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Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT), or Hashimoto's encephalopathy, is a rare autoimmune neurological disorder linked to elevated antithyroid antibodies and presenting various neurological symptoms. This report deals with a case of a 54-year-old female with a history of hypothyroidism who presented with hypertensive emergency and atypical neurological symptoms that deteriorated during the hospital stay. On testing, she was euthyroid. Initial investigations, including MRI and CSF analysis, were inconclusive, but high levels of antithyroid peroxidase (Anti TPO) antibodies confirmed the diagnosis of SREAT. The patient was managed with intravenous methylprednisolone, leading to rapid clinical improvement. SREAT, being a diagnosis of exclusion, presents with various neurological and neuropsychiatric symptoms that can be difficult to identify. This condition remains poorly understood, though autoimmune factors and antithyroid antibodies seem to play a role. Glucocorticoids remain the primary treatment choice. At the same time, other immunosuppressive agents are reserved for non-responders. This case highlights the critical importance of early diagnosis in SREAT, as early recognition and corticosteroid treatment can lead to significant recovery. Timely intervention is essential for improved patient prognosis, emphasizing the need for awareness of SREAT in patients with a history of hypothyroidism presenting with neurological symptoms. [ABSTRACT FROM AUTHOR]

Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT), or Hashimoto’s encephalopathy, is a rare autoimmune neurological disorder linked to elevated antithyroid antibodies and presenting various neurological symptoms. This report deals with a case of a 54-year-old female with a history of hypothyroidism who presented with hypertensive emergency and atypical neurological symptoms that deteriorated during the hospital stay. On testing, she was euthyroid. Initial investigations, including MRI and CSF analysis, were inconclusive, but high levels of antithyroid peroxidase (Anti TPO) antibodies confirmed the diagnosis of SREAT. The patient was managed with intravenous methylprednisolone, leading to rapid clinical improvement. SREAT, being a diagnosis of exclusion, presents with various neurological and neuropsychiatric symptoms that can be difficult to identify. This condition remains poorly understood, though autoimmune factors and antithyroid antibodies seem to play a role. Glucocorticoids remain the primary treatment choice. At the same time, other immunosuppressive agents are reserved for non-responders. This case highlights the critical importance of early diagnosis in SREAT, as early recognition and corticosteroid treatment can lead to significant recovery. Timely intervention is essential for improved patient prognosis, emphasizing the need for awareness of SREAT in patients with a history of hypothyroidism presenting with neurological symptoms.

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune encephalitis due to immune production of anti-NMDAR antibodies against the NR1 subunit of the NMDA receptor which is present throughout the central nervous system. This condition had been reported to be prevalent in patients with certain medical conditions; however so far, there have been limited systematic reviews and meta-analyses on the prevalence and factors associated. Objective: This study was to determine the prevalence and factors associated with anti-NMDAR encephalitis among affected patients. Material and Methods: The protocol of this study has been registered (2019: CRD42019142002) with the International Prospective Register of Systematic Reviews (PROSPERO). The primary outcome was the incidence or prevalence of anti-NMDAR encephalitis and secondary outcomes were factors associated with anti-NMDAR encephalitis. Results: There were 11 studies and a total of 873 million patients taken from high-risk populations across 11 countries that were included in the primary analysis. The overall pooled prevalence of anti-NMDAR encephalitis among patients with medical conditions was 7.0% (95% CI = 4.4, 9.6). Those with first episode of psychosis or schizophrenia were at a higher risk of developing anti-NMDAR encephalitis with an odds ratio of 5.976 (95% CI = 1.122, 31.825). Conclusion: We found that almost one-tenth of patients with medical conditions had anti-NMDAR encephalitis; particularly those with first episode of psychosis or schizophrenia were among the high-risk medical conditions. [ABSTRACT FROM AUTHOR]

Background: Hashimoto's encephalopathy (HE) is a controversial immunological neuropsychiatric disease, with a poorly understood pathogenesis. It is characterized by symptoms of acute or subacute encephalopathy which usually occur in the presence of elevated levels of antithyroid antibodies. Even though it is also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), some cases appear to be steroid-resistant. This review examined whether treatment of Hashimoto's encephalopathy with intravenous immunoglobulin (IVIG) is associated with better clinical outcomes than the standard therapy. Additionally, we presented a case of a 59-year-old man who presented with severe neurological manifestations and was successfully treated with intravenous immunoglobulin. Methods: The online databases PubMed and EMBASE were searched. Results: A total of 1,365 articles were identified. After the deletion of 112 duplicates, 1,253 studies were screened by evaluating the title and abstract, focusing on Hashimoto's encephalopathy cases where IVIG were used. 846 studies were excluded because they were not relevant to the topic or included pediatric population. Therefore, 407 full-text articles were assessed for eligibility. The final analysis included 14 eligible articles after 393 were excluded (irrelevant texts, not written in English, full-text not available). In the majority of the selected case-reports, IVIG was associated with a good outcome, sometimes even with dramatic improvements in patient's status. Conclusion: In last years, intravenous immunoglobulin therapy proved its utility in Hashimoto's encephalopathy's treatment, being a well tolerated therapy associated with remarkable improvement in patient's status. Further research is still needed in order to define the optimal treatment protocol for Hashimoto's encephalopathy and to establish if intravenous immunoglobulin can also be used as a first-line therapy, alone or in combination with steroids. [ABSTRACT FROM AUTHOR]

