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Rationale: Hashimoto thyroiditis (HT), a common cause of hypothyroidism, has shown an increasing incidence in recent years, particularly among women. In addition to the common complications such as lipid metabolism disorders, patients with HT may also experience some serious complications, acute kidney injury and severe muscle damage for instance. This article explored the effectiveness of levothyroxine sodium tablets (L-T4) replacement therapy in severe complications of hypothyroidism, including treatment dosage, duration of complication recovery, and whether additional treatment is needed., Patient Concerns, Diagnoses, and Interventions: We described a case of a 52-year-old woman with HT who exhibited kidney injury, muscle injury, and lipid metabolism disorders. The increased levels of serum creatinine, creatine kinase, cholesterol, triglyceride, low density lipoprotein cholesterol, high density lipoprotein cholesterol, and the decreased levels of estimated glomerular filtration rate were obviously observed. This patient was started on L-T4 (75 and 100 µg, alternate)., Outcomes and Lessons: Following a two-month treatment, the serum creatine kinase level decreased to within normal range. The estimated glomerular filtration rate level was restored, and the serum creatinine level was down-regulated, although slightly higher than the normal range. L-T4 partially reversed HT-induced the disorders of muscle, renal function, and lipid profile of this patient and remarkably alleviated her HT-related symptoms., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Hashimoto thyroiditis (HT) is a common autoimmune thyroid disease for which there is no specific treatment. Oral levothyroxine sodium tablets significantly improved thyroid function but did not promote a reduction in thyroid-related antibody concentrations. Acupuncture can improve clinical symptoms and thyroid function in HT patients, reduce serum TPOAb and TGAb levels in HT patients, and improve patients' quality of life., Methods: We conducted a systematic review and meta-analysis to evaluate the effect of acupuncture versus levothyroxine sodium tablets on Hashimoto thyroiditis. We searched Web of Science, Embase, China National Knowledge Infrastructure, WanFang, VIP, SinoMed and the Cochrane Central Registry of Controlled Trials to identify candidate randomized controlled trials (RCTs)., Results: A total of 1020 patients participated in 14 randomized controlled trials. The results of meta-analysis showed that acupuncture regulated TPOAb content (mean difference [MD] = -63.18, 95%CI = -91.73 to -34.62, P < .00001), TGAb content (MD = -68.56, 95%CI = -101.55 to -35.57, P < .00001), serum free triiodothyronine (FT3) content (MD = 0.74, 95%CI = 0.20 to 1.27, P < .00001), serum free thyroxine (FT4) content (MD = 1.10, 95%CI = 0.29 to 1.92, P < .00001), TSH content (MD = -2.16, 95%CI = -3.14 to -1.19, P < .00001) had a significant effect., Conclusion: Compared with levothyroxine sodium tablets alone, acupuncture can significantly regulate the contents of TPOAb, TGAb, FT3, FT4 and TSH., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Hashimoto's thyroiditis (HT) is the prevailing form of autoimmune thyroiditis and the leading cause of hypothyroidism in iodine-sufficient regions worldwide. This study aims to evaluate the efficacy of vitamin D supplementation on HT through a meta-analysis of randomized controlled trials (RCTs)., Methods: The databases searched included PubMed, and others. We included RCTs that the treatment group received vitamin D, while the control group received either a placebo or no treatment. The studies measured the baseline and endpoint levels of 25-hydroxyvitamin D [25(OH)D], thyroid-stimulating hormone (TSH), free thyroxine (FT4), free triiodothyronine (FT3), anti-thyroid peroxidase antibody (TPO-Ab), and thyroglobulin antibody (TG-Ab). We performed a meta-analysis to calculate the standardized mean difference (SMD) and 95% confidence interval (CI)., Results: A total of 12 studies involving 862 individuals were included. Vitamin D supplementation has a significant impact on reducing the titers of TPO-Ab (SMD = -1.084, 95% CI = -1.624 to -0.545) and TG-Ab (SMD = -0.996, 95% CI = -1.579 to -0.413) in patients with HT, and it also improves thyroid function by decreasing TSH level (SMD = -0.167, 95% CI = -0.302 to 0.031) and increasing FT3 (SMD = 0.549, 95% CI = 0.077-1.020) and FT4 (SMD = 0.734, 95% CI = 0.184-1.285) levels. Active vitamin D (calcitriol) significantly reduces the titer of TPO-Ab compared to naive forms of vitamin D (vitamin D2 or D3); treatment durations > 12 weeks result in a more effective reduction of TPO-Ab levels and a more significant increase in FT4 and FT3 levels in patients with HT (meta-regression P < .05)., Conclusion: Vitamin D supplementation may have beneficial effects on HT patients by modulating immune responses and improving thyroid function., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Encephalopathy can be associated with autoimmune disorders such as autoimmune thyroiditis, and it can present with a wide range of neuropsychiatric manifestations. However, it rarely presents with catatonia. We present the case of a middle-aged female with Hashimoto's thyroiditis presenting with catatonia. A literature review of previous similar cases highlighting significant points is also included. A 48-year-old female presented to the emergency department with catatonic symptoms that had worsened over the previous 5 days. A similar condition was reported to have occurred and resolved spontaneously 3 months earlier. On examination, the patient appeared uncooperative and unresponsive. She showed typical symptoms of catatonia, with a score of 21 points on the Bush-Francis Catatonia Rating Scale. Routine tests were within normal ranges except for an elevated level of C-reactive protein and an elevated erythrocyte sedimentation rate. Computed tomography, magnetic resonance imaging, and cerebrospinal fluid analysis were all normal. An electroencephalogram showed diffuse delta-theta range slowing with no epileptiform discharges. Lorazepam was initiated but did not control the catatonic symptoms. Re-evaluation revealed thyroid swelling and elevated levels of thyroperoxidase antibodies. IV methylprednisolone was therefore initiated and produced complete resolution of the catatonic symptoms in 4 hours. The patient was discharged and prescribed prednisone 1 mg/kg daily. At follow-up, the patient continued to show complete resolution of the catatonic symptoms. It is noteworthy that the patient developed hypothyroidism 6 months after this catatonic episode for which levothyroxine 50 mcg/d was prescribed. Encephalopathy associated with autoimmune thyroiditis can initially present with catatonic symptoms in euthyroid cases. The mainstay of treatment is steroids which result in complete resolution of the catatonic symptoms., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Evidence suggests that selenium supplementation could be useful in the treatment of Hashimoto thyroiditis (HT), but the available trials are heterogeneous. This study investigates clinically relevant effects of selenium supplementation in patients with HT., Methods: A systematic search was performed in PubMed, Web of Science, EMBASE, Scopus, and the Cochrane Library. The latest update was performed on December 3, 2022. We investigated the changes in thyroid peroxidase antibodies (TPOAb) and thyroglobulin antibodies (TgAb) after selenium supplementation. The effect sizes were expressed as weighted mean difference (WMD) with 95% confidence intervals (CIs)., Results: After screening and full-text assessment, 7 controlled trials comprising 342 patients were included in the systematic review. The results showed that there was no significant change in TPOAb levels (WMD = -124.28 [95% CI: -631.08 to 382.52], P = .631, I2 = 94.5%) after 3 months of treatment. But there was a significant decrease in TPOAb levels (WMD = -284.00 [95% CI: -553.41 to -14.60], P < .05, I2 = 93.9%) and TgAb levels (WMD = -159.86 [95% CI: -293.48 to -26.24], P < .05, I2 = 85.3%) after 6 months of treatment., Conclusions: Selenium supplementation reduces serum TPOAb and TgAb levels after 6 months of treatment in patients with HT, but future studies are warranted to evaluate health-related quality or disease progression., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Immune thrombocytopenia (ITP) is one of the most commonly acquired bleeding diseases in children. Infection and autoimmune disorders are the most common causes of ITP. The pathogenic mechanism of ITP is complex and is not completely understood. Understanding the underlying causes or disorders of ITP will improve the prognosis and make therapy more targeted., Patient Concerns: An 8-year-old girl with ITP responded poorly to first- and second-line treatment. The patient showed multiple scattered petechiae, ecchymoses, and purpura in the skin and blood clots in the oral mucous membrane., Diagnoses: The patient was diagnosed with ITP associated with Hashimoto thyroiditis., Interventions: The patient was admitted to our emergency department and received platelet transfusion, IVIG, glucocorticoids and eltrombopag. The patient's thrombocytopenia resolved within 18 days after the administration of levothyroxine treatment., Outcomes: The patient was diagnosed with Hashimoto thyroiditis, and the platelet count recovered on the 3rd day of levothyroxine treatment. The platelet count became steadily normal with levothyroxine and prednisone treatment within 2 months of follow-up., Lessons: Early identification of the underlying reasons and treatment with multiple modalities may be useful in improving the prognosis of ITP. The treatment of thyroid disease and restoration of the euthyroid state impact the clinical outcome of ITP in children., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: This review aims to bring together recent advances in basic, translational and clinical research on the pathogenesis and treatment of orbital inflammatory conditions., Recent Findings: Basic science studies provide mechanistic insights into why the orbit is targeted for inflammation by autoimmune inflammatory disorders. Using Graves' disease as a test case reveals that endocrine pathways, such as the TSH and IGF1 receptor pathways play important roles in stimulating orbital inflammation. Furthermore, orbital tissues contain high concentrations of retinoids - byproducts of the visual pathway that diffuse across the sclera and can activate de novo transcription of inflammatory cytokines. Such cytokine expression places the orbit in a hyper-inflammatory 'resting' state, prone to respond to any additional systemic or local pro-inflammatory signals. The HIF2A--LOX pathway appears important for orbital tissue fibrosis. Lastly, bench-to-bedside studies of the IGF1R pathway have led to an FDA-approved drug, teprotumumab that represents a novel treatment approach for Graves' orbitopathy. Unfortunately, high drug costs and misplaced insurance company 'step-therapy' policies may block patients from receiving therapy that can protect vision and improve quality of life., Summary: Improved understanding of orbital inflammatory conditions has led to a new drug and promises additional breakthroughs. Translational research is successful, but requires time, resources, and patience., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: Autoimmune encephalitides are established diagnoses in contemporary neurology. Their management poses a regular challenge for almost all neurologists. One may ask if the concept of 1st line and 2nd line treatment is still up to date, which new data on the antibody-defined encephalitis types exist, and how to organize long-term management., Recent Findings: The 1st line/2nd line concept of initial immunological intervention is accepted worldwide. A randomized controlled trial confirmed that one 1st line compound (intravenous immunoglobulins) is superior to a placebo in patients with antibodies against leucine-rich glioma inactivated protein 1. Rituximab, a 2nd line compound, is increasingly and apparently successfully used in treating different types of autoimmune encephalitis. It may find its place even earlier in the treatment cascade. Long-term management needs to be improved and is under development., Summary: There have been no groundbreaking new developments in the field. The published experience confirms existing suggestions. Aspects of long-term management including rehabilitation measures and counseling about driving eligibility require further research., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Hashimoto's Thyroiditis (HT) is one of the common autoimmune diseases, which can lead to thyroid reduction, increase the risk of tumor, and seriously affect women's reproductive health. Many other autoimmune diseases are easy to occur, seriously harming people's health.large dose herb Prunella or compound prescription contain large dose Prunella for treatment of HT has already been confirmed. However, due to the lack of evidence, there is no specific method or suggestion, it is necessary to carry out a systematic evaluation on Prunella and provide effective evidence for further research., Methods and Analysis: The following databases will be searched from their inception to October 2020: Electronic database includes PubMed, Embase, Cochrane Library, Web of Science, Nature, Science online, Chinese Biomedical Database WangFang, VIP medicine information, and China National Knowledge Infrastructure., Main Results: serum thyroid peroxidase antibody (TPOAb), thyroid globulin antibody (TGAb), other results: serum thyroid stimulating hormone (TSH), serum free triiodothyronine (FT3), serum free thyroid hormone (FT4). Data will be extracted by 2 researchers independently, risk of bias of the meta-analysis will be evaluated based on the Cochrane Handbook for Systematic Reviews (SR)of Interventions. All data analysis will be conducted by data statistics software Review Manager V.5.3. and Stata V.12.0., Results: The results of this study will systematically evaluate the efficacy and safety of large dose prunella salicorrhizae in the intervention of people with HT., Conclusion: The systematic review of this study will summarize the current published evidence of large dose prunella for the treatment of HT, which can further guide the promotion and application of it., Ethics and Communication: This study is a systematic review, the outcomes are based on the published evidence, so examination and agreement by the ethics committee are not required in this study. We intend to publish the study results in a journal or conference presentations.Open Science Fra mework (OSF) registration number:October 21, 2020.osf.io/fcyqp. (https://osf.io/fcyqp).

