Steroid-responsive Encephalopathy Associated With Autoimmune Thyroiditis Research Articles

Steroid responsive encephalopathy associated with autoimmune thyroiditis as a cause of acuteencephalopathy.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), known as Hashimoto's encephalopathy (HE), represents a heterogeneous group of neurological and neuropsychiatric symptoms associated with a presence of antithyroid antibodies in case of other causes of encephalopathy were excluded. Clinical symptoms most commonly includes acute onset of encephalopathy, behaviour changes and cognitive dysfunction, epileptic seizures as well as cerebellar and extrapyramidal symptoms. Corticoids provides rapid and sustained therapeutic benefit in most patients and only a few patients require other immunosuppressive therapy such as plasmapheresis, intravenous immunoglobulins, or others. We present the cases of two patients with acute onset of encephalopathy, status epilepticus based on SREAT, with rapid improvement after steroid treatment.

The use of intravenous immunoglobulin in the treatment of Hashimoto's encephalopathy: case based review.

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. The online databases PubMed and EMBASE were searched. 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. 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.

A Prospective Observational Study of Autoimmune Encephalitis in Northwestern India.

Autoimmune encephalitis (AIE) is a group of rare, increasingly recognized, potentially reversible, noninfectious causes of unexplained encephalitis. It affects any age-group and has a plethora of clinical presentations, the most common being the neuropsychiatric manifestation. The diagnosis of this entity at the right time and proper treatment with immunotherapy can save many lives. In this study, we describe the demographic profile, clinical spectrum, diagnosis, and treatment of 42 patients with features of AIE. This is a prospective study where 42 cases were selected from a tertiary care center in Northwestern India. Patients with suspected AIE underwent detailed clinical assessment, routine blood tests, magnetic resonance imaging (MRI) brain, electroencephalography (EEG), cerebrospinal fluid (CSF) study, and autoimmune profile in blood and CSF. Screening for malignancy was done in all patients with computer tomography (CT) thorax and abdomen and tumor markers. Among 42 patients, males, and females were almost equally affected. The mean age of onset was 31 years. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) Encephalitis was the commonest of all AIE (57%) followed by anti-leucine-rich glioma inactivated-1 (anti-LGI-1) related AIE (11.9%), anti-contactin-associated protein 2 (anti-CASPR2) related AIE (4.7%), and steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) related to antithyroid peroxidase (anti-TPO) antibody (2.3%). Neuropsychiatric manifestation is the commonest. The seizure was noted in around 72% of patients, the commonest in the anti-NMDAR group. Faciobrachial dystonic seizure (FBDS) was noted in all five anti-LG1-1 encephalitis patients. CSF abnormalities were seen in 33.3% of patients in the form of pleocytosis or raised protein, or both. MRI abnormality was seen in 52% of patients. EEG was abnormal in 10% of patients, and delta brush was noted in three anti-NMDAR patients. All patients received immunotherapy in the form of intravenous immunoglobulin (IVIg) or pulse IV methylprednisolone (IVMPS), or both. Two patients nonresponsive to IVIg and IVMPS received rituximab. Almost all patients responded to immunotherapy. Autoimmune encephalitis (AIE), a potentially treatable immune-responsive entity, is a common neurological problem and may be an answer to a large number of cases having unexplained encephalitis. Good clinical acumen and knowledge are required for early diagnosis and treatment of this potentially reversible disorder. How to cite this article: Sharma B, Paul M, Bagaria AK. A Prospective Observational Study of Autoimmune Encephalitis in Northwestern India. J Assoc Physicians India 2023;71(9):39-44.

Streaming through a case of SREAT

BackgroundHashimoto’s encephalopathy, also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune neuroendocrine disorder marked by impaired brain function. It is a diagnosis of exclusion with variable nature of presentation and no gold standard investigation of choice.Case presentationHere, we report a case of SREAT in a 26-year-old female who presented to our Emergency Department with altered sensorium and generalised tonic clonic seizures. After thorough clinical examination and initial resuscitation, a provisional diagnosis of neuroglycopenic injury or possible encephalitis was made. Broad-spectrum antibiotics were initiated. Routine investigations and cerebrospinal fluid (CSF) study were inconclusive except for neutrophilic leucocytosis. Magnetic resonance imaging (MRI) depicted hyper-intense signal changes around bilateral hippocampus and thalamus. Serum anti-thyroid peroxidase (anti-TPO) was strongly positive while other serum and CSF autoantibodies were within normal limits. A diagnosis of SREAT was made and she responded brilliantly to systemic corticosteroids. Incidentally, anti-SSA (anti-Ro) and anti-SSB (anti-La) were positive and a possible association between Sjogren’s syndrome and SREAT was insinuated.ConclusionThere is a long list of differentials for SREAT and a proper diagnostic criteria must be followed to reach at a conclusion. It can be easily missed and remain underreported due to its overlapping nature and ambiguous presentation. Hence, clinicians must have high index of suspicion for the disease and optimal therapy should be initiated early to improve the long term mortality.

