring anti-LGI1 autoimmune encephalitis.

The First Case of Familiar Anti-leucine-rich Glioma-Inactivated1 Autoimmune Encephalitis: A Case Report and Literature Review

In summary, the latest studies above have confirmed that anti-LGI1 encephalitis is genetically susceptible, highly associated with specific alleles located on HLA class II. However, more researches with large samples and more races are necessary to verify it. In addition, the frequencies of these HLA variants were much higher than the prevalence of anti-LGI1 encephalitis, suggesting that people with unique alleles may not develop the disease. Therefore, further studies should focus on the possibility of other influencing factors of anti-LGI1 encephalitis, for instance, additional haplotypes, environmental impacts, and other random effects.

Caspr2 antibodies in herpes simplex encephalitis: an extension of the spectrum of virus induced autoimmunity? – A case report

This is the first report of a patient with autoimmune encephalitis who tested positive for HSV as well as for Caspr2 antibodies.

Cortical and Subcortical Dysmetabolism Are Dynamic Markers of Clinical Disability and Course in Anti-LGI1 Encephalitis

This study provides Class II evidence that semiquantitative measures of putaminal metabolism on PET can differentiate LGI1-AE group from non–LGI1-AE group, Alzheimer disease [AD] group, or negative controls (NCs).

Comprehensive B-Cell Immune Repertoire Analysis of Anti-NMDAR Encephalitis and Anti-LGI1 Encephalitis

 To our knowledge, this is the first study comparing the two most common types of autoimmune encephalitis by analyzing the B-cell immune repertoire. Identification of relatively high frequencies of common clones in BCR repertoires in patients with anti-NMDARE and anti-LGI1E was unexpected and interesting.

The results of flow cytometric analysis demonstrated that patients with anti-NMDARE or anti-LGI1E had more B cells in the peripheral blood that can combine with the NR1 antigen or LGI1 antigen than HC.

Compared with the HC group, patients with anti-NMDARE or anti-LGI1E showed a decreased diversity of the B-cell repertoire, with the smallest value in the anti-LGI1E group. The two encephalitis groups also had lower Chao1 values, fewer rare clones, and more hyperexpanded clones, which suggests the existence of antigen-driven expanded BCR clones in autoimmune encephalitis. Other antigen-driven changes observed in these patients included the IGHV/IGHJ gene usage preference, IGHV–IGHJ combination usage preference, and somatic mutation rate. NR1-positive sequences from patients with anti-NMDARE have been reported as more likely to have a low mutation rate or no mutations (). Our study showed that the circulating BCRs were hypermutated, and the somatic hypermutation rate was lower in the anti-LGI1E group

Recognition of seizure semiology and semiquantitative FDGPET analysis of anti-LGI1 encephalitis

Results: Thirty-three patients were enrolled. According to the patients’ seizure semiology, we divided the patients into four groups: focal impaired awareness seizures
(FIAS, n = 17), faciobrachial dystonic seizures (FBDS)-only (n = 6), FBDS-plus (n = 8),
and focal aware motor seizures (FAMS) (n = 2). No significant differences were found in
the clinical manifestations or accessory tests except for the onset age (FIAS < FBDSplus) and seizure semiology. This was the first study to extensively describe the clinical
manifestations and EEG of FAMS in anti-LGI1 AE patients. In addition, we found that
the patients with different semiologies all showed a wide range of abnormal metabolism, which is not limited to the temporal regions and basal ganglia, and extends far
beyond our previous interpretation of FDG-PET data.
Conclusion: Our results showed that FAMS can serve as a rare indicative seizure semiology of anti-LGI1 AE and that individuals with this disease exhibited widespread
functional network alterations.

CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

NMDAR- and LGI1-E are the 2 most common AE subtypes. Each is representative of 1 of 2 clusters of AE subtypes: NMDAR-E for those with prominent CSF inflammation and LGI1-E for those with little or no CSF inflammation. Beyond the expected quantitative differences, our detailed analysis of CSF findings in therapy-naive acute NMDAR- and LGI1-E shows that each of the 2 AE subtype exhibits a distinct pattern of CSF changes. These are characterized by differences in the interdependencies of CSF parameters and their association with age, disease duration, and disease severity.