Rationale: Anti-gamma-aminobutyric-acid B receptor (anti-GABAB R) encephalitis is clinically characterized by seizures, cognitive disorders, and behavioral changes. Most patients are diagnosed with small-cell lung carcinoma., Patient Concerns: The patient suffered from a repeated grand mal seizure lasting for 10 minutes, intermittent speech vagueness, manic at night, and mental disorder., Diagnosis: The patient was diagnosed with autoimmune encephalitis. The gamma-aminobutyric-acid B(GABAB) receptor antibody test result was positive. After a bronchoscopic biopsy, the patient was diagnosed with small-cell lung carcinoma., Interventions: The patient was administered with intravenous immunoglobulin and Methylprednisolone. Etoposide was used after the small-cell lung carcinoma was diagnosed., Outcomes: After immunotherapy, following the 4 months of Etoposide and antiseizure treatment, the neurology examination revealed a remarkable improvement. MRS score reduced from 5 to 1. Electroencephalogram (EEG) recovered to normal from an extreme delta brush (EDB) electroencephalographic-pattern., Conclusion: Immunotherapy and Etoposide can improve the outcome of severe anti-γ-aminobutyric acid B receptor encephalitis with small-cell lung carcinoma. After immunotherapy and antineoplastic therapy, Electroencephalogram (EEG) can be recovered to normal from an extreme delta brush.

