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Hashimoto's encephalopathy is an autoimmune disease characterized by diverse clinical and imaging manifestations, often presenting considerable challenges in its diagnosis, especially when myelitis occurs before the onset of encephalopathy. Anti-N-terminal of α-enolase antibodies targeting the N-terminal of α-enolase are specific serum markers of Hashimoto's encephalopathy; however, studies analyzing their association with myelitis are lacking. Herein, we describe the case of a patient with myelitis who later developed encephalopathy. Owing to positivity for anti-N-terminal of α-enolase antibodies in the serum, a diagnosis of Hashimoto's encephalopathy was made. Detecting anti-NAE antibodies can be useful in diagnosing myelitis of unknown etiology.

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.

This article provides an overview of Hashimoto's encephalopathy (HE) from an endocrinological perspective. HE is a rare condition characterized by encephalopathy and central nervous system dysfunction, with positive tests for thyroid auto-antibodies. The prevalence of HE is estimated to be around 2.1 per 100,000, with a higher incidence in females and an average age between 45 and 55 years. The clinical presentation of HE can vary widely, with symptoms including stroke-like episodes, cognitive dysfunction, tremor, psychosis, and seizures. The diagnosis of HE is mainly a diagnosis of exclusion, and the pathogenesis of the condition is still largely unknown. Positive tests for thyroid autoantibodies are a mandatory criteria for diagnosing HE, but their specificity is low. The article also discusses the possible role of thyroid autoantibodies in the cerebrospinal fluid as a diagnostic and pathogenetic marker. Overall, HE remains a controversial condition with much still to be understood. The text discusses Hashimoto's Encephalopathy (HE), a neurological disorder that is potentially autoimmune in nature. The diagnostic criteria for HE are outlined, including the presence of thyroid antibodies, normal brain MRI, and exclusion of other causes. The text also highlights the challenges in diagnosing HE, as many patients receive alternative diagnoses. Treatment options for HE include corticosteroids and other immunosuppressive drugs. The text concludes by acknowledging that despite advancements in diagnostic procedures, there [Extracted from the article]

A rare type of encephalopathy that typically manifests with neuropsychiatric symptoms is Hashimoto's encephalopathy, also known as steroid-responsive encephalopathy associated with autoimmune disease (SREAT). The majority of individuals with SREAT are typically euthyroid when they first appear, while the condition can manifest at any level of T3, T4, or thyroid-stimulating hormone. A significant diagnostic factor for this uncommon disorder is elevated thyroid antibodies, which are supported by magnetic resonance imaging (MRI), cerebrospinal fluid analysis (CSF), and electroencephalogram (EEG) results indicative of diffuse encephalopathy. An effective dose of steroids works for the majority of patients. In this case, a 43-year-old woman with rheumatoid arthritis and hypothyroidism who is on disease-modifying medication presented with symptoms that were similar to those of catatonia. After careful consideration, the patient was put on intravenous pulse steroids with the suspicion of SREAT after ruling out other metabolic reasons. Steroid therapy produced a substantial improvement in the patient, who was discharged with a premorbid condition. This case is distinctive due to its appearance, occurrence, therapeutic response, and the existence of another autoimmune illness like rheumatoid arthritis. Hashimoto's encephalopathy (SREAT) is a rare manifestation of a common disease. According to the Diagnostic and Statistical Manual, our patient was diagnosed with catatonia (DSM-5). She displayed posturing, catalepsy, mutism, stupor, and agitation. It is crucial to comprehend this rare disease, which affects 1 in every 2 lakh people, because it could be confused for a primary psychiatric disorder. [ABSTRACT FROM AUTHOR]

Background/Objective: Our objective is to highlight the importance of identifying symptoms of steroid-responsive encephalopathy with associated thyroiditis (SREAT), especially in the setting of intermittent cognitive dysfunction, and to inform that SREAT can develop even in patients with a history of partial thyroidectomies. Case Report: We present a case of a 51-year-old woman with a long-standing history of hypothyroidism presenting with acute onset myoclonus, involuntary tremors, fatigue, malaise, and palpitations for two weeks, with intermittent lapses in cognitive function. The patient’s workup is completely within normal limits, including her cognition, except for elevated thyroid stimulating hormone levels and markedly elevated levels of antithyroid peroxidase antibodies, despite the fact that she previously had a partial thyroidectomy. Discussion: SREAT is an autoimmune condition characterized by cognitive dysfunction, elevated thyroid autoantibodies, and therapeutic response to corticosteroids. SREAT is primarily considered a diagnosis of exclusion. A crucial feature is the hallmark of significant improvement in symptoms when glucocorticoids are administered. There is a significant correlation between patients with elevated antithyroid peroxidase antibodies and new-onset SREAT. Although total thyroidectomy has been reported as a definitive treatment of SREAT, response to corticosteroids is the “sine qua non” in diagnosing this condition. Conclusion: Hashimoto's thyroiditis can lead to a rare complication called SREAT, presenting with various neurologic symptoms. Prompt glucocorticoid treatment is vital, and a positive response confirms the diagnosis. Total thyroidectomy may be necessary for definitive SREAT treatment. More research is needed for alternate treatments and an understanding of the pathophysiology of SREAT.