Core cerebrospinal fluid biomarker profile in anti-LGI1 encephalitis

Objective

To compare CSF biomarkers’ levels in patients suffering from anti-Leucine-rich Glioma-Inactivated 1 (LGI1) encephalitis to neurodegenerative [Alzheimer’s disease (AD), Creutzfeldt–Jakob’s disease (CJD)] and primary psychiatric (PSY) disorders.

Conclusion

LGI encephalitis patients showed higher Nf L levels than PSY, comparable to AD, and even higher when presenting epilepsy suggesting axonal or synaptic damage linked to epileptic seizures.

Neuropsychological Evaluations in Limbic Encephalitis

Conclusions
Limbic encephalitis (LE) can negatively affect cognition, mood and behavior. On
the cognitive level, LE is primarily associated with different variants of mostly subacute episodic long-term memory dysfunction but also with impairments in attention and executive functions. On the behavioral level, patients with LE often show altered affective states, but other and partially severe psychiatric symptoms have been described as well. Cognition, affect and behavior can recover after immunomodulatory treatment as long as no persistent structural damage has been induced.

An evidence-based neuropsychological baseline assessment for supporting the diagnosis of LE should ideally be conducted before treatment initiation. Repeated assessments for demonstrating disease- or treatment-related disease dynamics should become an essential part of the diagnostic workup of patients with evident or suspected limbic encephalitis.
Therefore, neuropsychology contributes to the diagnosis of LE, it is an important outcome parameter for monitoring the course of the disease and the success of therapeutic interventions, and therewith may guide treatment decisions.

Distinctive binding properties of human monoclonal LGI1 autoantibodies determine pathogenic mechanisms

Overall, in this study, focused molecular hypotheses have been accurately dissected via the generation of patient-derived mAbs. The findings have highlighted intriguing aspects of the immunology and neuroscience, which inform multiple mechanisms underlying this highly amnesic and epileptogenic disease. While several mechanisms remain to be explored, these findings provide insights into the potential of mAbs to inform many aspects of the underlying disease biology which could not easily be highlighted by the study of polyclonal sera and CSFs

Structural network topology in limbic encephalitis is associated with amygdala enlargement, memory performance and serostatus

In the present study, we describe altered network topology in three distinct LE serogroups and aim to relate our findings to
their clinical and cognitive profiles.

Here, we show that lower clustering in
LE patients is associated with greater amygdala enlargement in the predominantly affected hemisphere. Beyond imaging characteristics, memory impairment ranks among the most relevant cognitive deficits associated with LE. Although figural memory was independent of network topology, lower clustering was predictive for verbal memory impairment. This finding confirms a previously reported association between disease severity and verbal memory performance, which was related to structural integrity of the left hippocampus. This association was not found for figural memory performance.

Structural network topology in limbic encephalitis is associated with amygdala enlargement, memory performance and serostatus

In the present study, we describe altered network topology in three distinct LE serogroups and aim to relate our findings to
their clinical and cognitive profiles. 

Here, we show that lower clustering in
LE patients is associated with greater amygdala enlargement in the predominantly affected hemisphere. Beyond imaging characteristics, memory impairment ranks among the most relevant cognitive deficits associated with LE. Although figural memory was independent of network topology, lower clustering was predictive for verbal memory impairment. This finding confirms a previously reported association between disease severity and verbal memory performance, which was related to structural integrity of the left hippocampus. This association was not found for figural memory performance.

Anti-CASPR2 antibody associated encephalitis with anosmia and demyelinating pseudotumor: A case report

Highlight

Anti-CASPR2 antibodies associated disease can present with no widely known core symptoms.
The clinical spectrum of anti-CASPR2 antibodies associated disease is still unfulfilled.
Anti-CASPR2 antibodies can cause anosmia (the loss of the sense of smell, either total or partial).
Comprehensive serological and CSF test is necessary in detecting autoimmune encephalitis.