Objective: To report the presenting syndromes and to determine whether pretreatment criteria of Hashimoto encephalopathy (HE) predict response to steroids., Methods: We assessed symptoms and steroid responsiveness in 24 patients with pretreatment criteria of HE, including (1) subacute onset of cognitive impairment, psychiatric symptoms, or seizures; (2) euthyroid status or mild hypothyroidism; (3) serum thyroid peroxidase antibodies (TPOAb) >200 IU/mL; (4) absent neuronal antibodies in serum/CSF; and (5) no other etiologies. Additional studies included determination of TPOAb (>200 IU/mL) in 74 patients with criteria of possible autoimmune encephalitis (AE) without neuronal antibodies and 205 patients with different neuroimmunologic diseases, psychosis, or new-onset refractory status epilepticus (NORSE). Serum antibodies to the amino (ΝΗ2)-terminal of α-enolase (NH2-α-enolaseAb) were examined in the indicated 24 patients and 13 controls., Results: The 24 patients (14 women) with suspected HE had a median age of 48 years (range 8-79 years). Four syndromes were identified: psychiatric (7, 29%), encephalopathy (7, 29%), NORSE-like (6, 25%), and limbic encephalitis (4, 17%). Only 6 of 19 (31.6%) patients completely responded to steroids. The frequency of TPOAb in the 74 patients with possible AE (6 of 74, 8.1%) was similar to that of the 205 controls (17 of 205, 8.2%; p = 0.84). NH2-α-enolaseAb were identified in 1 of 24 suspected HE cases and 1 of 13 controls., Conclusion: Current pretreatment criteria of HE do not predict steroid responsiveness. The detection of TPOAb across all control groups reveals their poor disease-specificity. NH2-α-enolaseAb did not help in the diagnosis of HE. These findings imply a redefinition of HE that requires a systematic exclusion of antibody-mediated encephalitis., (© 2019 American Academy of Neurology.)