BackgroundHashimoto’s encephalopathy (HE) is a controversial immunological neuropsychiatric disease, with a poorly understood pathogenesis. It is characterized by symptoms of acute or subacute encephalopathy which usually occur in the presence of elevated levels of antithyroid antibodies. Even though it is also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), some cases appear to be steroid-resistant. This review examined whether treatment of Hashimoto’s encephalopathy with intravenous immunoglobulin (IVIG) is associated with better clinical outcomes than the standard therapy. Additionally, we presented a case of a 59-year-old man who presented with severe neurological manifestations and was successfully treated with intravenous immunoglobulin.MethodsThe online databases PubMed and EMBASE were searched.ResultsA total of 1,365 articles were identified. After the deletion of 112 duplicates, 1,253 studies were screened by evaluating the title and abstract, focusing on Hashimoto’s encephalopathy cases where IVIG were used. 846 studies were excluded because they were not relevant to the topic or included pediatric population. Therefore, 407 full-text articles were assessed for eligibility. The final analysis included 14 eligible articles after 393 were excluded (irrelevant texts, not written in English, full-text not available). In the majority of the selected case-reports, IVIG was associated with a good outcome, sometimes even with dramatic improvements in patient’s status.ConclusionIn last years, intravenous immunoglobulin therapy proved its utility in Hashimoto’s encephalopathy’s treatment, being a well tolerated therapy associated with remarkable improvement in patient’s status. Further research is still needed in order to define the optimal treatment protocol for Hashimoto’s encephalopathy and to establish if intravenous immunoglobulin can also be used as a first-line therapy, alone or in combination with steroids.

It is daunting to determine the etiology of rapidly progressive dementia (RPD), which includes metabolic, neoplastic, infectious, autoimmune, neurodegenerative and other conditions. Herein, we illustrate an unusual case of a patient primarily exhibiting RPD, overlapping sleep dysfunction, psychosis and abnormal movement, which was finally defined as anti-IgLON5 disease, a novel and rare autoimmune encephalopathy. Furthermore, we longitudinally described his cognitive and psychological performance in detail, and determined that early initiation of immunotherapy in this patient did not result in a good outcome. These data highlight anti-IgLON5 disease as a possible differential diagnosis in patients with RPD. [ABSTRACT FROM AUTHOR]

Objectives: To examine the chemokine profile in the cerebrospinal fluid (CSF) of patients with glial fibrillary acidic protein astrocytopathy (GFAP‐A), central nervous system immune‐related adverse event (CNS‐irAE), neurosarcoidosis (NS), neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS), and human T‐cell leukemia virus‐1 (HTLV‐1)‐associated myelopathy (HAM). Methods: The study included 38 patients presenting to St. Marianna University Hospital between May 2013 and November 2021 with GFAP‐A, CNS‐irAE, NMOSD, MS, NS, HAM and noninflammatory neurological diseases (NIND). We recorded the age, sex, duration of disease, brain/spinal lesions on magnetic resonance imaging (MRI), blood data, and measured chemokines (CXCL9, −10, −13, CCL3, −4, −17, −20, −22) in CSF. In patients with GFAP‐A, clinical symptoms, and CSF CXCL10 levels were compared before and after steroid treatment. Results: Patients with GFAP‐A had higher CSF levels of CXCL10, CXCL13, and CCL22 (10736.1 [8786.7–149079.0] pg/ml (p <.05), 378.4 [239.9–412.2] pg/ml (p <.01) and 159.9 [130.5–413.9] pg/ml (p <.01), respectively). The CSF levels of CXCL10 improved from 10736.1 [8786.7–149079.0] pg/ml to 1879.0 [783.9–4360.0] pg/ml in patients with GFAP‐A by steroid therapy. Conclusion: CSF CXCL10 levels were particularly high in GFAP‐A, and changes in levels after treatment correlated with clinical improvements, suggesting CXCL10 involvement in GFAP‐A pathogenesis. [ABSTRACT FROM AUTHOR]

The normal function of the nervous system is conditioned by the undisturbed function of the thyroid gland and its hormones. Comprehensive clinical manifestations, including neurological disorders in Hashimoto's thyroiditis, have long been understood and, in recent years, attention has been paid to neurological symptoms in euthyroid patients. Hashimoto encephalopathy is a controversial and poorly understood disease entity and the pathogenesis of the condition remains unclear. We still derive our understanding of this condition from case reports, but on the basis of these, a clear clinical picture of this entity can be proposed. Based on a review of the recent literature, the authors present the current view on the subject, discuss controversies and questions that still remain unanswered, as well as ongoing research in this area and the results of our own work in patients with Hashimoto's thyroiditis. [ABSTRACT FROM AUTHOR]