A rare case of anti-LGI1 limbic encephalitis with concomitant positive NMDAR antibodies

N-methyl-D-aspartate receptor (NMDAR) and leucine-rich glioma-inactivated 1 (LGI1) antibodies define the most prevalently recognized autoimmune encephalitis syndromes, while the simultaneous occurrence of both conditions has hardly been published before.
Conclusions
We report a rare case of LGI1-LE with overlapping symptoms and simultaneous positive NMDAR antibodies. It is necessary to evaluate the presence of NMDAR antibodies in certain LGI1-LE patients with unusual symptoms. However, caution should be exercised in interpreting the observation, given the fact of a single-case study.

CASPR2 antibody encephalitis presenting as transient epileptic amnesia

Highlights

Isolated transient amnesia can be of epileptic origin.
The epileptic origin may be suggested by a short duration of the episodes (< 1 h).
Interictal cognitive complaints are highly suggestive of the diagnosis.
Both interictal and ictal features can be of autoimmune origin (here, anti-CASPR2).

Atypical presentation of an elderly male with autoimmune encephalitis: anti-LG1 limbic encephalitis

A 74-year-old male with a past medical history of stage III CKD and paroxysmal atrial fibrillation was taken to his primary care physician (PCP) by his wife for changes in his mental status. At baseline, he was independent and able to carry out activities of daily living. With the subacute onset of his symptoms, he had trouble remembering recent things and following directions while driving. He had had no weight loss or appetite changes. During his outpatient workup, an MRI of his head, delayed for 10 days due to insurance issues, showed bilateral hippocampal edema. His memory loss progressed, and he developed fever with chills for which he was referred to the emergency department (ED).

Long-Term Memory Dysfunction in Limbic Encephalitis

Functional memory impairment seems to be based on the structural integrity of mesiotemporal brain structures, as memory function is known to correlate with reduced hippocampal subfield volumes.

LE involves frontal lobe structures also, but the main underlying brain pathology involving infiltrating lymphocytes affects the amygdalohippocampal complex indicating that dysfunctional LTM is more probable than impaired working-memory pathways. In particular, some LE forms are susceptible to LTM dysfunction as their disease-mediating antibodies against membrane receptors are critically involved in hippocampal synaptic long-term plasticity and LTM formation. It is thus not surprising that some AMPAR-antibodies associated LE patients present with an amnestic syndrome, such as the unique clinical manifestation of autoimmunity. Antibody-mediated immunopathology involving distinct memory phenotypes fluctuates.

Hippocampal Functional Dynamics Are Clinically Implicated in Autoimmune Encephalitis With Faciobrachial Dystonic Seizures

This is the first study to investigate functional brain activity in patients affected by autoimmune encephalitis with faciobrancial dystonic seizures (FBDS).

Eight treated patients with a presenting clinical diagnosis of autoimmune encephalitis with FBDS participated.

Anti-CASPR2 clinical phenotypes correlate with HLA and immunological features

Antibodies against contactin-associated protein-like 2 (CASPR2-Abs) have been described in acquired neuromyotonia, limbic encephalitis (LE) and Morvan syndrome (MoS). However, it is unknown whether these constitute one sole spectrum of diseases with the same immunopathogenesis or three distinct entities with different mechanisms.

Interpretation Symptoms’ distribution supports specific clinical phenotypes without overlap between LE and MoS. The distinct immunogenetic characteristics shared by all patients with LE and the particular oncological and autoimmune associations of MoS suggest two very different aetiopathogenesis.

A Case Report of Seronegative Limbic Encephalitis

Limbic encephalitis (LE) is an established cause of neurocognitive decline and psychiatric symptoms that are treatable if identified early; delay in diagnosis can leave the patient with permanent losses. MRI plays a pivotal role in establishing a diagnosis, where it shows hyper-intensive lesions, usually in the medial part of the temporal lobes. Patients diagnosed with LE should undergo oncological diagnostics and observatory studies to look for a neoplastic or a paraneoplastic etiology, and they should also undertake the autoimmune screen to look for a possible autoimmune etiology. New and more sensitive techniques should be used to identify unrecognized antibodies that might be associated with those cases of autoimmune encephalitis that are currently considered seronegative. Prompt treatment should be initiated to prevent long-term sequela and to allow a speedy recovery. Treatment includes resection of the underlying tumor, steroids, IVIG, and cyclophosphamide and rituximab in resistant cases.