Key Clinical Message: The report underscores the necessity for a comprehensive evaluation in patients with suggestive laboratory findings or AITD history. Prompt diagnosis and appropriate management are imperative in averting long‐term complications. [ABSTRACT FROM AUTHOR]

Using proteomic analysis, we identified anti‐NAE antibodies (autoantibodies against NH2‐terminal of alpha‐enolase) as a diagnostic marker for Hashimoto's encephalopathy (HE). As cases of HE with serum anti‐NAE antibodies accumulated, the clinical spectrum of HE with anti‐NAE antibodies became expanded. In addition, it is now known that antibodies can be detected in other diseases that differ from conventional HE. We recently reported the detection of anti‐NAE antibodies in patients with a clinical diagnosis of multiple system atrophy or corticobasal syndrome. These findings suggest that anti‐NAE antibodies indicate an immune mechanism in the pathogenesis of neurodegenerative diseases. We propose a novel disease concept for anti‐NAE antibody‐associated disorders (NAEAD). The clinical spectrum of NAEAD is not limited to HE but is a broad spectrum that partially shares several autoimmune neurological diseases, including autoimmune acute encephalopathy, autoimmune cerebellar ataxia, and autoimmune psychosis, and even extends to immune‐associated neurodegenerative diseases. [ABSTRACT FROM AUTHOR]

Hashimoto’s encephalopathy (HE), a rare autoimmune disorder associated with thyroiditis, is a condition with no definitive cause. Symptoms include altered mental status, seizures, and cognitive impairment. When diagnostic criteria are met, steroids are the cornerstone of treatment with a reported good response. This report presents a 26-year-old female with HE who presented with persistent status epilepticus and altered mental status, complicated by aspiration pneumonia and laryngeal edema, and had been managed successfully. After exclusion of the common causes of acute altered mental status and seizures, early workup for treatable rare causes such as HE can help rapid recovery and avoid complications. However, proper management of complications such as lower respiratory tract infection, if happened, is necessary for good outcome.

Mental disorders often accompany autoimmune diseases, for example, since 1949 it has been known about “myxedematous madness”, a psychosis caused by hypothyroidism. The most common cause of hypothyroidism is Hashimoto's autoimmune thyroiditis. It is also known about another neuropsychiatric disorder associated with autoimmune thyroiditis, Hashimoto's encephalopathy. It is a severe dysfunction of the central nervous system, the pathogenesis of which is not associated with hormonal disorders. Cytokines are regulators and participants of inflammation, including autoimmune. Certainly, when we are talking about high concentrations cytokines, we mean systemic inflammation. The minimal or mediocre fluctuations in cytokines within the ranges that are characteristic of healthy status or normergic acute phase response in disease cannot be interpreted from the point of view of binary endocrinological logic. In the CNS, cytokines are able to influence on the neuroendocrine control of systemically regulated functions. It is also important that glial cells (astroglia, microglia) are capable of producing a number of cytokines and can affect neurons and develop behavioral changes. In addition, the ability of a number of cytokines outside the CNS itself to act on vagal afferents and through them to convey information to the CNS, affecting its state and functions, has been proven. It is reasonable to assume that minimal fluctuations in cytokine levels may also affect the state and function of the CNS. The aim of the study was to investigate the levels of cytokines in patients with thyroiditis; in patients with thyroiditis associated with mental disorders; in a group of healthy individuals; and evaluate the effect of cytokine levels on clinical manifestations. In the group of patients with thyroiditis and mental disorders, the levels of CCL20/MIP3α, IL-13, IL-2, IL-27, IL-5 were significantly higher than in other groups. At the same time, no positive correlation was found between the clinical manifestations of mental disorders and the levels of cytokines. A positive correlation was found between the levels of some cytokines and free triiodothyronine, as well as the level of antithyroid antibodies. Mental disorders associated with autoimmune thyroiditis may be associated with changes in the cytokine profile and result from neuroinflammation.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) or Hashimoto's encephalopathy is a relatively rare condition; it is a poorly understood entity and is often misdiagnosed and mistreated. It is seen in a very small percentage of patients, who have an autoimmune thyroid disease and a high level of anti-thyroid antibodies. This condition causes various neurological and psychiatric disorders. Although the exact association between SREAT and thyroid disorder is not yet established, it is important to diagnose SREAT. This condition is treatable and often reversible. Here, we report a case of a 24-year-old female, who was being treated for Vitamin B12 deficiency, anaemia, and hypothyroidism. She suddenly developed encephalopathy. After ruling out other causes, we detected elevated levels of thyroid peroxidase (TPO). Therefore, a diagnosis of Hashimoto's encephalopathy was made, and the patient responded dramatically to steroids. [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]