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune disease that appears as a fulminant, subacute or chronic course of altered mental status, often accompanied by seizures and myoclonus. This case report demonstrates that SREAT can be present with solitary neuropsychiatric signs long before seizures and myoclonus. Methods: A 42-year-old female presented with abrupt-onset whole-body myoclonic jerks. She had been suffering from depression for 15 years before being diagnosed with hypothyroidism. She was conscious, oriented and alert. At rest, all four limbs had multifocal mildto-moderate myoclonus, which was significantly aggravated by muscle activation; her Hamilton Depression Rating Scale score was 21. Results: Antithyroglobulin and antithyroid peroxidase autoantibodies were both above 2,000 IU/mL. The thyroid-stimulating hormone level was 5.65 mIU/mL, free triiodothyronine level was 3.36 pg/mL and free thyroxine level was 1.02 ng/dL. Vasculitis profile and the serum test for autoimmune encephalitis panel were negative. Brain neuroimaging was normal. Pulse dose of methylprednisolone followed by oral steroids resulted in significant clinical improvement. Conclusion: SREAT can present with chronic neuropsychiatric symptoms with abrupt exacerbation of seizures and myoclonus. This case study emphasizes the importance of screening individuals with depression and thyroid problems for serum antithyroid antibody levels. In most cases, steroid treatment yields positive results. [ABSTRACT FROM AUTHOR]

Introduction: Hashimoto’s encephalopathy (HE) is a rare, potentially life-threatening disease with a wide spectrum of clinical manifestations from slight symptoms to mainly neurological and psychiatric syndromes, which significantly debilitate the quality of life and make the diagnostic process complicated, especially in patients with no previous history of thyroid disorders. According to the recent data, it occurs in about 2.1 cases per 100 000 population with still increasing prevalence. Despite, over than fifty years have passed since the first case of HE was reported, its etiopathogenesis is not completely clarify and there is no universal diagnostic criteria. Aim of the study: This article summarizes the current knowledge about pathophysiology, clinical manifestations, difficulties in differential diagnosis and therapeutic dilemmas in patients with HE. Description of knowledge: HE is an autoimmune-mediated encephalopathy associated with Hashimoto’s disease and elevated titers of anti-thyroid antibodies, mainly anti-thyroid peroxidase antibody. The diagnostic process is usually multi-step due to various diseases mimicking HE, such as Creutzfeldt-Jacob disease, brain tumors, epilepsy, Alzheimer's disease, stroke, other forms of autoimmune encephalitis, schizophrenia, spontaneous cerebrospinal fluid leak or infectious encephalitis. The range of diagnostic procedures includes physical and mental examination, laboratory tests, brain imaging, EEG as well as cerebrospinal fluid analysis. However, the first line strategy based on steroids is effective, there are some cases in the literature that reveal this management as not fully sufficient, because only partial improvement was achieved or the steroid-dependence was observed. Among other therapeutic methods, plasmapheresis may be used additionally. Conclusions: HE requires interdisciplinary approach and it constitutes a great challenge for clinicians of various specialties, such as endocrinologists, neurologists, and psychiatrists. The prompt implementation of adequate therapy usually provides the full recovery of the patients without any early or late complications.

Background: For many laboratories, autoimmune encephalopathy (AE) panels are send-out tests. These tests are expensive, and ordering patterns vary greatly. There is also a lack of consensus on which panel to order and poor understanding of the clinical utility of these panels. These challenges were presented to our newly formed, multidisciplinary, diagnostic stewardship committee (DSC). Through this collaboration, we developed an algorithm for ordering AE panels; combining diagnostic criteria with practice guidelines. Methods: We analyzed test-ordering patterns in 2018 and calculated a true-positive rate based on clinical presentation and panel interpretation. An evidence-based approach was combined with input from the Department of Neurology to synthesize our algorithm. Efficacy of the algorithm (number of panels ordered, cost, and true positives) was assessed before and after implementation. Results: In 2018, 77 AE-related panels were ordered, costing $137 510. The true-positive rate was 10%, although ordering multiple, similar panels for the same patient was common. Before implementing the algorithm (January 1–July 31, 2019), 55 panels were ordered, costing $105 120. The total true-positive rate was 3.6%. After implementation, 23 tests were ordered in a 5-month period, totaling $50 220. The true-positive rate was 13%. Conclusion: With the DSC-directed mandate, we developed an algorithm for ordering AE panels. Comparison of pre- and postimplementation data showed a higher true-positive rate, indicating that our algorithm was able to successfully identify the at-risk population for AE disorders. This was met with a 43% decrease in the number of tests ordered, with total cost savings of $25 000 over 5 months. [ABSTRACT FROM AUTHOR]

Steroid-responsive encephalopathy associated with thyroiditis (SREAT) is a rare autoimmune disorder characterized by cognitive dysfunction. SREAT is frequently overlooked despite its profound impact on patients and the healthcare system. This case report details a male patient who experienced a series of neuropsychiatric symptoms over several months, ultimately attributed to SREAT, emphasizing the critical impact of delayed recognition. The case underscores the diverse and often complicated presentations of SREAT, advocating for the timely consideration of autoimmune encephalopathy in patients with unexplained neuropsychiatric symptoms and abnormal thyroid function. Furthermore, it illustrates the effectiveness of steroids in managing SREAT and the challenges posed by long-term steroid use. Comprehensive diagnostic criteria and tailored treatment strategies are crucial for improving patient outcomes in this rare but impactful disorder., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Kayali et al.)