Rationale: Anti-IgLON5 disease is a complex neurological illness which is characterized by progressive sleep and movement disorders and defined by specific autoantibodies to IgLON5. We here describe the first case of a patient with coexisting anti-IgLON5 as well as anti-γ-aminobutyric acid B (GABAB)-receptor antibodies and predominant clinical features of anti-IgLON5 disease., Patient Concerns: The patient initially presented with subacute symptoms of severe sleep disorder, gait stability, dysarthria, cognitive impairment, depressive episode and hallucinations., Diagnoses: The patient was diagnosed with autoimmune encephalitis, based on clinical features and positive anti-IgLON5 antibodies in serum as well as in cerebrospinal fluid and anti-GABAB-receptor antibodies in serum only., Interventions: Initially, the patient was treated with high dosages of methylprednisolone and subsequently with plasmapheresis. Due to the lack of clinical improvement immunosuppressive treatment with intravenous cyclophosphamide was initiated., Outcomes: Following the first year of cyclophosphamide treatment, neurological examination revealed an improvement in gait instability, visual and acoustic hallucinations and sleep disorder., Lessons: The case report demonstrates that anti-IgLON5 and anti-GABAB-receptor antibodies can coexist in the same patient whereas clinical leading symptoms are determined by those antibodies that were tested positive in cerebrospinal fluid.

