Hashimoto Encephalopathy and Thyroid Storm by Diabetic Ketoacidosis and Acute Pancreatitis: A Case Report.

Hashimoto encephalopathy (HE) is a rare condition related to autoimmune origin characterized by high titers of antithyroid antibodies. Steroids are effective for treatment of HE, suggesting the autoimmunity as an underlying mechanism. In addition, anti-NH2-terminal of α-enolase antibodies (anti-NAE antibodies) is useful for diagnosis of HE. This report describes a 69-year-old woman developing both HE and thyroid storm (TS), following diabetic ketoacidosis (DKA) and acute pancreatitis. She had a history of Basedow's disease and uncontrolled type 2 diabetes mellitus, and her serum hemoglobin A1c was 10%. She complained of nausea and visited our hospital. She was diagnosed with DKA and acute pancreatitis. After admission, she went into cardiopulmonary arrest and she was diagnosed with TS after resuscitation. In addition, blood test collected during acute phase of TS revealed positive for not only anti-thyroid peroxidase (TPO) antibodies, thyroid stimulating hormone receptor antibodies and thyroid stimulating antibodies, but also anti-NAE antibodies. She was treated with intravenous steroids, potassium iodide and thiamazole under respirator and recovered sufficiently to do daily activities of life. We should keep in mind that there might be cases of HE in cases of TS presenting with central nervous system symptoms.

Focal central neurological disorder in patients with autoimmune thyroid disease without encephalopathy.

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.

[A case of Hashimoto's encephalopathy with acute onset of psychiatric symptoms and diffuse deep white matter lesions on brain MRI].

A 51-year-old man developed acute disturbances in consciousness and psychiatric symptoms one month prior to admission. He was referred and admitted to the Department of Psychiatry of our hospital and transferred to the neurology department because diffuse white matter lesions were found on his brain during MRI. 123I-IMP-SPECT showed extensive cerebral hypoperfusion mainly in the frontal lobes. Anti-Tg, anti-TPO, and anti-NAE antibodies were positive. These findings led to a diagnosis of Hashimoto's encephalopathy. The patient responded to steroid pulse therapy, high-dose steroid therapy, and intravenous immunoglobulin therapy, showing improvement in symptoms and imaging findings. Hashimoto's encephalopathy often presents with MRI findings similar to those of limbic encephalitis, when the patient presents with acute consciousness disturbance and psychiatric symptoms. However, this case showed diffuse white matter lesions, which may be clinically important for the differential diagnosis.

Characterization of neurological morbidity associated with thyroid antibodies: Hashimoto's encephalopathy and beyond.

BACKGROUND: Hashimoto's Encephalopathy (HE) manifests with various neurologic symptoms associated with elevated thyroglobulin (TG) and/or thyroperoxidase (TPO) antibodies. Some patients with thyroid antibodies exhibit neurological presentations not consistent with HE. This study aims to characterize the spectrum of neurological morbidity in patients with thyroid antibodies. METHODS: We reviewed all patients tested for TG or TPO antibodies from 2010 to 2019. Patients tested for thyroid antibodies as part of a neurological workup for new symptoms were classified into the following categories: patients meeting full criteria for HE, patients with other neuroimmunological disorders, patients with unexplained neurological symptoms not fully meeting HE criteria, and patients with incidental non neuroimmunological disorders. RESULTS: There were 2717 patients with positive thyroid antibodies in the dataset including 227 patients (78% female, age 54 ± 19 years) who met inclusion criteria. Twelve patients (5%) met HE criteria, 30 (13%) had other neuroimmunological disorders, 32 (14%) had unexplained neurological symptoms, and 153 (67.4%) had incidental disorders. In addition to cognitive dysfunction, seizures, movement disorders, motor weakness, and psychosis, HE patients were also more likely to have cerebellar dysfunction, language impairment, and sensory deficits. They were more likely to carry a Hashimoto's thyroiditis diagnosis and had higher titers of thyroid antibodies. They all had a robust response to steroids. CONCLUSION: The neurological spectrum of HE may be wider than previously reported, including frequent cerebellar, sensory, and language dysfunction. A subgroup of thyroid antibody positive patients with unexplained neurological symptoms may represent further expansion of thyroid antibody-related neurological disorders.