IgLON5 antibody encephalopathy is a rare but increasingly recognized disorder with a variety of clinical signs. Typical symptoms are sleep disorder, gait disturbances, signs of bulbar dysfunction and a variety of neurological symptoms like oculomotor abnormalities and movement disorders. In addition, cognitive decline can be a prominent symptom. So far, there are only a few studies that have dealt with the course and possible treatment options of IgLON5 antibody encephalopathy. In this study the clinical case of a female patient with IgLON5 antibody disease and the response to treatment is described. Here we report on the case of a 67-year-old female patient who showed cognitive deterioration, gait difficulties, and chronic obstructive sleep disorder. The diagnostic course showed a positive anti-IgLON5 serum and anti-IgLON5 IgG antibodies in cerebrospinal fluid. The patient was subsequently treated with high dosage i.v. methylprednisolone, i.v. immunoglobulins and plasmapheresis. Neuropsychological tests showed cognitive deficits in different domains, including verbal and visual memory. Both, neuropsychological deficits and antibody titer, showed an improvement after plasmapheresis. The presented case shows that IgLON5 disease can present with rapidly progressing cognitive deterioration as the prominent symptom, adding to the variety of clinical signs in this disorder. Testing for IgLON5-antibodies should be considered in patients with progressing cognitive decline, especially if accompanied by sleep disorders or neurological symptoms like oculomotor abnormalities, dysautonomia or bulbar signs.

IgLON5 antibody encephalopathy is a rare but increasingly recognized disorder with a variety of clinical signs. Typical symptoms are sleep disorder, gait disturbances, signs of bulbar dysfunction and a variety of neurological symptoms like oculomotor abnormalities and movement disorders. In addition, cognitive decline can be a prominent symptom. So far, there are only a few studies that have dealt with the course and possible treatment options of IgLON5 antibody encephalopathy. In this study the clinical case of a female patient with IgLON5 antibody disease and the response to treatment is described. Here we report on the case of a 67-year-old female patient who showed cognitive deterioration, gait difficulties, and chronic obstructive sleep disorder. The diagnostic course showed a positive anti-IgLON5 serum and anti-IgLON5 IgG antibodies in cerebrospinal fluid. The patient was subsequently treated with high dosage i.v. methylprednisolone, i.v. immunoglobulins and plasmapheresis. Neuropsychological tests showed cognitive deficits in different domains, including verbal and visual memory. Both, neuropsychological deficits and antibody titer, showed an improvement after plasmapheresis. The presented case shows that IgLON5 disease can present with rapidly progressing cognitive deterioration as the prominent symptom, adding to the variety of clinical signs in this disorder. Testing for IgLON5-antibodies should be considered in patients with progressing cognitive decline, especially if accompanied by sleep disorders or neurological symptoms like oculomotor abnormalities, dysautonomia or bulbar signs. [ABSTRACT FROM AUTHOR]

Background: Hashimoto encephalopathy (HE) is a rare condition associated with autoimmune thyroid disease. We aimed to report the youngest patient with Down syndrome and HE with an unusual presentation. Case Report: Six years and six months old boy with Down syndrome admitted due to loss of speech. His physical development was appropriate for his age and had no goiter. Neurological examination revealed the absence of eye contact and stereotypic movements. Autism spectrum disorder was considered based on his result on Gilliam autism evaluation scale. He had subclinical hypothyroidism with markedly elevated anti-thyroid peroxidase antibody level, rare spikes in the frontocentral area were found in electroencephalography, and cranial magnetic resonance imaging was normal. Neurologic improvement was observed to a treatment with glucocorticoid and thyroid hormone. Conclusion: HE might be considered in patients with Down syndrome along with progressive cognitive decline and autistic regression. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical manifestations of the first case of autoimmune encephalopathy due to antibodies against n-methyl-d-aspartate receptor, confirmed in Pastaza, Ecuador. Clinical case: A 34-year-old male patient is reported because of insidious behaviour disorders associated with progressive impairment of cognitive functions. He underwent psychiatric evaluation and tests to detect autoimmune diseases, which resulted negative. Due to the presentation of these neurological and psychiatric manifestations in a young patient, a surface electroencephalogram and detection of antibodies against the n-methyl-daspartate receptor were indicated for the final diagnosis and to prescribe therapy. The patient was found to have autoimmune encephalopathy. After treatment with steroids, the patient evolved favorably. Conclusion: The clinical manifestations of this case were mental dysfunctions such as insomnia of conciliation and maintenance of sleep, abnormal behaviors, irritability, impulsivity, aggressiveness, hypersexuality, compulsion, zealoty, rambling language and memory disorder in the short and medium term. [ABSTRACT FROM AUTHOR]