Purpose of Review: This article reviews epilepsy emergencies, including status epilepticus, acute repetitive seizures, autoimmune encephalitis, and the current perspective on their diagnosis and treatment., Recent Findings: Recent guidelines on the treatment of status epilepticus from the Neurocritical Care Society in 2012 and the American Epilepsy Society in 2016 highlight areas of consensus in the treatment of status epilepticus as well as areas of uncertainty. The TRENdS (Treatment of Recurrent Electrographic Nonconvulsive Seizures) trial is the first prospective randomized clinical trial to evaluate the efficacy of IV antiseizure medications in controlling nonconvulsive seizures on continuous EEG. It demonstrated that IV lacosamide is noninferior to fosphenytoin in this setting. Autoimmune encephalitis is an increasingly recognized cause of new-onset seizures or status epilepticus. Recently described scoring systems, the Antibody Prevalence in Epilepsy score and the Response to Immunotherapy in Epilepsy score, can help in the assessment of autoimmune encephalitis., Summary: Status epilepticus, acute repetitive seizures, and autoimmune encephalitis are neurologic emergencies. For all these conditions, rapid and appropriate treatment may influence patient prognosis and mitigate neuronal injury. For convulsive status epilepticus, there is reasonable consensus on the initial steps that need to be taken. There is less agreement about the management of acute repetitive seizures and nonconvulsive status epilepticus. An increasingly recognized etiology of status epilepticus is autoimmune encephalitis, which may not be as rare as previously thought.

Rationale: A sellar mass in children is most often seen in craniopharyngeal tumors, intracranial germ cell tumors, or pituitary adenomas. However, pituitary hyperplasia secondary to primary hypothyroidism (PHPH) is not commonly seen in children., Patient Concerns: A 10-year-old girl was admitted due to growth retardation and obesity for 4 years. On physical examination, the patient had a height of 118 cm, body weight of 46 kg, body mass index (BMI) of 33.0 kg/m., Diagnoses: After magnetic resonance imaging (MRI) and laboratory tests, her initial diagnosis was Hashimoto's thyroiditis, primary hypothyroidism, and reactive pituitary hyperplasia., Interventions: She was treated with oral L-thyroxine tablets., Outcomes: After 6 months, physical examination showed a height of 125 cm, weight of 36 kg, BMI of 23.0 kg/m. She developed well, with 12 cm of yearly growth thereafter., Lessons: The diagnosis of PHPH in a child is very important and sometimes difficult. Based on the summary and analysis of previous cases, we can learn that the main manifestations of PHPH include growth arrest and obesity, perhaps accompanied by symptoms caused by a decreased thyroid hormone concentration and elevated prolactin (PRL) concentration. Intracranial MRI shows diffuse enlargement of the anterior lobe of the pituitary gland, with a dome-shaped blunt edge change. Thyroid hormone levels may decrease, whereas the thyroid stimulating hormone (TSH) level increases, commonly accompanied by an elevated PRL, reduced growth hormone (GH) levels, and positive findings of TPOAb and TGAb. Improvement of symptoms and the normalization of hormone levels as well as restoration of pituitary size can be achieved after treated with thyroid hormone replacement therapy. And a hasty decision on surgical resection should be avoided when the diagnosis is uncertain.

Rationale: Autoimmune encephalitis and Lambert-Eaton myasthenic syndrome are classic paraneoplastic neurological conditions common in patients with small cell lung cancer., Patient Concerns: The patient complained of tiredness, fluctuating recent memory loss, and inability to find his home. His family members reported a change in character, irritability, and paranoia. One month later, the patient had 1 grand mal seizure lasting 5 minutes., Diagnosis: The patient was diagnosed with limbic encephalitis combined with Lambert-Eaton myasthenic syndrome. The gamma-aminobutyric acid B (GABAB) receptor and collapsin response mediator protein 5 (CRMP5, also called CV2) antibody test results were positive. Nine months after the onset of symptoms, the patient was diagnosed with small cell lung cancer., Interventions: The patient was administered intravenous immunoglobulin for 5 days. He was then treated with 60 mg prednisone once per day. The prednisone dose was gradually reduced by 1 tablet every 2 weeks. After the diagnosis, the patient underwent 6 courses of chemotherapy with cisplatin combined with sequential chemoradiation therapy., Outcomes: The patient was able to take care of himself. Neurological examination revealed a lower limb proximal muscle strength level of 4 and a reduced limb tendon reflex. The patient had deficits in short-term memory, a Mini-Mental State Examination score of 26, Montreal Cognitive Assessment score of 24, Self-rating Depression Scale score of 54 (mild depression), and Self-Rating Anxiety Scale score of 42 (normal)., Lessons: Autoimmune diseases of the peripheral and central nervous systems can be observed at the same time in patients with small cell lung cancer, even when magnetic resonance imaging findings are negative and immune therapy is effective.