Encephalopathy of Autoimmune Origin: Steroid-Responsive Encephalopathy With Associated Thyroiditis.

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.

Cerebellar Ataxia in the Setting of Hashimoto's Thyroiditis: A Case Report Update and Review.

Hashimoto's encephalopathy (HE) is a rare diagnosis. Establishing the diagnosis itself is quite challenging, as symptoms vary among cases and there is still no standard confirmatory test. The clinical presentation is heterogeneous; however, patients with HE most commonly experience focal neurological deficits, frequently accompanied by cognitive dysfunction, aphasia, or paresis. The most widely recommended initial treatment for cases of HE is a course of corticosteroids. Nonetheless, their response varies from patient to patient, and some may become resistant to them. There are many proposed second-line treatments; however, there is little data and no consensus on the best alternative treatment when steroid therapy fails. This article provides an update on a case of cerebellar ataxia in a 30-year-old female patient with Hashimoto's thyroiditis. She initially presented with rapid-onset progressive symptoms of cerebellar ataxia (movement incoordination, dysmetria, and balance problems) and had elevated serum anti-thyroid peroxidase antibodies. She was diagnosed with HE and was initially treated with methylprednisolone. However, her symptoms recurred after tapering steroid therapy, and eventually, they ceased to manage her symptoms, plus she developed steroid-induced osteoporosis. She began treatment with intravenous immunoglobulin (IVIG) as an alternative in April 2022. Since then, she has had four infusions of IVIG that have allowed her to remain symptom-free.

Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital - Thailand's largest national tertiary referral center.

BACKGROUND: Hashimoto encephalopathy has multiple clinical presentations, and other than the presence of thyroid antibody, laboratory and imaging investigations are all non-specific. Data specific to the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy in Thailand remain scarce. OBJECTIVES: To retrospectively investigate the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy at Siriraj Hospital. METHODS: Patients who presented with acute encephalopathy at our center during July 2012-March 2017 were evaluated for eligibility. The inclusion criteria were positive anti-thyroperoxidase (anti-TPO) or anti-thyroglobulin (anti-Tg) in serum with negative neuronal antibody in serum or cerebral spinal fluid (CSF). Clinical presentations, symptom duration, laboratory results of thyroid status and thyroid autoantibody, CSF study, and clinical outcomes were collected. RESULTS: Of the 204 patients who presented with encephalopathy, 31 (15.2%) were positive for the anti-TPO or anti-Tg antibody. Of those, 13 patients met the diagnostic criteria for Hashimoto encephalopathy. Clinical presentations included cognitive impairment (76.9%), clouding of consciousness (46.2%), and behavior change (30.8%). The neuropsychiatric presentations were visual hallucination (30.8%), auditory hallucination (15.4%), delusion (7.7%), and mood disturbance (23.1%). Other clinical presentations included seizure (38.5%), abnormal movement (23.1%), sleep disturbance (38.5%), ataxia (46.2%), stroke-like episode (15.4%), and fever (15.4%). Most patients (76.9%) had onset within < 3 months. Regarding outcomes, 1 patient who did not receive corticosteroid died from status epilepticus and septic shock. Among the 12 patients who received corticosteroid, 9 (75%) had marked improvement, 1 (8.3%) had slight improvement, and 2 (16.6%) had no clinical improvement. Seven patients (53.9%) had normal thyroid function, 4 patients (30.8%) had subclinical hypothyroidism, and 2 patients (15.4%) had subclinical hyperthyroidism. CONCLUSIONS: The results of this study revealed cognitive impairment, neuropsychiatric symptoms, seizure, ataxia, and sleep disturbance to be common manifestations of Hashimoto encephalopathy. This condition should always be considered in individuals with subacute onset of unexplained cognitive impairment or cerebellar ataxia. Laboratory and neuroimaging investigations were all found to be nonspecific in Hashimoto encephalopathy. Most patients responded well to treatment, so clinical suspicion and early diagnosis and treatment will lead to improved patient outcomes.