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Parry-Romberg syndrome (PRS) is a progressive facial hemiatrophy often associated with severe epilepsy. Although an immune-mediated vasculitic pathogenesis is widely assumed, no CNS-specific autoantibody has been described so far. A 2-year-old boy was admitted for a status epilepticus preceded by fever, restlessness, insomnia, and left facial rash. Cerebrospinal fluid was positive for glutamic acid decarboxylase (GAD)-antibodies. Brain MRI revealed FLAIR hyperintensities on left mediotemporal areas. He was successfully treated with intravenous methylprednisolone. One month later, seizures and facial rash reappeared and steroids were satisfactorily repeated. However, left hemifacial rash reappeared 5 months later, slowly followed by sclerotic skin lesions on frontal scalp and hemifacial sub-atrophy, leading to a diagnosis of PRS. Three years later, and despite chronic immunosuppression, new MRI lesions on left white matter are seen and left hemifacial atrophy has progressed. For the first time, we describe GAD autoantibodies in a PRS patient with epileptic encephalopathy. Epileptic syndromes with GAD autoantibodies are frequently described though with a questionable pathogenic significance. Given the clinical and MRI similarities of PRS with both Morphea and Rasmussen's encephalitis, we suggest that, in our patient, the initial facial skin vasculitis spread into CNS vessels through perforating arteries, inducing neuronal MHC-class I presentation of GAD epitopes, ultimately causing CD8-mediated neuronal cytotoxicity and the epileptic encephalopathy. GAD autoantibodies might represent the missing pathophysiological link between PRS and neuropsychiatric manifestations. [ABSTRACT FROM AUTHOR]

Hashimoto's encephalopathy (HE) is a rare, clinically heterogeneous condition associated with positive thyroid autoantibodies. It is increasingly recognized as an important and treatable cause of autoimmune encephalopathy. Thyroid-associated antibodies such as thyroperoxidase (TPO) antibody, thyroglobulin (TG) antibody, and thyrotropin receptor (TR) antibody were found in HE patients with seizure disorders. Although antithyroid antibodies are required for the diagnosis of HE, their role in the pathogenesis of HE remains uncertain. Instead of playing a key role in the pathophysiology processes of HE, it is suggested that thyroid-associated antibodies are hallmarks of HE. Seizure disorders were found in approximately two-thirds of HE patients, and common anticonvulsant therapy alone is usually ineffective. Some patients did not respond to any antiepileptic drugs. The use of immunotherapy can effectively control seizure disorders. Electroencephalography and imaging findings are not specific to HE patients and can also be seen in other causes of encephalopathies. However, the prognosis in the majority of patients with HE was usually good if it is diagnosed and treated correctly.

Febrile infection-related epilepsy syndrome (FIRES) is a rare epileptic encephalopathy that occurs in children or adolescents. To date, evidence for the management of the post-acute phase of FIRES is focused on drug-resistant epilepsy that continues from the acute phase. Information on involuntary movements, which are newly developed in the chronic phase, is limited. We report a 13-year-old boy, who had a history of FIRES at nine years of age and experienced worsening seizure control that was accompanied by unremitting involuntary　movements after two years of a fairly controlled period. The involuntary movements resulted in motor deterioration and forced him to be bedridden. Although no neuronal autoantibodies were detected, we hypothesized that the boy's neurological deterioration was triggered by an autoimmune response based on the elevation of serum anti-glutamic acid decarboxylase and serum anti-thyroid peroxidase antibodies and hypermetabolism of bilateral lenticular nuclei on 18-fluorodeoxyglucose positron emission tomography that resembled those reported in patients with other types of autoimmune encephalitis. Serial methylprednisolone pulse therapy and intravenous immunoglobulin therapy ameliorated involuntary movements and improved his activities of daily living. Late-onset involuntary movements, along with seizure exacerbation, may appear in the chronic phase of FIRES. Immunotherapy could be effective in treating these symptoms., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Ohno et al.)