Hashimoto's Encephalopathy: Case Series and Literature Review.

To describe the clinical manifestations of Hashimoto's encephalopathy (HE) and discuss its pathogenesis in light of recent research. The pathogenesis of HE is uncertain. Available evidences point towards an autoimmune etiology due to vasculitis or other inflammatory process. Detection of thyroid antibodies - antithyroid peroxidase and anti-thyroglobulin are essential for diagnosis. Autoimmune encephalitis including Anti-IgLON5 disease needs to be excluded in suspected cases with appropriate tests for neuronal surface antibodies. Detection of thyroid autoantibodies is nonspecific, as these can be detected in some normal individuals and in other autoimmune diseases. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels in younger males with very high levels of thyroid antibodies. The role of the thyroid autoantibodies in the central nervous system (CNS) tissue damage remains unclear and these can act only as markers for diagnosis. Conversely, they have a role to play in determining the thyroid pathology - more glandular fibrosis associated with thyro-peroxidase antibody than with the thyroglobulin antibody. HE is a syndrome characterized by altered mental status, confusion, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually responsive to glucocorticoid therapy. Diagnosis requires the exclusion of other causes of encephalopathies and encephalitis including autoimmune encephalitis associated with neuronal surface antibodies and paraneoplastic ones. Diagnosis also is dependent on the demonstration of thyroid autoantibodies in serum. Since there is no direct pathophysiologic link between antithyroid antibodies, Hashimoto thyroiditis and the cerebral syndrome, the nomenclature HE could be misleading. The response to steroids led to a renaming of the syndrome to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), though some cases do not respond to steroids. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels (IgG4-related disease). This is characterized by a higher incidence in men (5:1) than in women, onset at a younger age, more intense thyroid inflammation and higher antithyroid antibody titters. Such patients have excessive production of IgG4 + plasmacytes, which infiltrate various organs leading to their fibrosis and sclerosis, sometimes resulting in inflammatory tumors. HE is treated with corticosteroids along with treatment of the dysthyroid condition, if any. There are yet no guidelines regarding steroid dose and/or duration.

A Rare Presentation of Steroid-responsive Encephalopathy Associated with Autoimmune Thyroiditis with Neuropsychiatric Symptoms: A Case Report

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 mild-to-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.

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Masquerading as New-Onset Bipolar Disorder in an Elderly Female

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) Masquerading as New-Onset Bipolar Disorder in an Elderly Female

Two PerPLEXing Cases of Hashimoto’s Encephalopathy Unresponsive to Steroid and Intravenous Immunoglobulin Therapy

Hashimoto’s encephalopathy is a nebulous entity over which much controversy exists. Often referred to as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), it describes a myriad of neurological sequelae that typically are observed to occur in patients with the presence of thyroid antibodies. We aim to raise clinical awareness of this seldom diagnosed entity as a potential etiology for altered mental status in patients who present with supporting clinical features and elevated thyroid antibodies. While steroid responsiveness is deemed a typical presenting feature of this medical condition, our cases aim to describe two cases that required escalation of therapy to intravenous immunoglobulins, and ultimately, plasmapheresis therapy for improvement in their clinical status. Our patients had a dramatic improvement in their mentation within three to four sessions of plasmapheresis, improving rapidly toward their baseline. Such a dramatic improvement, coupled with the corresponding reduction in their thyroid antibody titer supports the diagnosis of Hashimoto's encephalopathy and highlights the importance of having a low clinical threshold for the diagnosis of this entity in patients who, despite extensive evaluation, reveal no apparent cause for their altered mental status.