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Background: Encephalopathy associated with autoimmune thyroid disease (EAATD) is an uncommon disorder affecting the central nervous system and associated with autoimmune thyroid disorders. It has myriad manifestations. Although most commonly seen with Hashimoto's thyroiditis, it has also been reported with Graves' disease. As it has no pathognomonic features (apart from response to steroids), it is a diagnosis of exclusion, and can be very challenging for the clinician. Clinical Description: An 11-year-old girl presented with a fever and altered sensorium for a few hours. In-depth history was unable to identify any significant history apart from recent weight loss. Salient examination findings were severe thinness, tachycardia, an enlarged thyroid gland, and altered sensorium, but the absence of neurological deficit, meningeal involvement, and increased intracranial tension. The clinical phenotype was acute encephalitis syndrome. Investigations ruled out biomarkers of infection, metabolic acidosis, hypoglycemia, dyselectrolytemia, uremia, hepatic dysfunction, and diabetic ketoacidosis. Neuroimaging was normal. Cerebrospinal fluid abnormalities were lymphocytosis, high protein, and normal glucose. A thyroid function test identified hyperthyroidism. Doppler ultrasonography revealed a diffusely enlarged and hypervascular thyroid gland. Thyrotropin-receptor antibodies were elevated (>10 IU/L). The final diagnosis was EAATD secondary to Graves' disease. Management and Outcome: Intravenous methylprednisolone (30 mg/kg/day), antithyroid drugs, and beta-blockers were started, on which she showed rapid and marked improvement. Conclusions: A detailed history, diligent clinical examination, and rational systemic approach needs to be undertaken to establish a diagnosis of EAATD.

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.

Abstract Background Hashimoto’s encephalopathy (HE) is a rare but controversial syndrome showing a variety of neurological and psychiatric manifestations associated with autoimmune thyroiditis. Case presentation We present a case of 46-year-old hypertensive female who developed acute onset of behavioral symptoms mimicking affective disorder (mania). Investigations revealed high levels of anti-thyroid peroxidase antibodies with elevated thyroid-stimulating hormone. EEG and MRI findings were consistent with those seen in Hashimoto’s encephalopathy. She showed excellent response to high dose corticosteroids that helped in making the diagnosis of Hashimoto’s encephalopathy. Conclusions Hashimoto’s encephalopathy is an extremely important, though rare, diagnosis. It should be suspected and screened for in patients with encephalopathy of unknown origin because responses to treatment are usually excellent.

Hashimoto’s encephalopathy is a rare life-threatening disease entity with clinical presentation like psychiatric as well as neurological diseases, thus making it a challenging clinical scenario for physicians leading to delay in diagnosis and management of the patient. The incidence overall is under-estimated because of low overall awareness about the disease. Here, we report a 44-year-old female who was being treated for psychiatric disorders because of clinical presentation like catatonia but ultimately was diagnosed with Hashimoto’s encephalopathy and responded very well to steroids.

Background: Hashimoto's encephalopathy (HE) is a rare but controversial syndrome showing a variety of neurological and psychiatric manifestations associated with autoimmune thyroiditis. Case presentation: We present a case of 46-year-old hypertensive female who developed acute onset of behavioral symptoms mimicking affective disorder (mania). Investigations revealed high levels of anti-thyroid peroxidase antibodies with elevated thyroid-stimulating hormone. EEG and MRI findings were consistent with those seen in Hashimoto's encephalopathy. She showed excellent response to high dose corticosteroids that helped in making the diagnosis of Hashimoto's encephalopathy. Conclusions: Hashimoto's encephalopathy is an extremely important, though rare, diagnosis. It should be suspected and screened for in patients with encephalopathy of unknown origin because responses to treatment are usually excellent. [ABSTRACT FROM AUTHOR]

Hashimoto’s encephalopathy is a rare disease, first reported in 1966, with a prevalence of 2.1 in 1000 inhabitants. We present the case of a 42- year- old woman, with no relevant medical history, who suddenly started having symptoms of altered consciousness, visual hallucinations and de-lusions. Laboratory tests showed anti- thyroperoxidase antibodies greater than 600 U/ml, thy-roxin 0.93 U/ml, and thyroid stimulating hormone 1.60 U/ml. Magnetic resonance imaging showed bilateral subcortical focal lesions with a nonspecific demyelinating appearance. The electroencephalogram was nonspecific. The diagnosis of Hashimoto encephalopathy was made, and symptoms remitted after treatment with steroids. This article highlights the importance of conducting a comprehensive evaluation of patients with atypical psychiatric symptoms and a thorough differential diagnosis.

Hashimoto's encephalopathy (HE), also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), has a variety of clinical manifestations, with various neuropsychiatric characteristics, including tremors, transient aphasia, seizures, altered consciousness, myoclonus, cognitive impairment, and psychiatric manifestations. The hallmark presenting feature is a non-specific encephalopathy characterized by alteration of mental status and consciousness ranging from confusion to coma and impaired cognitive function, while those with cerebellar ataxia as the main manifestation is rare. We reported a case of Hashimoto's encephalopathy with cerebellar ataxia as the main manifestation, elevated anti-thyroid antibodies (anti-TPO/TG), and normal thyroid function. The symptoms of cerebellar ataxia improved after steroid treatment. Meanwhile, we reviewed the clinical features of 20 representative cases of HE with cerebellar ataxia as the core symptoms. In conclusion, based on our case findings and literature review, the diagnosis of HE should be suspected in cases of encephalopathy without an obvious cause, to quickly start an e􀀀ective treatment. [ABSTRACT FROM AUTHOR]

