Hippocampal dysfunction after autoimmune encephalitis depending on the antibody type
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Abstract Background Comprehensive neurocognitive function analyses of autoimmune encephalitis (AE) patients, especially long-term ones, are rare. This study aims to measure cognitive function in patients diagnosed with AE. Methods This case–control study included AE patients ( n = 11) with antibodies against NMDA receptor (NMDAR) ( n = 4), VGKC ( n = 3), GAD (3), and one antibody-negative patient. The control group contained 12 pneumococcal meningo-encephalitis patients (PC). Subgroup analyses compared AE patients with and without NMDAR antibodies. Neurocognitive tests were performed to evaluate verbal and visual memory, face recognition, attentional capacity, incidental learning capacity, and overall cognitive function (Montreal cognitive assessment, MoCA). Limbic structural involvement was assessed through magnetic resonance imaging (MRI). Statistical analyses investigated correlations between antibody status, results of neurocognitive tests, and MRI findings. Results Follow-up (AE vs. PC) was 33 (11–95) vs. 96 (26–132) months after diagnosis. Neurocognitive functions were normal in both AE and PC groups in all tests except face recognition, which was pathological in both groups. The overall/recognition/long-delay visual memory ( p = 0.009/0.008/0.005) and incidental learning ( p = 0.017) scores were significantly higher in NMDAR patients compared to non-NMDAR patients. Non-NMDAR patients with right-sided limbic MRI pathologies had significantly lower overall/recognition/long-delay visual memory ( p = 0.006/0.044/0.024) and incidental learning ( p = 0.009) scores compared to NMDAR patients. Conclusions We observed mainly normal neurocognitive functions after autoimmune and bacterial encephalitis. However, compared to NMDAR patients, patients with non-NMDAR autoimmune encephalitis showed a significant and material-specific association between a right-sided hippocampal lesion and limitations in figural-mnestic and incidental learning capacities. Neurocognitive functions in AE patients should be further evaluated prospectively and in more detail.Keywords:
Neurocognitive
Autoimmune encephalitis
Limbic Encephalitis
Introduction: Autoimmune encephalitides are neurologic disorders associated with antibodies against intracellular or synaptic proteins. Anti-Caspr2 encephalitis is an autoimmune encephalitis associated with antibodies against contactin associated protein-like 2 (Caspr2). Caspr2 is a membrane protein expressed in the neurons and it is important for proper function of voltage-gated potassium channels. Caspr2 antibodies can often occur as paraneoplastic process especially in patients with thymoma and small cell lung cancer. ----- Case presentation: A 56-year-old man presented with sudden loss of consciousness on his work. Initial internal and neurological diagnostic procedures did not find the cause of loss of consciousness. In the following period he noticed that he had become forgetful and that he could not remember some events. He also reported difficulties falling asleep and weight lost. After two months of having these symptoms he underwent EEG which showed dysrhythmia bilateral in temporoparietal region and MRI which revealed changes that pointed to the limbic encephalitis. Following diagnostic procedures found Caspr2 antibodies that cause limbic encephalitis. Patient also underwent diagnostic procedures to exclude tumor process as possible cause of autoimmune encephalitis. Treatment started with administering corticosteroids and IVIg and response to that therapy was good. Four months after treatment had started, his memory recovered and repeated MRI of brain revealed regression of limbic encephalitis. ----- Conclusion: Autoimmune encephalitis is a relatively rare but potentially dangerous condition. In patients with seizures, cognitive disturbance especially short term memory loss, sleep dysfunction and weight loss autoimmune limbic encephalitis needs to be considered as a possible diagnose. Analysis of serum and cerebrospinal fluid is necessary to detect cause of limbic encephalitis. In patients with positive autoimmune antibodies it is necessary to exclude tumor and start treatment with immunotherapy.
Autoimmune encephalitis
Limbic Encephalitis
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Limbic encephalitis (LE) is an autoimmune or paraneoplastic disease that affects the medial temporal lobes. The patient will usually present with cognitive impairment, psychiatric changes, and seizures. Autoimmune limbic encephalitis (LE) is a challenging diagnosis as it is not always included in the typical paraneoplastic/autoimmune panels. Anti-GAD antibodies are associated with various disease including type I diabetes mellitus, various autoimmune processes, some neoplastic and infectious diseases. Thus, it is not as specific as some of the antibodies causing LE. We are presenting two cases of isolated anti-GAD antibody-associated limbic encephalitis. Both patients were adults who developed status epilepticus and refractory seizures, cognitive impairment and mood instability. Patients' cerebrospinal fluid (CSF) and serum anti- GAD antibodies were elevated and after treatment returned to normal reference range. The diagnosis for both patients was delayed (by over one month following hospitalization), both patients required prolonged hospitalization and rehabilitation after discharge. Patient's condition improved only after immunotherapy, but required several antiepileptic drugs for seizure control. The diagnosis was more difficult in the first patient, who had numerous other medical problems including ESRD and moderately severe microvascular changes on brain imaging. In this particular patient, it was hard to appreciate any signal changes on MRI in the mesial temporal lobes given the underlying white matter disease. We recommend inclusion of anti- GAD antibody in the paraneoplastic/encephalopathy panels in order to decrease missed cases of this important cause of LE as well as to hasten the diagnosis. This is a treatable disease, and timely diagnosis is imperative to improve outcomes.