Steroid-Responsive Encephalopathy Associated with Thyroiditis

steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune entity with a strong association with elevated antithyroid antibodies. It is a rare cause of encephalopathy and is usually a diagnosis of exclusion. Responsive to corticosteroids is required to make the diagnosis. Herein, we report a male patient presented with recurrent convulsive episodes not controlled well by anticonvulsant drugs and had drops in Glasgow coma scale (GCS). After unremarkable of extensive investigations, Hashimoto's encephalitis was suspected and antithyroid peroxidase antibodies test turned out to be positive, while thyroid function tests were normal and the diagnosis of steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) was made. He received a course of intravenous methylprednisolone for 5 days and responded very well to therapy, with an improvement of his GCS to 15/15.

Patients with a relapsing course of steroid-responsive encephalopathy associated with autoimmune thyroiditis exhibit persistent intrathecal CD4+ T-cell activation.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition defined by encephalopathy with acute or subacute onset, the presence of serum anti-thyroid antibodies, and reasonable exclusion of alternative causes. Despite having strong response towards corticosteroid treatment, some patients exhibit a chronic-relapsing course and require long-term immunosuppression. Markers for early identification of those patients are still absent. Thus, we aimed to characterise clinical as well as laboratory parameters of our local SREAT cohort. We retrospectively evaluated a cohort of 22 SREAT patients treated in our hospital from January 2014. A total of 14 patients with a monophasic disease course and eight patients with multiple relapses were identified. Neither baseline characteristics nor routine cerebrospinal fluid (CSF) parameters were able to distinguish between those patient groups. Flow cytometry following initial relapse therapy showed treatment-resistant sequestration of activated CD4+ T cells in patients with a relapsing disease course, whereas other lymphocyte subsets showed uniform changes. Such changes were also present in long-term follow-up CSF examination. Our findings indicate a potential biomarker for risk stratification in patients with SREAT. Currently, it remains unclear whether the observed two phenotypes are different spectra of SREAT or represent separate diseases in terms of pathophysiology.

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): A Case Report

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also known as Hashimoto’s encephalopathy, is a disorder characterized by an encephalopathy associated with anti-thyroid antibodies in the absence of alternative causes. It has a wide range of clinical, laboratory and radiological features. Objective: Cases of steroid-responsive encephalopathy associated with autoimmune thyroiditis are difficult to diagnose and requires a high index of suspicion, as this will determine the early timing of management and disease outcome. Patient and Methods: Here we present a case report of twenty-year-old woman presenting with neurological and psychological symptoms. She was found to have high anti-thyroid peroxidase antibodies (Anti-TPO) with normal thyroid function and negative screening for other etiologies of encephalopathy. Based on her presentation, she was diagnosed with steroid-responsive encephalopathy associated with autoimmune thyroiditis. Results: Patient was started on high dose steroids upon which she responded partially. Then she required the addition of immunotherapy in the form of Rituximab to improve her symptoms. Conclusion: After exclusion of other causes of encephalopathy, the presence of thyroid antibodies prompts the susp icion of SREAT and therefore the early initiation of steroids to improve patients’ outcome.

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): A Case Report

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also known as Hashimoto’s encephalopathy, is a disorder characterized by an encephalopathy associated with anti-thyroid antibodies in the absence of alternative causes. It has a wide range of clinical, laboratory and radiological features. Objective: Cases of steroid-responsive encephalopathy associated with autoimmune thyroiditis are difficult to diagnose and requires a high index of suspicion, as this will determine the early timing of management and disease outcome. Patient and Methods: Here we present a case report of twenty-year-old woman presenting with neurological and psychological symptoms. She was found to have high anti-thyroid peroxidase antibodies (Anti-TPO) with normal thyroid function and negative screening for other etiologies of encephalopathy. Based on her presentation, she was diagnosed with steroid-responsive encephalopathy associated with autoimmune thyroiditis. Results: Patient was started on high dose steroids upon which she responded partially. Then she required the addition of immunotherapy in the form of Rituximab to improve her symptoms. Conclusion: After exclusion of other causes of encephalopathy, the presence of thyroid antibodies prompts the susp icion of SREAT and therefore the early initiation of steroids to improve patients’ outcome.

SAT-465 Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis: Diagnosis or Distraction?