In recent years the neurobiological underpinnings of catatonia have been an emerging area of interest. Catatonia is frequently encountered in mood disorders, neurological disorders and systemic illnesses. Furthermore, the manifestation of catatonia in autoimmune disorders such as NMDA receptor antibody encephalitis and thyroiditis reinforces its neuropsychiatric nature. Irrespective of cause benzodiazepines and electroconvulsive therapy remain the standard treatments for catatonia, although a proportion fail to respond to the same. This report describes three women with pre-existing bipolar disorder presenting in catatonia. Interestingly in all three, while benzodiazepines and electroconvulsive therapy failed, a dramatic resolution of catatonia with corticosteroids was noted following the detection of Hashimoto's thyroiditis. Hashimoto's encephalopathy presenting as catatonia has been reported, but our patients' profile differed in having had an a priory diagnosis of bipolar disorder. Given that both catatonia and thyroid dysfunction are frequently encountered in bipolar disorder, Hashimoto's encephalopathy as a potential cause for this concurrent manifestation in bipolar disorder may be overlooked. Therefore, it is essential to suspect Hashimoto's encephalopathy when catatonia manifests in bipolar disorder. A timely evaluation would be prudent as they may fail to respond to standard treatments for catatonia but respond remarkably to corticosteroids, saving much time and angst. Recent evidence implicates immune system dysfunction, with neuroinflammation and peripheral immune dysregulation contributing to the pathophysiology of bipolar disorder as well as catatonia. Findings from this study reaffirm the role of immune system dysfunction common to the etiopathogenesis of all these disorders, highlighting the complex interplay between catatonia, thyroiditis and bipolar disorder.

Steroid-responsive encephalopathy, also known as Hashimoto's encephalopathy, is an uncommon autoimmune neuroendocrine disorder linked to thyroiditis. It presents a variable range of clinical symptoms, making it challenging to diagnose. SREAT (steroid-responsive encephalopathy associated with autoimmune thyroiditis) is considered a diagnosis of exclusion. Diagnosing SREAT poses a challenge as its symptoms can overlap with those of acute conditions such as encephalitis and meningitis, as well as various autoimmune disorders and issues related to substance toxicity and abuse, all of which must be ruled out prior to establishing a definitive diagnosis and starting treatment. However, high anti-TPO antibodies (anti-thyroid peroxidase) in serum and rarely in the CSF favor the diagnosis of the condition. Although rare, Hashimoto's encephalopathy should be considered when managing cases of unexplained encephalopathy. Treatment of choice in these sets of patients includes high-dose corticosteroid therapy. We present a case of Hashimoto's encephalopathy in a middle-aged woman who arrived at our emergency department with altered mental status and facio-brachial seizures. After excluding various potential causes of acute encephalopathy, a diagnosis of SREAT was made. Testing of serum and cerebrospinal fluid (CSF) indicated the presence of anti-thyroid peroxidase (anti-TPO) antibodies, which is uncommon in CSF. Following intravenous steroid therapy, the patient experienced substantial improvement, reinforcing the diagnosis., 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, Surapaneni et al.)

Hashimoto's encephalopathy is a rare life-threatening disease entity with clinical presentation like psychiatric as well as neurological diseases, thus making it a challenging clinical scenario for physicians leading to delay in diagnosis and management of the patient. The incidence overall is under-estimated because of low overall awareness about the disease. Here, we report a 44-year-old female who was being treated for psychiatric disorders because of clinical presentation like catatonia but ultimately was diagnosed with Hashimoto's encephalopathy and responded very well to steroids. [ABSTRACT FROM AUTHOR]

Hashimoto's encephalopathy is a rare immune-mediated disorder characterized by subacute encephalopathy with elevated thyroid antibodies. Hashimoto's encephalopathy is also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis. We report a rare presentation of Hashimoto's encephalopathy presenting with acute neuropsychiatric disturbances, rapidly progressive dementia, seizures, and extrapyramidal failure. Neuroimaging revealed multifocal vasculitides of major cerebral vessels that support the autoimmune vasculitic theory as the underlying pathogenesis for Hashimoto's encephalopathy. Unfortunately, permanent irreversible cerebral damage has already ensued before her presentation to our center, which rendered steroid therapy ineffective. Serological testing for Hashimoto's thyroiditis must be in the investigation of all rapidly progressive dementias as early diagnosis and timely management of autoimmune thyroiditis may salvage sizable and eloquent cerebral tissues. The rarity of the condition should not preclude the investigation of Hashimoto's disease even in the presence of normal levels of thyroid hormones. Delayed diagnosis may result in irreversibly catastrophic encephalopathy in patients who once presented with potentially curable dementia. [ABSTRACT FROM AUTHOR]

In the following, we report a therapeutic challenge faced by reduction in corticosteroid therapeutic dosage in a patient diagnosed with Hashimoto's encephalopathy (HE), which is equivalent to corticosteroid‐responsive encephalopathy related to autoimmune thyroiditis and often misdiagnosed as neuropsychiatric status. The patient developed psychiatric symptoms. [ABSTRACT FROM AUTHOR]