Hashimoto encephalopathy (HE) is an autoimmune encephalopathy associated with autoimmune chronic thyroiditis. The clinical entity and nosology of HE have long been debated. Recently, new autoantibodies associated with autoimmune encephalitis have been discovered. With accumulated reported cases, and our discovery of serum autoantibody against NH2‐terminal of alpha‐enolase as a specific diagnostic marker, HE has been recognized as a distinct clinical entity. The condition appears dominant in females. The clinical features of HE are characterized by various neuropsychiatric symptoms, such as altered consciousness, psychosis and seizures, the presence of serum anti‐thyroid antibodies, normal or non‐specific changes in brain magnetic resonance imaging, diffuse slow wave activities in electroencephalogram and responsiveness to immunotherapy. A few pathological studies showed a lymphocytic infiltration of the small and medium‐sized vessels of the brain, which suggested vasculitis. Non‐specific neurological symptoms, laboratory findings and neuroimaging of HE make its diagnosis an oversight and difficult, whereas antibodies against NH2‐terminal of alpha‐enolase can be useful as a diagnostic biomarker for HE. In this review, we describe the current state of knowledge about HE and discuss progress in the diagnosis of HE. [ABSTRACT FROM AUTHOR]

Hashimoto's encephalopathy (HE) is a rare, clinically heterogeneous condition associated with positive thyroid autoantibodies. It is increasingly recognized as an important and treatable cause of autoimmune encephalopathy. Thyroid-associated antibodies such as thyroperoxidase (TPO) antibody, thyroglobulin (TG) antibody, and thyrotropin receptor (TR) antibody were found in HE patients with seizure disorders. Although antithyroid antibodies are required for the diagnosis of HE, their role in the pathogenesis of HE remains uncertain. Instead of playing a key role in the pathophysiology processes of HE, it is suggested that thyroid-associated antibodies are hallmarks of HE. Seizure disorders were found in approximately two-thirds of HE patients, and common anticonvulsant therapy alone is usually ineffective. Some patients did not respond to any antiepileptic drugs. The use of immunotherapy can effectively control seizure disorders. Electroencephalography and imaging findings are not specific to HE patients and can also be seen in other causes of encephalopathies. However, the prognosis in the majority of patients with HE was usually good if it is diagnosed and treated correctly. [ABSTRACT FROM AUTHOR]

Autoimmune encephalopathy is one of the treatable causes of rapidly progressive dementia; however, it is often underdiagnosed. Autoantibodies against voltage-gated calcium channel (VGCC) have been linked to several neurological disorders, including Lambert-Eaton syndrome, but VGCC antibody-associated encephalopathy is uncommon. Herein, we present a case of a 74-year-old woman with prominent neuropsychiatric symptoms followed by rapid cognitive decline. Extensive initial studies were nondiagnostic. Subsequently, serum N-type VGCC antibody was positive. After treatment with intravenous immunoglobulin, the patient's cognition and neuropsychiatric symptoms significantly improved. [ABSTRACT FROM AUTHOR]

Purpose: Glutamic acid decarboxylase (GAD65) has been implicated in a number of autoimmune-associated neurologic syndromes, including autoimmune epilepsy. This study categorizes the spectrum of MRI findings in patients with a clinical diagnosis of autoimmune epilepsy and elevated serum GAD65 autoantibodies.Methods: An institutional database search identified patients with elevated serum GAD65 antibodies and a clinical diagnosis of autoimmune epilepsy who had undergone brain MRI. Imaging studies were reviewed by three board-certified neuroradiologists and one neuroradiology fellow. Studies were evaluated for cortical/subcortical and hippocampal signal abnormality, cerebellar and cerebral volume loss, mesial temporal sclerosis, and parenchymal/leptomeningeal enhancement. The electronic medical record was reviewed for relevant clinical information and laboratory markers.Results: A study cohort of 19 patients was identified. The majority of patients were female (84%), with a mean age of onset of 27 years. Serum GAD65 titers ranged from 33 to 4415 nmol/L (normal < 0.02 nmol/L). The most common presentation was medically intractable, complex partial seizures with temporal lobe onset. Parenchymal atrophy was the most common imaging finding (47%), with a subset of patients demonstrating cortical/subcortical parenchymal T2 hyperintensity (37%) or abnormal hippocampal signal (26%). No patients demonstrated abnormal parenchymal/leptomeningeal enhancement.Conclusion: The most common MRI finding in GAD65-associated autoimmune epilepsy is disproportionate parenchymal atrophy for age, often associated with abnormal cortical/subcortical T2 hyperintensities. Hippocampal abnormalities are seen in a minority of patients. This constellation of findings in a patient with medically intractable epilepsy should raise the possibility of GAD65 autoimmunity. [ABSTRACT FROM AUTHOR]

Susac syndrome is a relatively uncommon autoimmune disease that predominantly affects young females, with the highest incidence between the third and fourth decade of life, presenting classically with encephalopathy, various CNS dysfunctions, visual impairment due to retinal artery occlusion, and hearing loss. Despite treatment options, such as glucocorticoid steroids, intravenous immunoglobulin, methotrexate, azathioprine, mycophenolate mofetil, or rituximab, some patients with Susac syndrome remain refractory to therapy. We present a case report of a 38-year-old female with refractory Susac syndrome who was treated successfully with plasmapheresis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Vilayet et al.)