In 1966 a case of episodic encephalopathy with anti-thyroid antibody (ATAb) positive Hashimoto’s thyroiditis was first reported. Controversy persists regarding Hashimoto’s encephalopathy, now known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and any relationship with ATAb. There is no literature supporting an etiologic role of ATAb in SREAT. Nor has there been correlation shown between ATAb titer and severity of thyroid dysfunction or SREAT(1).A 56 yo female was admitted to a community hospital three times with visual hallucinations and slurred speech. Brain MRI, LP and standard tests including C3/C4, folate, B12, ANA and ANCA were unrevealing. Thyroglobulin antibodies (TgAb) and anti-thyroid peroxidase antibodies (TPOAb) were positive. Diagnosed with SREAT with relief of symptoms on high dose IV corticosteroids. Discharged on oral prednisone. Seven months later she began experiencing progressive proximal weakness of all extremities associated with exertional dyspnea, fatigue and weight gain. EMG studies indicated steroid-induced myopathy of the proximal extremities. After prednisone tapering, she was admitted to our institution with acute confusion, blurred vision, fever, tachycardia, tachypnea and hypoxemia.Examination showed Cushingoid features. Labs showed hyperglycemia, ANA (+), TPOAb (+), TgAb (+), low FreeT4, low T3, normal TSH and elevated IgG in the CSF. Brain MRI showed meningeal enhancement of parieto-occipital lobes and left tentorium. Empiric antibiotics started to cover infectious meningoencephalitis. Endocrinology consulted for iatrogenic Cushing’s syndrome with hyperglycemia, abnormal thyroid function tests (TFTs) with positive ATAb and evaluation for SREAT. We recommended tapering steroids and evaluation for infectious, autoimmune or paraneoplastic causes of encephalopathy, before presuming SREAT. Rheumatological and infectious work-up was negative. CT chest showed nodular densities and CT abdomen showed hepatic, splenic and right inguinal region masses. Biopsy of inguinal mass showed Diffuse Large B-cell Lymphoma confirmed on bone marrow biopsy. CSF flow cytometry was unrevealing, likely due to prior steroids. Leptomeningeal enhancement likely reflected leptomeningeal metastases. Treated with systemic R-CHOP, high dose MTX, intrathecal MTX, cytarabine and corticosteroid tapering. Complete remission was achieved with normalization of TFTs, resolution of her Cushingoid and encephalopathic features.This case illustrates SREAT as a diagnosis of exclusion. ATAbs may be positive in non-thyroid autoimmune disorders, other neurological conditions and 10-20% of the general population (2). B cell lymphoma with leptomeningeal metastasis caused encephalopathic features which improved with corticosteroids. Ascribing a diagnosis of SREAT led to delayed cancer diagnosis and treatment.

A young man with convulsive laughter.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) can manifest with a wide range of neurological and psychiatric symptoms. A previously healthy man in his late twenties was admitted several times over the course of half a year. He had acute episodes of reduced consciousness, involuntary movements and psychotic symptoms (e.g. hallucinations and delusions). Initial examinations were normal except for a positive urine drug screen (tetrahydrocannabinol), and the patient was diagnosed with cannabinoid intoxication. During the next admission cerebrospinal fluid analysis showed mild pleocytosis. Screening for anti-neuronal antibodies was negative, but anti-thyroid peroxidase antibodies were detected in serum and cerebrospinal fluid. He was successfully given steroid treatment on a tentative diagnosis of SREAT, but relapsed when the steroids were discontinued. After receiving a prolonged steroid treatment with gradual dose reduction over a year, he remains symptom-free 18months after treatment discontinuation. The diagnostic delay might have been mitigated with an earlier inclusion of neuroimmunological disorders in the differential diagnosis. Unexplained pleocytosis in the cerebrospinal fluid in the presence of paroxysmal neuropsychiatric symptoms should trigger an investigation that includes autoimmune encephalopathies.

AN UNUSUAL CAUSE OF ENCEPHALOPATHY: STEROID RESPONSIVE ENCEPHALOPATHY ASSOCIATED WITH AUTOIMMUNE THYROIDITIS (SREAT)