Hashimoto's encephalopathy (HE) is a steroid-responsive encephalopathy characterized by several neurological symptoms. HE mainly involves the central nervous system; the peripheral nervous system is rarely involved. We treated a previously healthy elderly man showing mild cognitive decline and subacute progressive gait disturbance due to severe sensory deficits, including sensation of touch and deep sensation with elevated anti-NH2 terminal of α-enolase and anti-thyroid antibodies. His sensory disturbance symptoms improved after steroid therapy, suggesting that the neuropathy was related to HE. His disease was characteristic of HE in that his sensory deficits responded well and rapidly to steroid therapy. A nerve conduction study showed reduced sensory nerve action potentials in all limbs, indicating that his neuropathy was not “axonopathy”, but “sensory ganglionopathy”, which can occur concurrently with autoimmune disorders. Dysautonomia may be the responsible pathomechanism because of the vulnerability of the blood–nerve barrier at the ganglia. Although the pathophysiology of HE has not been clearly elucidated, autoimmune inflammation has been reported in a number of autopsy cases, indicating that sensory ganglionopathy can develop with HE. Therefore, HE should be recognized as one type of “treatable neuropathy”.

A few patients with Hashimoto’s thyroiditis or Graves’ disease develop a multiform syndrome of the central nervous system (CNS) termed Hashimoto’s encephalopathy or steroid-responsive encephalopathy associated with autoimmune thyroid disease (HE/SREAT). They have high levels of thyroid autoantibodies (TgAb, TPOAb and/or TSH-R-Ab) in blood and cerebrospinal fluid. Autoantibodies against alpha-enolase, aldehyde reductase-I (AKRIA) and/or dimethylargininase-I (DDAHI), proteins expressed in the CNS among other tissues, were detected in the blood and, when searched, in the cerebrospinal fluid of HE/SREAT patients. Recently, we reported that alpha-enolase, AKRIA and DDAHI share local sequence homology with each of the three autoantigens (TgAb, TPOAb, TSH-R-Ab), often in epitope-containing segments of the thyroid autoantigens. We hypothesized that there might be additional CNS-expressed proteins homologous to thyroid autoantigens, possibly overlapping known epitopes of the thyroid autoantigens. We used bioinformatic methods to address this hypothesis.Six, 27 and 47 of 46,809 CNS-expressed proteins share homology with TSH-R, Tg and TPO, respectively. The homologous regions often contain epitopes, and some match regions of thyroid autoantigens which have homology with alpha-enolase, AKRIA and/or DDAHI. Several of the aforementioned proteins are present in CNS areas that show abnormalities at neuroimaging in HE/SREAT patients. Furthermore, autoantibodies against some of the said six, 27 and 47 proteins were reported to be associated with a number of autoimmune diseases.Not only we validated our hypothesis, but we think that such a variety of potential CNS targets for thyroid Ab against epitopes contained in regions that have local homology with CNS proteins may explain the polymorphic phenotypes of HE/SREAT. Only when elevated amounts of these Ab are synthesized and trespass the blood-brain barrier, HE/SREAT appears. This might explain why HE/SREAT is so relatively rare.

Hashimoto’s encephalopathy (HE) commonly presents with two types of neurological presentation: recurrent stroke-like episodes or seizures and progressive cognitive decline. Being a treatable disorder, one cannot afford to miss the diagnosis. We report a female with HE who presented with a history of recurrent episodes of catatonia, recovering completely with steroids. The diagnosis of the primary disease got delayed for 5 months. HE presenting as catatonia has been reported rarely. A high degree of suspicion in appropriate clinical settings, resulting in early diagnosis, is rewarding as HE usually show good response to corticosteroids.

Hashimoto’s encephalopathy (HE) is a syndrome occurring in some patients with Hashimoto’s thyroiditis or, less frequently, Graves’ disease. Three known autoantigens are involved in HE: alpha-enolase, dimethylargininase-I (DDAHI) and aldehyde reductase-I (AKRIAI). We searched for amino acid sequence homologies between these proteins and the three classical thyroid autoantigens (thyroperoxidase (TPO), thyroglobulin (Tg), TSH-receptor (TSH-R)), which are also expressed in the central nervous system (CNS). TSH-R shows homologies with alpha-enolase (n=4), DDAHI (n=2) and AKRIAI (n=5); of these segments, two, two and four, respectively, overlap totally or partially with epitope-containing TSH-R segments. Tg has 10 homologies with alpha-enolase, five with DDAHI, and eight with AKRIAI; epitope-containing segments of Tg overlap four, three and four segments, respectively. TPO has six segments homologous to alpha-enolase, three to DDAHI and seven to AKRIAI; of these segments, five, one and four, respectively, are located in epitope-containing parts. These data suggest that cross-reactivity between CNS autoantigens and thyroid autoantigens might contribute to the HE pathogenesis, together with other proposed mechanisms, including autoimmunity involving autoantigens common to CNS and thyroid.