The aim of this study was to evaluate the risk of depression in and effect of L-thyroxine therapy on patients with Hashimoto thyroiditis (HT) in Taiwan.In this retrospective, nationwide cohort study, we retrieved data from the Longitudinal Health Insurance Database 2000. We collected data of 1220 patients with HT and 4880 patients without HT for the period 2000 to 2011. The mean follow-up period for the HT cohort was 5.77 years. Univariate and multivariate Cox proportional hazards regression models were used to estimate the risk of depression in the HT cohort.In the HT cohort, 89.6% of the patients were women. Compared with the non-HT cohort, the HT cohort exhibited a higher prevalence of diabetes mellitus, hyperlipidemia, and coronary artery disease. Furthermore, the HT cohort showed a higher overall incidence of depression compared with the non-HT cohort (8.67 and 5.49 per 1000 person-year; crude hazard ratio [HR] = 1.58, 95% confidence interval [CI] = 1.18-2.13). The risk of depression decreased after administration of L-thyroxine treatment for more than 1 year (adjusted HR = 1.02; 95% CI = 0.66-1.59).In Taiwan, the overall incidence of depression was greater in the young HT cohort. L-thyroxine treatment reduced the risk of depression.

Objective: To describe and analyze etiologies, clinical presentations, therapeutic management, and outcomes of patients with acute encephalitis and identify outcome predictors., Methods: We conducted a retrospective review of patients diagnosed with acute encephalitis at Mayo Clinic Rochester between January 2000 and December 2012. On the basis of the final diagnosis, the patients were categorized into 3 groups: viral encephalitis, autoimmune encephalitis, and encephalitis of unknown/other etiology. Multivariate logistic regression analysis was used to analyze factors associated with good (modified Rankin Scale scores of 0-2) or poor (scores of 3-6) outcome at hospital discharge., Results: We identified 198 patients (100 [50.5%] men and 98 [49.5%] women) with a median age of 58 years (range 41.8-69). Etiologies included viral infection (n = 95, 48%), autoimmune (n = 44, 22%), and unknown/others (n = 59, 30%). Good outcome at discharge was achieved by 48 patients (50.5%) with viral encephalitis, 17 (40.5%) with autoimmune encephalitis, and 32 (54.2%) with unknown/other etiologies. Mortality rates were 8%, 12%, and 5% in each of the 3 categories, respectively. On multivariate regression analysis, the factors that were associated with poor outcome were age 65 years or older (p = 0.0307), immunocompromised state (p = 0.0153), coma (p = 0.0062), mechanical ventilation (p = 0.0124), and acute thrombocytopenia (p = 0.0329). CSF polymorphonuclear cell count was also strongly associated with poor outcome in viral encephalitis (p = 0.0027)., Conclusions: Advanced age, immunocompromised state, coma, mechanical ventilation, and acute thrombocytopenia portend a worse prognosis in acute encephalitis. In contrast, the etiology of encephalitis, development of seizures or focal neurologic deficit, and MRI findings were not associated with clinical outcomes., (© 2014 American Academy of Neurology.)

Membranous nephropathy is a common cause of nephrotic syndrome in adults. It usually occurs secondary to underlying disease processes such as autoimmune disorders, malignancy, infection, and drugs. The presentation of nephrotic syndrome with concomitant precipitous decline in renal function warrants investigation of a coexistent disorder.We report the case of a 30-year-old male who presented with symptoms and signs of hypothyroidism.A diagnosis of Hashimoto's thyroiditis was contemplated based on the presence of high serum levels of antithyroglobulin and antithyroid peroxidase antibodies. Upon initiation of treatment with levothyroxine, patient symptomatology improved; however, the laboratory studies demonstrated continued elevated creatinine, hematuria, and proteinuria, which had not been addressed. Two months following treatment initiation, he had progressive deterioration in renal function and proteinuria. A renal biopsy revealed coexistent necrotizing and crescentic glomerulonephritis and membranous nephropathy.The final diagnosis was necrotizing, crescentic glomerulonephritis with superimposed membranous nephropathy likely secondary to Hashimoto's thyrodiitis.Induction treatment with oral cyclophosphamide and prednisone was started.At the end of 6 months of treatment, there was improvement in renal function and proteinuria and maintenance treatment with azathioprine and low-dose prednisone was initiated. This case highlights the importance of precise and detailed evaluation of patients with autoimmune diseases such as Hashimoto's thyroiditis particularly in the presence of active urine sediment. Proper evaluation and diagnosis of such patients has implications on the prognosis and response to treatment.