We herewith describe a patient with acute confusion, expressive aphasia and generalized seizures. A through workup excluded most causes of encephalopathy. He was, however, found to have TSH = 18.6 MIU/ml, T3reverse = 0.44nmol/L, T4 = 0.8ng/dl and Anti-Thyroid-Peroxidase AB titer >1000 IU/ml. Based on the above findings the patient was diagnosed with Hashimoto's encephalopathy and his mental status showed dramatic improvement (MMS 30/30) with high dose prednisone. Hashimoto's encephalopathy is rare disorder of presumed autoimmune origin characterized by cognitive decline, seizures, neuro-psychiatric symptoms, high titers of Anti-Thyroid-Peroxidase AB, and a positive response to steroids.

A large proportion of "encephalitis" is caused by unknown agents. Of late, a new category of disorders, "autoimmune encephalitis," has been described, which present with features similar to viral encephalitides. A well-delineated and common entity among this group is the recently described anti-NMDAR encephalitis (NMDARE). Although this entity was initially described in young women harboring ovarian teratomas, it is now characterised as well in children and men. Approximately 60% of the patients have an underlying tumor, usually an ovarian teratoma. In 40% of the patients, no cause can be found (idiopathic NMDARE). NMDARE typically presents with psychiatric features followed by altered level of consciousness, severe dysautonomia, hyperkinetic movement disorders, seizures and central hypoventilation. Orofacial dyskinesias resulting in lip and tongue mutilation are quite common. Seizures, are common and may be difficult to treat. The disease can be confirmed by serum and cerebrospinal fluid anti-NMDAR antibodies. Titers of these antibodies can also guide response to treatment. Tumor removal is necessary if identified, followed by immunological treatment. Intravenous methylprednisolone and immunoglobulins aim to suppress/modulate immune response while plasma exchange attempts to remove antibodies and other inflammatory cytokines. Rituximab and cyclophosphamide aim to suppress antibody production. Recovery is slow and often with neurological deficits if treatment is delayed. With many distinctive clinical features, a specific antibody that aids diagnosis, and early effective treatment with commonly available drugs leading to good outcomes, NMDARE is a diagnosis that should be considered early in any case of "unexplained encephalitis."

Autoimmune encephalitis (AE) is a newly defined group of disorders characterized by psychiatric symptoms such as psychosis and manic or hypomanic symptoms, with or without neurological symptoms. The most common neurological symptoms include seizures, altered mental status, autonomic disability, disorientation, and movement disorders. Our case report describes a type of AE caused by circulating autoantibodies against voltage-gated potassium channels (VGKC), which has not been reported before in the United Arab Emirates. This case report describes the psychiatric manifestations in a 17-year-old female with AE. It aims to shed light on the rare presentations of AE, discuss the various causes and management in greater depth, and the importance of suspecting and diagnosing AE early in the illness course. This rare case highlights the need for further research on the underlying biological, psychological, and social risk factors for developing AE in this region, and to direct further attention to developing early-intervention strategies in the vulnerable patient population., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Hasan et al.)

Background Status epilepticus (SE) is a neurological emergency associated with a high mortality rate and long-term cognitive sequelae. Status epilepticus in pregnancy poses a tremendous threat to both mother and fetus, making a correct diagnosis and treatment a challenging task for clinicians. The prevalence, underlying etiology, and outcomes of pregnancy-related SE remain largely unknown. Methods We retrospectively studied all SE episodes (n = 366) in patients admitted to our neurological ICU over a period of 8.5 years. The patients who developed SE during pregnancy and within 6 months after delivery were considered to have pregnancy-related SE. Patients with eclampsia were not included as they were usually cared for in our obstetric unit. Results Seven patients with pregnancy-related SE were identified (2.1% of all cases of SE), with the majority (85%) occurring de novo except for one patient who had a previous history of epilepsy-related SE due to withdrawal of antiepileptic medication. In terms of etiology, limbic encephalitis and cerebral venous sinus thrombosis were the two main etiologies of de novo SE associated with pregnancy. The overall mortality rate was 28.5% at discharge, and poor outcomes were especially noted in the patients with limbic encephalitis compared to other etiologies. Conclusions Pregnancy-associated SE is rare and predominantly occurs in patients without a history of epilepsy. An autoimmune etiology should be considered in pregnant patients with de novo SE, which was associated with poor outcomes. Thorough investigations and prompt treatment according to the etiology may be required to improve the final outcomes of both mother and fetus. [ABSTRACT FROM AUTHOR]

Background: Hashimoto encephalopathy is a rare form of encephalopathy thought to be of autoimmune etiology. Cognitive changes and seizures are the most commonly reported presenting manifestation. Stroke-like episodes have also been documented in these individuals. We describe a rare adolescent with Hashimoto encephalopathy who presented with stroke-like symptoms.Patient Description: A previously well 15-year-old girl experienced sudden-onset language disturbance and right hemiparesis. Her symptoms resolved, but weeks later, she began experiencing refractory seizures, episodes of status epilepticus, and cognitive decline. An extensive evaluation was unremarkable, but thyroid peroxidase antibodies were elevated, and a diagnosis of Hashimoto encephalitis was made. Steroid therapy was initiated, and her symptoms resolved with return to baseline cognitive function.Conclusion: Hashimoto encephalopathy is a highly treatable condition that may be considered in the differential diagnosis of children and adolescents presenting with stroke-like symptoms. [ABSTRACT FROM AUTHOR]