Limbic Encephalitis
Autoimmune encephalitis
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Neuromyotonia
Autoimmune encephalitis
Limbic Encephalitis
Clinical phenotype
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Limbic Encephalitis
Autoimmune encephalitis
Fluid-attenuated inversion recovery
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We present a case of an elderly male with anti-LG1 limbic encephalitis involving hypothalamus presenting with acute changes in mental status and persistent hyperthermia.A 74-year-old male presented to the hospital with fever and chills. He had also been evaluated by his PCP for changes in his mental status, trouble remembering things, and following directions while driving. A lumbar puncture was performed, and empiric meningitis coverage was initiated. His LP results were not suggestive for any infectious process. An MRI showed bilateral hippocampal edema. As an infectious workup was non-revealing and other causes were ruled out, with the concern for paraneoplastic or autoimmune encephalitis, patient was started on high dose steroids and plasma exchange while results of antibodies for autoimmune and paraneoplastic encephalitis were awaited. After plasmapheresis and a course of steroids, the patient's mental status began to slowly improve. He was discharged from the hospital and on subsequent neurology office visit, his serum autoimmune encephalitis panel returned positive for anti-LGI 1 antibodies. Further management consisted of outpatient rituximab infusion.Diagnosis of limbic encephalitis can be challenging and can present with symptoms of limbic dysfunction. A modest index of suspicion of limbic encephalitis should be kept in adults with altered mental changes. Early recognition and initiation of therapy can be crucial in the management of patients with autoimmune encephalitis and can prevent permanent cognitive impairment and damage.
Limbic Encephalitis
Autoimmune encephalitis
Altered Mental Status
Plasmapheresis
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Limbic Encephalitis
Autoimmune encephalitis
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Autoimmune encephalitis is one of the main causes of noninfectious encephalitis. Can be broadly classied into non-paraneoplastic, paraneoplastic and vasculitis associated encephalopathy. It can be triggered by tumors and infections. Autoimmune encephalitis presents with variety of clinical features such as acute onset behavioral changes, psychiatric symptoms, memory loss, movement disorders, dystonia, mutism and seizures. Neuronal antibodies are directed against cell surface Ag(CSAab) ,synaptic antigen (SyAab), intraneuronal Ag(INAab). Anti LG1 and CASPR2 encephalitis are usually non-neoplastic autoimmune encephalopathy where antibodies are directed against proteins associated with Voltage gated k channel. About 50% patients with anti-VGKC encephalitis do not present antibodies against LGI1 or CASPR-2. Anually 1 case is detected amongst 100000 patients. This is a case report of a patient presented to Geriatrics OPD and the series of evaluation which led to diagnosis and treatment outcome.
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Autoimmune encephalitis
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Autoimmune encephalitis (AE) results from immune-mediated damage to the central nervous system (CNS) with varying clinical manifestations depending on autoimmune antibodies present and the antigens they target. Leucine-rich glioma-inactivated protein 1 (LGI1) has been recognized as one of the leading causes of limbic encephalitis (LE), presenting with seizures, memory loss, and faciobrachial dystonic seizures. A better understanding of the unique presentations of these AE allows for quick and effective diagnosis and treatment. We are presenting a very unusual case of LGI1 autoimmune LE with two additional autoantibodies, anti-acetylcholine receptor (AChR) and anti-striational, in a patient with an underlying thymoma. We will discuss the pathophysiology and common clinical presentation of anti-LGI1 autoimmune LE.
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Autoimmune encephalitis
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Background: Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients, including stiff person syndrome, cerebellar ataxia, refractory epilepsy, and limbic and extralimbic encephalitis. There have been some case reports and investigations regarding anti-GAD65 antibody-associated encephalitis in adult populations, but pediatric cases are rare. We retrospectively analyzed the clinical data of three anti-GAD65 antibody-positive patients to explore the diversity and clinical features of anti-GAD65 antibody-associated pediatric autoimmune encephalitis. Methods: The clinical data of a series of three patients positive for anti-GAD65 antibody were retrospectively analyzed. GAD65 antibodies were determined in serum and CSF using a cell-based assay. Results: All three patients were female, and the onset ages were 4 years and 9 months, 6 years, and 16 years old. Their clinical phenotypes included autoimmune limbic encephalitis, extralimbic encephalitis, and encephalitis combining limbic and extralimbic encephalitis. The clinical symptoms included seizures, memory deficits, drowsiness, dysautonomia, and headache. All patients had abnormal carinal MRI and EEG. All patients received immunotherapy and had transiently good responsiveness, but one patient then experienced relapse. In follow-up, one patient with extralimbic encephalitis recovered completely, while two patients with limbic involvement had poor outcomes with refractory focal epilepsy. Conclusion: In addition to limbic encephalitis, extralimbic encephalitis is also an important phenotype in patients who are positive for anti-GAD65 antibodies. Early diagnosis and immunotherapy can improve the symptoms. However, patients with limbic encephalitis often have refractory epilepsy in the chronic phase and have a poor long-term outcome.
Limbic Encephalitis
Autoimmune encephalitis
Cerebellar ataxia
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BACKGROUND:Autoimmune encephalitis might coexist in patients with autoimmune demyelinating disorders. CASE REPORT:We report on a case of a 45-year-old female multiple sclerosis (MS) patient presenting with acute onset short-term memory loss, altered mental status, inflammatory cerebrospinal fluid (CSF) findings and an MRI lesion on the left temporal lobe. An extensive panel for neuronal autoantibodies proved negative. Neuropsychological symptoms gave a prompt response to immunotherapy but nevertheless control MRI showed left hippocampal atrophy. CONCLUSIONS:Several recent reports of concurrent emergence of autoimmune encephalitis and MS suggest a common mechanism for these disorders. Since autoimmune encephalitis and MS share certain common CSF and neuroimaging findings, an increased understanding of overlapping autoimmune brain disorders is required to avoid misdiagnosis especially in antibody negative autoimmune encephalitis cases.
Autoimmune encephalitis
Limbic Encephalitis
Demyelinating Disorder
Hippocampal sclerosis
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