SESSION TITLE: Critical Care 4 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: SREAT is an autoimmune disease associated with anti-thyroid antibodies. Its features mimic the fatal Creutzfeldt-Jacob disease (CJD), but SREAT responds to steroids. We present a case of SREAT to increase physician awareness of an important fully reversible disease. CASE PRESENTATION: An 84-year-old woman with hypothyroidism was brought to our hospital due to decreased responsiveness for 1 day. Examination: BP: 75/42 mmHg, heart rate: 38 bpm, temperature: 33.8 ºC, minimal response to sternal rub, no focal neurological abnormalities. Chest X-ray: Small right pleural effusion. CT head: Chronic microvascular changes. ECG: Sinus bradycardia. Laboratory evaluation: WBC: 2.2 K/CUMM, mild hyponatremia and acute kidney injury. After initial resuscitation, we started IV fluids, high dose levothyroxine, antibiotics and hydrocortisone for myxedema coma and pneumonia. TSH and free thyroxine were normal, making myxedema coma unlikely and levothyroxine was discontinued. Encephalopathy did not improve and seizures developed. SREAT was considered due to rapid onset encephalopathy, seizures and prior hypothyroidism. Thyroid peroxidase antibody level was 1,021 IU/mL (normal < 4 IU/mL). EEG: Moderate diffuse slowing and triphasic waves. CSF: Normal protein and cell count. IV methylprednisolone 1 g/day was started, and on day 3, she became alert, oriented and started conversing. Subsequently, she developed uremic encephalopathy requiring hemodialysis due to worsening acute tubular necrosis (ATN), recurrent sepsis, and upper gastrointestinal bleed and expired despite aggressive ICU care. DISCUSSION: SREAT presents with acute/subacute encephalopathy consistently. Myoclonus, seizures, stroke like or psychiatric manifestations are associated features. Due to reversibility of SREAT, antithyroid antibodies should be ordered in acute encephalopathy syndromes associated with neuropsychiatric manifestations. Diagnosis is based on clinical features, high titer antithyroid antibodies and response to steroids. Most patients with SREAT have generalized slowing in EEG, but focal temporal or frontal slowing, triphasic waves and periodic sharp waves may be seen. 60% develop seizures. MRI is normal in 50%, but generalized cerebral atrophy, restricted diffusion, and increased T2 and flair white matter signal may be seen. Patients may be euthyroid, have subclinical or overt hypothyroidism, or rarely hyperthyroidism. CSF shows elevated protein in 85% of cases. 98% of patients with SREAT respond well to high dose steroids. CONCLUSIONS: SREAT is important to consider in unexplained encephalopathy, due to its excellent response to steroids. Though high dose steroids have excellent efficacy in SREAT, steroids are associated with adverse events such as GI bleed and sepsis. ATN in ICU patients has a mortality of > 60%, and high dose steroids increases risk of uremic bleeding. Early institution of hemodialysis may improve ATN outcome. Reference #1: Laurent, Charlotte, et al. “Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT): Characteristics, Treatment and Outcome in 251 Cases from the Literature.” Autoimmunity Reviews, vol. 15, no. 12, Dec. 2016, pp. 1129–33. CrossRef, https://doi.org/10.1016/j.autrev.2016.09.008. Reference #2: Lee, Susan W., et al. “Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) or Hashimoto’s Encephalopathy: A Case and Review.” Psychosomatics, vol. 52, no. 2, Apr. 2011, pp. 99–108. PubMed, https://doi.org/10.1016/j.psym.2010.12.010. Reference #3: Annals of Long-Term Care: Clinical Care and Aging. 2015;23(9):25-27. DISCLOSURES: No relevant relationships by Camelia Arsene, source=Web Response No relevant relationships by Maxwell Cretcher, source=Web Response no disclosure on file for E.M. Malitha Hettiarachchi; No relevant relationships by Katherine Kelley, source=Web Response No relevant relationships by Aditya Kotecha, source=Web Response No relevant relationships by Parker Latshaw, source=Web Response no disclosure on file for Sidrah Najam; no disclosure on file for Udorji Ogochukwu; no disclosure on file for Christina Rose

Cerebellopathy secondary to anti-peroxidase antibody-mediated toxicity. A special case of Hashimoto encephalopathy

ObjectiveThe aim of this case report is to highlight the importance of recognizing uncommon causes of cerebellar involvement. Case presentationA 45-year-old woman with no medical history who presented gait instability that appears suddenly and evolves rapidly in two weeks, causing frequent falls. Neurological examination revealed an inability to walk due to severe ataxia, accompanied by global hypotonia, appendicular dysmetria, opsoclonus and dysarthria. We studied this patient with cerebellar syndrome, obtaining as relevant findings global cerebellar atrophy in MRI (magnetic resonance imaging) and especially, considerably elevated levels of antibodies against thyroid peroxidase (TPO). The patient was treated with high doses of intravenous methylprednisolone for 5days. Six months after diagnosis and in treatment with low doses of prednisone, the patient is asymptomatic. ConclusionWe must considerer the cerebellar involvement secondary to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in a patient in whom other more frequent causes of isolated cerebellar disease have been ruled out.