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

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.

Encefalopatía de Hashimoto. Un diagnóstico por exclusión y revisión de la literatura a partir de un reporte de caso / Hashimoto Encephalopathy as Diagnosis by Exclusion Review of the Literature from a Case Report

La encefalopatía de Hashimoto es una entidad poco frecuente, con una amplia gama de manifestaciones neurológicas que incluyen déficits focales, alteraciones cognitivas, crisis convulsivas, trastorno del movimiento e incluso el coma. Con un curso de la enfermedad de subagudo a fluctuante. Afecta más a mujeres que a hombres, con edad de presentación alrededor de los 44 años, aunque se han reportado casos en la edad pediátrica. De etiología poco clara, se desarrolla en el contexto de la presencia de anticuerpos antitiroideos, independientemente de la función tiroidea. La presencia de estos anticuerpos, sumado a la exclusión de otras etiologías y la respuesta al manejo esteroide son claves para su diagnóstico. Presentamos un caso clínico de una mujer de 57 años de edad que evoluciona con psicosis, alteración del lenguaje, deterioro cognitivo, mioclonías y crisis convulsivas de 5 meses de evolución, quien se excluyó otras causas de demencia rápidamente progresiva con presencia de anticuerpos anti tiroglobulina de 83,6 UI/mL (V.R. < 100 UI/mL) normal y anti tiroperoxidasa en 217 UI/mL (V.R. < 100 UI/mL) elevado. Recibió valoración por el Servicio de Endocrinología, donde se detectó hipotiroidismo y se indicó manejo con levotiroxina sin mejoría del cuadro neurológico. Se indicó manejo esteroide con pulsos de metilprednisona a 500 mg/día por 5 días, con mejoría clínica y se concluyó por criterios de exclusión como una encefalopatía de Hashimoto(AU)

Hashimoto encephalopathy is a rare entity, with wide range of neurological manifestations including focal deficits, cognitive alterations, seizures, movement disorders, and even coma, with a subacute to fluctuating disease course. It affects more women than men, it has age of presentation around 44 years, although cases have been reported in the pediatric age. Its etiology is unclear, it develops in the presence of antithyroid antibodies, regardless of thyroid function. The presence of these antibodies, added to the exclusion of other etiologies and the response to steroid management are key to the diagnosis. We report a clinical case of a 57-year-old woman who evolved with psychosis, language impairment, cognitive impairment, myoclonus, and seizures of 5 month-duration. Other causes of rapidly progressive dementia with the presence of normal antithyroglobulin antibodies of 83.6 IU/mL (RV < 100 IU/mL) and elevated antithyroperoxidase 217 IU/mL (RV < 100 IU/mL) were excluded. She was evaluated in the Endocrinology Department that detected hypothyroidism and indicated management with levothyroxine with no improvement in the neurological condition. Steroid management with methylprednisone pulses at 500 mg/day for 5 days was indicated. Clinical improvement was observed and was concluded to be a Hashimoto encephalopathy by exclusion criteria(AU)

Hashimoto's encephalopathy versus catatonia: A diagnostic dilemma.

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.

Thyroid Disorders and Movement Disorders-A Systematic Review.

Background: There is overlap between movement disorders and neuroendocrine abnormalities. Objectives and methods: To provide a systematic review on the association of thyroid dysfunction and movement disorders. Thyroid physiological function and classical thyroid disorders highlighting typical and atypical manifestations including movement disorders, as well as diagnostic procedures, and treatments are discussed. Results: Hypothyroidism may be associated with hypokinetic and hyperkinetic disorders. There is debate whether their concomitance reflects a causal link, is coincidence, or the result of one unmasking the other. Hypothyroidism-associated parkinsonism may resemble idiopathic Parkinson's disease. Hypothyroidism-associated hyperkinetic disorders mainly occur in the context of steroid-responsive encephalopathy with autoimmune thyroiditis, that is, Hashimoto disease, mostly manifesting with tremor, myoclonus, and ataxia present in 28-80%, 42-65% and 33-65% in larger series. Congenital hypothyroidism manifesting with movement disorders, mostly chorea and dystonia, due to Mendelian genetic disease are rare.Hyperthyroidism on the other hand mostly manifests with hyperkinetic movement disorders, typically tremor (present in three quarters of patients). Chorea (present in about 2% of hyperthyroid patients), dystonia, myoclonus, ataxia and paroxysmal movement disorders, as well as parkinsonism have also been reported, with correlation between movement intensity and thyroid hormone levels.On a group level, studies on the role of thyroid dysfunction as a risk factor for the development of PD remain non-conclusive. Conclusions: In view of the treatability of movement disorders associated with thyroid disease, accurate diagnosis is important. The pathophysiology remains poorly understood. More detailed case documentation and systematic studies, along with experimental studies are needed.