We report a longstanding selective memory deficit in a euthyroid 45-year-old woman who was being treated with levothyroxine for Hashimoto thyroiditis. The patient had complained of memory problems and deterioration of her concentration skills for about 2 years. Her endocrinologist thought that she was depressed. The patient's physical examination was normal. She scored a full 30 points on the Mini-Mental State Examination, but neuropsychological evaluation showed a significant deficit in her verbal memory. Routine blood tests and cerebrospinal fluid analysis showed only antithyroid peroxidase antibodies. Brain magnetic resonance imaging was normal. Electroencephalogram showed scarce intermittent bilateral multifocal theta waves. We increased the patient's daily dose of levothyroxine and started her on dexamethasone therapy. Five months later, we repeated the entire evaluation and found both her cognitive function and her electroencephalogram to be normal. Autoimmune encephalopathy associated with Hashimoto thyroiditis is already known to present with either stroke-like episodes or diffuse progressive deterioration. Our patient shows that the encephalopathy can present as a chronic selective memory deficit that can spare executive functions and short-term memory. This presentation can be missed or mistaken for depression, but can be diagnosed with a detailed neuropsychological evaluation.

We report a case of dermatomyositis associated with rheumatoid arthritis, Hashimoto thyroiditis, and diabetes mellitus responsive only to combination of rituximab with mycophenolate. A 42-year-old woman presented with proximal muscle weakness, myalgias, fever, night sweats, and shortness of breath. Creatinine kinase was 8155 IU/L, and muscle biopsy was diagnostic of dermatomyositis. She was started on glucocorticoids; her systemic symptoms improved, but her muscle weakness persisted. She was serially treated with intravenous immunoglobulin, azathioprine, and mycophenolate mofetil without improvement in her weakness. She responded dramatically to combination therapy with rituximab and mycophenolate, with improvement in strength and normalization of creatinine kinase. She has been well controlled on rituximab infusion every 6 months and maintenance mycophenolate mofetil.

Introduction: Hashimoto encephalopathy is a rare encephalopathy with positive antithyroid antibodies and normal thyroid function. We describe the clinical manifestations, neuroimaging, and histopathologic changes in a 52-year-old female with Hashimoto encephalopathy., Results: The neuroimaging changes are multifocal abnormalities in cortical and subcortical areas bilaterally not enhanced by gadolinium. After high doses of corticosteroid therapy, the changes rapidly disappeared with marked improvement of clinical symptoms., Conclusions: This case shows the importance of assessment of antithyroid antibodies in patients with rapidly progressive cognitive dysfunction with/without the history of abnormality of thyroid function.

Acute febrile neutrophilic dermatosis (AFND; Sweet syndrome) is characterized by a constellation of symptoms and findings: fever, neutrophilia, and tender erythematous skin lesions that typically show an upper dermal infiltrate of mature neutrophils. Whereas some cases are idiopathic, others have been associated with a variety of disorders. In this report, we describe the occurrence of AFND with chronic lymphocytic thyroiditis (Hashimoto thyroiditis) and preexisting psoriasis. This is the first case report of the association of chronic lymphocytic thyroiditis with AFND from the United States and only the third reported in the world's literature. Because the coexistence of these disorders is rare, an underlying common pathogenic mechanism is a possibility. We postulate this to be CD4(+) T-cell dysfunction.