Objective: To determine the frequency of different neurological features in Hashimoto's encephalopathy and to evaluate association with underling disease. Study Design: Cross sectional study. Place and Duration of Study: A Three-year study from Jul 2015 to Jul 2018. Conducted in Pak Emirates Military Hospital (PEMH) Rawalpindi. Methodology: This study is comprised of diagnosed patients of Hashimoto's encephalopathy. These patients fulfilled the diagnostic criteria. Any case with metabolic, toxic, vascular or malignant etiology of encephalopathy was excluded. Data regarding clinical symptoms particularly neurological and neuropsychiatric features, of diagnosed cases with Hashimoto’s encephalopathy was obtained from hospital record. Moreover, data about antithyroid antibody type, cerebrospinal fluid analysis, and MRI findings was gathered. Gender and age of the patients were also mentioned. Data was gathered on proforma by a resident doctor. The analysis was carried out using SPSS 20. Results were mentioned in the form of the mean for variables like age and percentage and frequencies for categorical variables. Results: Out of 13 patients, 5 (38.5%) were male and 8 (61.5%) were females. Majority of the patients 8 (61.5%) were euthyroid. Neuropsychiatric symptoms were present in all 13 patients with depression present in 8 (61.5%) cases, while 5 (38.4%) had hallucinations. Most cases were cognitively normal 8 (61.5%), while rest had mild impairment. No neurological manifestation was found significantly more common in any of the thyroid disease. Migraine-like headaches was present in 4 (30.7%) cases while epileptic seizures were present in 8 (61.5%) patients with GTCS the most common seizures type in 3 (23%) patients. Only three patient had MRI changes including temporal and hippocampal T2WI hyperintensities. Conclusion: If etiological factors for encephalopathy has been ruled out in the presence of underlying thyroid disease with a short history of symptoms, one should have a low threshold for treatment with the steroid to observe for the response.

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.

Abstract Hashimoto's encephalopathy is a rare disease with nonspecific symptoms, associated with elevated levels of anti‐TPO and/or anti‐TG. It can be potentially fatal. However, it is responsive to steroid and treated in due time, it can be fully reversible.

Hashimoto's encephalopathy (HE) is a steroid-responsive encephalopathy associated with autoimmune thyroiditis; it is based on the inflammatory brain process associated with a cross-reaction to anti-thyroid autoantibodies. HE is manifested by impaired consciousness and focal neurological symptoms. The determination of the serum and cerebrospinal fluid levels of antithyroid autoantibodies plays a main role in diagnosing HE. The latter is treated with glucocorticoids and other immunosuppressive agents. The prognosis is favorable providing that the treatment of the disease is initiated in time.

Hashimoto's encephalopathy (HE) is an autoimmune encephalopathy that presents with various clinical symptoms, including cognitive deterioration, convulsive seizures, and personality changes. HE is associated with thyroid autoimmunity; however, few cases have been reported to develop as paraneoplastic syndrome. Herein, we report the case of a 73-year-old woman with onset of rapidly progressive dementia. Brain magnetic resonance imaging showed diffuse T2 hyperintensity areas involving the bilateral cerebral white matter, right midbrain tegmental area, left cerebral peduncle, and right middle cerebellar peduncle without clear diffusion hyperintensities and gadolinium enhancement. Her neurological symptoms worsened rapidly, and she presented with the apallic syndrome. Electroencephalogram showed periodic synchronous discharge, suggestive of Creutzfeldt–Jakob disease. However, a brain biopsy revealed infiltration of atypical lymphoid cells expressing CD20, and the anti-NH2 terminal of the α-enolase antibody was detected, diagnosing the complication with lymphomatosis cerebri and HE. High-dose intravenous methylprednisolone therapy and oral prednisolone with whole cranial irradiation enabled her to have simple conversations and consume food orally; however, severe cognitive impairment persisted. Although HE is a rare complication of malignant lymphoma, clinicians should be aware that it could be strongly suspected if the clinical symptoms worsen in the absence of imaging changes.

Hashimoto's encephalopathy (HE) is an autoimmune encephalopathy that presents with various clinical symptoms, including cognitive deterioration, convulsive seizures, and personality changes. HE is associated with thyroid autoimmunity; however, few cases have been reported to develop as paraneoplastic syndrome. Herein, we report the case of a 73-year-old woman with onset of rapidly progressive dementia. Brain magnetic resonance imaging showed diffuse T2 hyperintensity areas involving the bilateral cerebral white matter, right midbrain tegmental area, left cerebral peduncle, and right middle cerebellar peduncle without clear diffusion hyperintensities and gadolinium enhancement. Her neurological symptoms worsened rapidly, and she presented with the apallic syndrome. Electroencephalogram showed periodic synchronous discharge, suggestive of Creutzfeldt–Jakob disease. However, a brain biopsy revealed infiltration of atypical lymphoid cells expressing CD20, and the anti-NH2 terminal of the α-enolase antibody was detected, diagnosing the complication with lymphomatosis cerebri and HE. High-dose intravenous methylprednisolone therapy and oral prednisolone with whole cranial irradiation enabled her to have simple conversations and consume food orally; however, severe cognitive impairment persisted. Although HE is a rare complication of malignant lymphoma, clinicians should be aware that it could be strongly suspected if the clinical symptoms worsen in the absence of imaging changes. [ABSTRACT FROM AUTHOR]