An Unusual Patient with Hashimoto Thyroiditis, Features of Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis, and Concurrent Minimal Change Renal Disease

ABSTRACT Objective: To present an unusual patient with Hashimoto thyroiditis, encephalopathy with features of steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), and acute kidney injury secondary to minimal change renal disease. Methods: We present clinical, laboratory, imaging, electroencephalography, and renal histopathologic findings, and review the relevant literature for evaluation and treatment of SREAT. Results: A 53-year-old woman with a history of hypothyroidism on levothyroxine was admitted for progressive altered mental status and bizarre behavior for 3 weeks. On exam she was lethargic with myoclonic jerks, hypertonia, and hyperreflexia. Laboratory analyses showed thyroid-stimulating hormone of 0.057 μU/mL (normal, 0.27 to 4.0 μU/mL), free thyroxine of 1.5 ng/dL (normal, 0.7 to 1.5 ng/dL), triiodothyronine of 0.7 ng/mL (normal, 0.6 to 1.6 ng/dL), and elevated anti–thyroid peroxidase (>600 U/L) and antithyroglobulin (57.2 U/L) antibodies. The patient failed to impro...

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT): Characteristics, treatment and outcome in 251 cases from the literature.

Steroid-responsive encephalopathy and associated autoimmune thyroiditis (SREAT) is characterized by encephalopathy and the presence of antithyroid antibodies. We describe the clinical presentation, outcome and treatments for SREAT by a systematic review of the literature. MEDLINE via PubMed, Web of Science and the Cochrane Library were searched for articles published until 2015. Inclusion criteria were unexplained encephalopathy with antithyroid antibodies. We found reports of 251 patients (median age 52years [range 18-86], 73% females, 80 [32%] with preexisting thyroiditis). Patients presented encephalitis signs with convulsions (n=117; 47%), confusion (n=115, 46%), speech disorder (n=91, 37%), memory impairment (n=107, 43%), gait disturbance (n=67, 27%) and persecutory delusions (n=61, 25%). Twenty-eight patients (11%) presented progressive memory impairment and 26 (10%) isolated psychiatric disorders. In serum, 34% of patients were positive for anti-thyroid peroxidase (TPO) antibodies, 7% for anti-thyroglobulin (TG) antibodies, and 69% both. Thyroid-stimulating hormone levels were usually normal, at 2 UI/ml [0.001-205]. Cerebrospinal fluid from 10/53 patients (19%) was positive for anti-TPO antibodies, 2/53 (4%) anti-TG antibodies and 28 (53%) both. Electroencephalography findings were abnormal for 82% of patients, showing diffuse slowing consistent with encephalopathy (70%) or epileptic activity (14%). The first-line treatment was steroids in 193 patients and other immunosuppressive drugs in 10 cases. At a median follow-up of 12months [range 0.2-110], 91% of patients showed complete or partial neurological response, with anti-TPO and -TG antibody titers at 347 UI/ml [0-825,000] and 110 UI/ml [0-50,892], respectively. During follow-up, 40 patients (16%) experienced at least one relapse. Relapse was more frequent in patients with initial coma (26% vs 13%, p=0.08). The diagnosis of SREAT should be suspected in case of encephalopathy without obvious cause, to quickly start corticosteroid treatment. The exact modalities of treatment must be defined.

Delirious Mania Associated with Autoimmune Gastrothyroidal Syndrome of a Mid-Life Female: The Role of Hashimoto Encephalopathy and a 3-Year Follow-Up including Serum Autoantibody Levels.

We report the case study of a 57-year-old Caucasian female with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), commonly termed Hashimoto encephalopathy (HE). This presentation includes one of the longest lasting follow-up studies of HE considering the neuropsychiatric symptoms (here delirium, mania, and EEG-slowing) and their relation to serum autoantibody levels. Antithyroid-peroxidase autoantibodies, the hallmark of autoimmune thyroiditis, were found in the serum and also in the cerebrospinal fluid. Diagnostic analyses found no evidence of limbic encephalopathies characterized by serum antibodies against intracellular, synaptic, or further cell surface antigenic targets, neoplasm, and connective tissue or vasculitis diseases. A potential contribution of bipolar disorder and metabolic encephalopathies due to severe hypothyroidism, glucocorticoid treatment, accelerated thyroid hormone replacement therapy, or vitamin B deficiency is critically discussed. Another special feature of this case report is the linkage of HE to an autoimmune polyendocrine syndrome (type 3B) affecting the gastroduodenum in addition to the thyroid gland.

Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) with stroke like symptoms. A case report. (P5.104)

Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) with stroke like symptoms. A case report. (P5.104)