Introduction: Hashimoto encephalopathy (HE) is an immune-mediated encephalopathy associated with Hashimoto thyroiditis. Most patients with HE respond to corticosteroids. Diffuse or focal white matter changes suggesting primary demyelination on magnetic resonance imaging (MRI) has been reported in only a few patients with HE. Follow-up imaging studies have been sparse., Case Report: We report the case of a 48-year-old woman who presented with progressively declining cognitive function over 6 weeks without neurologic focal deficit on clinical examination. Her Mini-Mental State Examination Score was 3/30, and the MRI showed cortical and subcortical white matter demyelination. Biologic and radiologic investigations did not reveal an infective, vasculitic, or neoplastic etiology. Although her thyroid function tests were normal, thyroid antibodies were detected in high titers in her serum. A diagnosis of HE was made, and the patient was treated with high-dose corticosteroids. Over the next 8 weeks, her Mini-Mental State Examination Score improved to 24/30. The MRI changes showed resolution paralleling her clinical improvement., Conclusions: This case illustrates the importance of considering rare but treatable causes of encephalopathy in a patient presenting with acute or subacute cognitive decline.

Objective: Hashimoto encephalopathy (HE) is associated with numerous neuropsychiatric symptoms and responds well to steroid therapy. In the past, only a few cases were reported to present with pure psychiatric syndromes. We describe a case of HE with presenting symptoms like that of schizophrenic patients., Methods: We describe a 73-year-old woman with a history of autoimmune thyroiditis. She had psychotic symptoms for 3 years that responded poorly to antipsychotic agents, and she was thus admitted in 2007., Results: The diagnosis of HE was made, although the patient presented neurologic symptoms and signs including abnormal electroencephalography, recent memory impairment, and executive function declination. The psychotic symptoms subsided completely in a few days after high-dose intravenous steroid therapy., Conclusions: The neuropsychiatric manifestation of HE can be similar to typical schizophrenia. Considering the effectiveness of steroid therapy for HE, we suggested HE as an important differential diagnosis for psychotic disorders, particularly for those patients of late onset, with abnormal electroencephalography, history of autoimmune thyroiditis, or poor response to conventional psychiatric treatment, so as to provide prompt and effective treatment for these patients.

Introduction: Hashimoto's encephalopathy is an unusual condition that is associated with Hashimoto's thyroiditis. Myoclonus, epileptic seizures, dementia, and disturbances of consciousness are the most common features., Case Report: We present an atypical case of Hashimoto's encephalopathy in a 33-year-old woman who presented with several brief and acute psychotic episodes. After treatment with steroids, there was an improvement in the patient's psychiatric symptoms and electroencephalogram, and antithyroglobulin antibody titers returned to normal levels., Conclusions: It is our opinion that Hashimoto's encephalopathy should be considered in the differential diagnosis of atypical psychosis, especially because this is a treatable syndrome. This is particularly important in patients with a previous history of thyroid disease, despite current normal thyroid function.

A 56-year-old woman presented with an acute confusional state and moderate global aphasia. Thyroperoxidase antibody level was elevated (3,890 IU/mL) and SREAT was diagnosed. MRI findings were normal. Cerebrospinal fluid examination revealed only a mildly increased protein. The initial electroencephalogram EEG showed slowing and markedly decreased amplitude over the left hemisphere and left temporal sharp waves. An EEG performed after treatment with intravenous steroids showed a significant improvement of the background slowing, which correlated with clinical improvement. One week later, the patient had an episode of forced head-turning and fencing posture to the right. The EEG shortly afterward showed slowing and a decreased amplitude over the right hemisphere. Continuous EEG monitoring was performed at the time of steroid treatment. Again, there was a significant improvement of the EEG after this treatment that correlated with the clinical condition. SREAT is characterized by fluctuations in mental status and variable EEG findings. These patients often show an excellent clinical improvement to immunosuppressive therapy, including corticosteroids. This case report documents the dramatic clinical and EEG improvement with steroid therapy.

Multiple sclerosis (MS) occurs with immune-mediated mechanisms, but its pathogenesis is not accurately known. The coexistence of MS with other autoimmune diseases has been reported. The hypothesis that MS coexists with other autoimmune diseases has been supported by the reported association of MS with type I diabetes mellitus and inflammatory disorders. Even though there have been only rare reports of associations between Hashimoto thyroiditis and MS, this association is important for its clinical and therapeutic aspects. Proximal muscle weakness, myalgia, and fatigue are symptoms that are common in both MS and hypothyroidism. When MS patients demonstrate these symptoms, thyroid function tests should be performed. The thyroid hormone levels of MS patients being treated with interferon-beta and Campath-1H also should be monitored. The authors report the clinical data of 2 definite MS patients who also fulfilled criteria for Hashimoto thyroiditis.