Synaptic autoimmunity: new insights into LGI1 antibody-mediated neuronal dysfunction

This scientific commentary refers to ‘Distinctive binding properties of human monoclonal LGI1 autoantibodies determine pathogenic mechanisms’, by Ramberger et al. (doi:10.1093/brain/awaa104).

The neuropsychological spectrum of anti-LGI1 antibody mediated autoimmune encephalitis

This review provides a comprehensive summary of the currently available literature, conceptualising our current understanding of the neuropsychological manifestations of anti LGI-1 AE and summarises methodological limitations of the current research to inform and improve future investigations.

Novel Findings of HLA Association With anti-LGI1 Encephalitis: HLA-DRB1*03:01 and HLA-DQB1*02:01

These results indicated that HLA subtypes were only associated with anti-LGI1 encephalitis.

Clinical and Electroencephalographic Features of the Seizures in Neuronal Surface Antibody-Associated Autoimmune Encephalitis

To investigate clinical and electroencephalographic features of the seizures in different types of neuronal surface antibody (NSAb)-associated autoimmune encephalitis

Leucine-rich glioma-inactivated protein 1 (LGI-1) mediated limbic encephalitis associated with syndrome of inappropriate antidiuretic hormone secretion: a case report

We report a 57-year-old man who presented to the ER with recurrent seizures, visual hallucinations, and severe memory impairment over a seven-week period. Initial investigations revealed hyponatremia, indicating syndrome of inappropriate antidiuretic hormone secretion. MRI of the brain revealed bilateral asymmetrical high-T2 and low-T1 signal in the medial temporal lobes. Serum immunofluorescence assay tested positive for LGI-1 antibody. The patient responded to treatment with levetiracetam, intravenous methylprednisolone, and five plasma exchange sessions. The patient remains on a maintenance dose of prednisolone and azathioprine. It is imperative that clinicians recognize signs of autoimmune encephalitis in order to curb long-term sequelae and improve clinical outcomes.

Pathologic tearfulness after limbic encephalitis A novel disorder and its neural basis

Pathologic tearfulness was reported by 50% of the patients, while no patient-reported pathologic laughing. People with LE may cry their eyes out over the simplest things. Dr. Irani’s Oxford group said, “We coined the term ‘Disney syndrome’ when we saw the first few patients as they were tearful while watching kiddie movies with their children.”

Beyond the limbic system: disruption and functional compensation of large-scale brain networks in patients with anti-LGI1 encephalitis

Hippocampal Functional Dynamics Are Clinically Implicated in Autoimmune Encephalitis With Faciobrachial Dystonic Seizures

Distinct HLA associations of LGI1 and CASPR2-antibody diseases

Genetic predisposition in anti-LGI1 and anti-NMDA receptor encephalitis

Immune or Genetic-Mediated Disruption of CASPR2 Causes Pain Hypersensitivity Due to Enhanced Primary Afferent Excitability

Intracellular and non-neuronal targets of voltage-gated potassium channel complex antibodies

A Genetic Disposition for Autoimmune Encephalitis: Searching for Human Leukocyte Antigen (HLA) Complex Subtype

AE with LGI1 or CASPR2 (formerly called voltage-gated potassium channel-complex antibodies)

Clinical features of limbic encephalitis with LGI1 antibody

Delayed LGI1 seropositivity in voltage-gated potassium channel (VGKC)-complex antibody limbic encephalitis

Emergence of new-onset psychotic disorder following recovery from LGI1 antibody-associated limbic encephalitis

What are the emerging features of anti-VGKC autoimmune encephalitis?

VGKC-Complex Antibodies in the Absence of LGI1 and Caspr2 Antibodies

Creutzfeldt-Jakob Disease-Like Periodic Sharp Wave Complexes in VGKC Antibodies Encephalitis: A Case Report

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International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization. Tax ID# 81-3752344 Donations raised directly supports patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world.

Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY  provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society  cannot provide medical advice.


International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.

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