The artery of Percheron (AOP) is an anatomical variant of the thalamoperforating arteries. AOP occlusion can cause bilateral paramedian thalamic infarctions and is referred to as a "stroke chameleon" because it lacks the classic signs of stroke. Coexistence of AOP occlusion and other neurologic disease is rare and can cause disturbance of consciousness. A 78-year-old woman had acute onset of left limb weakness and drowsy consciousness. Brain magnetic resonance angiography (MRA) revealed acute bilateral paramedian thalamic infarctions. However, serum and cerebrospinal fluid (CSF) cryptococcal antigen titers were 1:16 and 1:128, respectively. The CSF culture grew Cryptococcus neoformans. Although consciousness and muscle power improved after treatment, the patient later died of pneumonia. A 68-year-old woman developed acute disturbance of consciousness followed by delirium. Brain MRA revealed acute bilateral paramedian thalamic infarctions. Elevated free thyroxine, anti-thyroperoxidase, and anti-thyroglobulin antibodies were detected. She received 3 days of steroid pulse therapy followed by oral prednisolone. Her consciousness gradually improved after Hashimoto encephalopathy and stroke were controlled. AOP occlusion was diagnosed early in these two patients. However, other concomitant life-threatening diseases could have been overlooked because of the complicated diagnostic determination. Further serum cryptococcal antigen, anti-TPO Ab, and anti-TG Ab surveys might help to exclude cryptococcal meningitis and Hashimoto encephalopathy. CSF study is warranted when central nervous system infection is strongly suspected. This "Percheron artery-plus syndrome" comprises multifaceted disorders beyond the stroke chameleon and requires attention. [ABSTRACT FROM AUTHOR]

Objective: To investigate the specificity of the antibodies related to autoimmune encephalitis and to identify possible associated factors with the false-positive result. Materials and Methods: The present study was a prospective observational study, conducted at the Ramathibodi Hospital between June and December 2019. All patients, who had acute to subacute encephalopathy from any causes, were recruited to the study. Their serum or cerebrospinal fluid (CSF) were taken to analyze for autoimmune encephalitis assays and anti-thyroid antibodies. The authors did not interfere with the primary physicians on any management of the patients. Clinical and laboratory data were systematically reviewed and collected from medical records. The clinical outcome was evaluated one month after the onset. Results: Fifty-one patients were recruited. Only one patient had autoimmune encephalitis related to anti-CV2/CRMP5 antibody. Seventeen out of the remaining fifty patients had positive tests for anti-thyroid antibodies of which five had Hashimoto's thyroiditis and one of them did not have the document of thyroid status. Eleven remaining patients appeared to have false-positive test since their medical conditions were all clearly explained by other causes. Comparison of clinical and laboratory data between patients with false-positive test and patients with true negative test did not show any significant difference except the duration of the symptoms, which was significantly shorter in the false-positive group. Conclusion: False-positive anti-thyroid antibodies appear to be common in patients with acute encephalopathy. The occurrence of serum/CSF antibody in acute encephalopathy may be a true association, but it may not be the cause of encephalopathy. Therefore, the diagnosis of autoimmune encephalopathy based on anti-thyroid antibodies should be carefully made and excluded from all other possible causes. [ABSTRACT FROM AUTHOR]

Hashimoto's encephalopathy, also known as steroid responsive encephalopathy, is associated with thyroid antibodies (SREAT) and is a rare but serious form of encephalopathy. In this paper, we describe the signs, symptoms, outcome, and treatments as noted in the case reports reviewed. We searched PubMed, Embase, and the Cochrane library for articles in which cases of Hashimoto's encephalopathy were described. The case description had to include the diagnosis, age, sex, presenting symptoms, and diagnostic tests. We retrieved 360 articles and 65 fulfilled the inclusion criteria. These articles gave reports of 100 cases, with a mean age of 10.9 (range 2.8–19), 78 of whom were female. Epilepsy (79) – including epileptic state [24] – behavioural problems [36], hallucinations [21], headache [21], and decline in school performance [19] were most often reported. Antithyroid peroxidase (aTPO) was reported elevated in all patients. Most children [70] recovered fully, however 16 had late sequelae, mostly epilepsy. Therapies used include steroids, intravenous gammaglobulines, and cytostatics. Epilepsy, behavioural problems, decline in school performance, and hallucinations are frequent symptoms of Hashimoto's encephalopathy. Steroids are the basis of treatment, although other immunomodulatory drugs seem to be successful. About one in ten children will experience late sequelae. In any child with unexplained neurological, psychiatric, or psychological dysfunction, serum anti -thyroidperoxidase (aTPO) should be determined. • Epilepsy is the most frequent symptom of Hashimoto's encephalopathy. • There is a wide variety of symptoms in Hashimoto's encephalopathy. • Psychological and psychiatric symptoms occur often in Hashimoto's encephalopathy. • One in ten children with Hashimoto's encephalopathy experience late sequelae. • Determine aTPO in children with unexplained neurological or psychiatric dysfunction. [ABSTRACT FROM AUTHOR]