Cases of severe central nervous system (CNS) complications have been reported in relation to coronavirus-19 (COVID-19). Cases of encephalitis have been reported primarily in older patients with multiple comorbidities. We present a case of encephalitis in a young female patient with a history of chronic marijuana use that presented with nausea, vomiting, and acute altered mental status. Extensive testing for infectious and autoimmune causes of encephalitis were negative, except for a positive COVID-19 test. She was treated with steroids and intravenous immune globulin (IVIG) and improved with residual mutism., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Kim et al.)

Over the last 15 years it has become clear that rare but highly recognizable diseases of the central nervous system (CNS), including newly identified forms of limbic encephalitis and other encephalopathies, are likely to be mediated by antibodies (Abs) to CNS proteins. The Abs are directed against membrane receptors and ion channel-associated proteins that are expressed on the surface of neurons in the CNS, such as N-methyl D-aspartate receptors and leucine-rich, glioma inactivated 1 protein and contactin-associated protein like 2, that are associated with voltage-gated potassium channels. The diseases are not invariably cancer-related and are therefore different from the classical paraneoplastic neurological diseases that are associated with, but not caused by, Abs to intracellular proteins. Most importantly, the new antibody-associated diseases almost invariably respond to immunotherapies with considerable and sometimes complete recovery, and there is convincing evidence of their pathogenicity in the relatively limited studies performed so far. Treatments include first-line steroids, intravenous immunoglobulins, and plasma exchange, and second-line rituximab and cyclophosphamide, followed in many cases by steroid-sparing agents in the long-term. This review focuses mainly on N-methyl D-aspartate receptor- and voltage-gated potassium channel complex-related Abs in adults, the clinical phenotypes, and treatment responses. Pediatric cases are referred to but not reviewed in detail. As there have been very few prospective studies, the conclusions regarding immunotherapies are based on retrospective studies. [ABSTRACT FROM AUTHOR]

Background: Hashimoto encephalopathy (HE) is a poorly understood and often misdiagnosed entity with variable clinical spectrum. There are many uncertainties that still remain about this condition and the pathological significance of thyroid peroxidase (TPO) antibody. Objective: To characterize the clinical, laboratory and radiologic findings in patients with HE. Design: Retrospective analysis of clinical features and diagnostic test data. Main Outcome Measures: Clinical features, laboratory, radiologic, electroencephalography (EEG) findings associated with HE and therapeutic outcome. Results: Thirteen consecutive patients were identified as having HE. The median age at onset was 48.5 years (range, 19-62 years). There was a female preponderance (76.9%). Clinical manifestations were cognitive impairment and behavioral changes in 10 (76.9%), sleep disturbance in 9 (69.2%), seizures in 6 (46.1%), headache in 4 (30.8%), psychosis or paranoia in 5 (38.5%), transient symptoms in 6 (46.1%), myoclonus in 4 (30.8%), ataxia or gait disorder in 4 (30.8%). The most frequent laboratory abnormalities were increased TPO (n = 13) in all cases, increased thyroid stimulating hormone levels (n = 6), and increased erythrocyte sedimentation rate (n = 5). The cerebrospinal fluid protein level was elevated in 8 of 9 patients (88.8%). Magnetic resonance imaging abnormalities were present in 2 patients (15.4%). EEG changes were seen in 7 patients (53.8%). All but two patients showed significant therapeutic benefit with steroids. Conclusions: HE has a wide range of clinical, laboratory, and radiologic findings. All patients with an unexplained encephalopathy should be screened for this condition as treatment response is excellent. To the best of our knowledge, this is the largest single center clinical series of HE from the Indian subcontinent. [ABSTRACT FROM AUTHOR]

Encephalitis, an inflammatory condition of the brain that results in substantial morbidity and mortality, has numerous causes. Over the past decade, it has become increasingly recognized that autoimmune conditions contribute significantly to the spectrum of encephalitis causes. Clinical suspicion and early diagnosis of autoimmune etiologies are of particular importance due to the need for early institution of immune suppressive therapies to improve outcome. Emerging clinical observations suggest that the most commonly recognized cause of antibody-mediated autoimmune encephalitis, anti- N-methyl- d-aspartate (NMDA) receptor encephalitis, may in some cases be triggered by herpes virus infection. Other conditions such as Rasmussen's encephalitis (RE) and febrile infection-related epilepsy syndrome (FIRES) have also been posited to be autoimmune conditions triggered by infectious agents. This review focuses on emerging concepts in central nervous system autoimmunity and addresses clinical and mechanistic findings linking autoimmune encephalitis and infections. Particular consideration will be given to anti-NMDA receptor encephalitis and its relation to herpes simplex encephalitis. [ABSTRACT FROM AUTHOR]