Background and purpose: 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. Methods: We retrospectively evaluated a cohort of 22 SREAT patients treated in our hospital from January 2014. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Anti-LGI1 encephalitis is an autoimmune encephalitis with antibodies against leucine-rich glioma-inactivated 1 (LGI1), first described in 2010. It is a non-frequent and poorly understood entity that represents the second most frequent cause of autoimmune encephalitis. This entity is characterized by the presence of limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Herein, we present the case of a male patient with an onset of epileptic seizures (generalized tonic-clonic seizure), and involuntary dystonic movements that affect the right side of his face and right upper limb associated with mental disorder, and affectation of higher functions. The electroencephalogram showed continuous generalized slowing of the background activity. The brain magnetic resonance imaging showed signal hyperintensity at the level of both mesial temporal lobes and hippocampi and in the head of the right caudate nucleus. Anti-thyroglobulin antibodies were positive, and he was initially diagnosed as Hashimoto's encephalopathy (HE). However, the response to corticosteroids was not completed as it is usually observed in HE. For that, antibodies for autoimmune encephalitis were tested, and the anti-LGI1 antibodies were positive in serum and cerebrospinal fluid. HE is an important differential diagnosis to consider. Furthermore, the presence of Anti-thyroglobulin antibodies should not be taken as the definitive diagnostic criteria, since these antibodies could be associated with other autoimmune encephalopathies, which include in addition to anti-LGI1, anti-NMDA and anti-Caspr2.

Hashimoto’s encephalopathy (HE) is a rare neuropsychiatric syndrome characterized by elevated levels of anti-thyroid antibodies. Diverse manifestations make timely diagnosis of HE difficult. Herein, we report a case of HE, in which the clinical symptoms and laboratory test results mimicked viral encephalitis. A 59-year-old male patient, who presented with a fever, headache, slow and unclear speech, sentence confusion, elevated levels of anti-thyroid antibodies in the serum, an increased white blood cell count, and positivity for anti-thyroid antibodies in the CSF, was finally diagnosed with HE and responded well to a small dose of methylprednisolone. This report helps bring the attention of clinicians to the fact that HE should be considered when cases of unexplained encephalopathy are encountered.

Hashimoto's Encephalopathy (HE), also known as steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a proteiform disorder known for its wide spectrum of presentations from subtle neuropsychiatric manifestations, movement disorders, seizures, stroke-like episodes to coma. Here, we report a case of HE which initially masqueraded as bipolar affective disorder (BPAD) and ultimately progressed to generalized tonic clonic seizures and coma. Although SREAT is characterized by exquisite responsive to steroid, in our case it was unresponsive to pulse methylprednisolone therapy. Rapid recovery was noted with intravenous immunoglobulin (IVIG) therapy. This case was also peculiar for its association with non-sydnromic retinitis pigmetosa (RP). To the best of our knowledge, this was the first reported case of HE which was associated with RP.

Anti-LGI1 encephalitis is an autoimmune encephalitis with antibodies against leucine-rich glioma-inactivated 1 (LGI1), first described in 2010. It is a non-frequent and poorly understood entity that represents the second most frequent cause of autoimmune encephalitis. This entity is characterized by the presence of limbic encephalitis, hyponatremia, and faciobrachial dystonic seizures. Herein, we present the case of a male patient with an onset of epileptic seizures (generalized tonic-clonic seizure), and involuntary dystonic movements that affect the right side of his face and right upper limb associated with mental disorder, and affectation of higher functions. The electroencephalogram showed continuous generalized slowing of the background activity. The brain magnetic resonance imaging showed signal hyperintensity at the level of both mesial temporal lobes and hippocampi and in the head of the right caudate nucleus. Anti-thyroglobulin antibodies were positive, and he was initially diagnosed as Hashimoto's encephalopathy (HE). However, the response to corticosteroids was not completed as it is usually observed in HE. For that, antibodies for autoimmune encephalitis were tested, and the anti-LGI1 antibodies were positive in serum and cerebrospinal fluid. HE is an important differential diagnosis to consider. Furthermore, the presence of Anti-thyroglobulin antibodies should not be taken as the definitive diagnostic criteria, since these antibodies could be associated with other autoimmune encephalopathies, which include in addition to anti-LGI1, anti-NMDA and anti-Caspr2. [ABSTRACT FROM AUTHOR]

Background: Hashimoto's encephalopathy, also known as steroid‐responsive encephalopathy associated with autoimmune thyroiditis, is a rare association of neuropsychiatric symptoms and autoimmune thyroid disease. Among the movement disorders reported in Hashimoto's encephalopathy, palatal tremor is relatively uncommon. Case presentation: We present the case of a 50‐year‐old woman with neuropsychiatric symptoms characterized by behavioral changes, cognitive impairment, epilepsy, parkinsonism, palatal tremor and ataxia with high levels of serum anti‐thyroid peroxidase and a clear response after steroid therapy. Conclusions: Given the reversibility of clinical manifestations, Hashimoto's encephalopathy should be in the differential diagnosis of patients with unexplained seizures or encephalopathy who present with these symptoms or other involuntary movements. [ABSTRACT FROM AUTHOR]