Anti-Glutamic Acid Decarboxylase (GAD) 65 Encephalitis Mistaken for Herpes Encephalitis and Hashimoto's Encephalitis (HE): A Case Report.

Autoimmune encephalitis is increasingly recognized in clinical practice. We are presenting a 72-year-old female patient who initially presented with a new onset seizure with temporal lobe abnormality on imaging. This was initially attributed to herpes encephalitis although herpes polymerase chain reaction (PCR) was negative. The patient was treated with acyclovir and antiepileptic medication (AEM) with some clinical improvement. She presented again with refractory seizure evolving to status epilepticus. Escalation of AEMs was pursued and positive anti-thyroid peroxidase (TPO) antibody prompted consideration of Hashimoto's encephalitis (HE) and treatment with high-dose corticosteroids and intravenous immunoglobulin (IVIG). However, poor response to steroid argued against HE, and extended autoimmune encephalitis panel revealed positive anti-glutamic acid decarboxylase (GAD) antibody. This case raises the clinical pearl that anti-thyroid antibodies, e.g anti-TPO antibody, can be seen in those with autoimmune encephalopathies other than HE and HE remains a diagnosis of exclusion. It also helps to remind clinicians that a new onset refractory seizure even with temporal lobe changes is not pathognomonic for herpes encephalitis, and although negative serology does not rule out the diagnosis, it is recommended to rule out autoimmune encephalitis as it shares similar clinical and radiological picture.

Steroid-responsive encephalopathy in autoimmune thyroiditis (SREAT) as a differential diagnosis of Creutzfeldt-Jakob disease.

INTRODUCTION: Steroid-responsive encephalopathy in autoimmune thyroiditis (SREAT) is characterised by a wide range of neuropsychiatric symptoms and elevated thyroid antibodies. SREAT can mimic sporadic Creutzfeldt-Jakob disease (sCJD) and distinguishing between both entities is important because SREAT responds to corticosteroids. MATERIAL AND METHODS: Data of patients reported to the National Reference Centre for the Surveillance of CJD in Göttingen, Germany between August 1994 and October 2008 was retrospectively reviewed. In the case and control groups, 49 patients had SREAT and 48 had sCJD with elevated thyroid antibodies. RESULTS: Antibodies against thyroid peroxidase were the most common antibodies in both SREAT (86%) and sCJD (88%), followed by antibodies against thyroglobulin (SREAT, 63.3%; sCJD, 39.6%; p = 0.020) and TSH-receptor-antibodies (SREAT, 14.3%; sCJD, 2.1%; p = 0.059). Epileptic seizures were observed more frequently in the SREAT group (SREAT, 44.9%; sCJD, 12.5%; p < 0.001). Dementia (SREAT, 61.2%; sCJD, 100%; p < 0.001), ataxia (SREAT, 44.9%; sCJD, 89.6%; p < 0.001), visual impairment (SREAT, 22.4%; sCJD, 50%; p = 0.005), extrapyramidal disorder (SREAT, 32.7%; sCJD, 60.4%; p = 0.006), myoclonus (SREAT, 38.8%; sCJD, 81.3%; p < 0.001) and akinetic mutism (SREAT, 6.1%; sCJD, 37.5%; p < 0.001) were observed more frequently in sCJD. Cerebrospinal fluid (CSF) pleocytosis was observed more frequently in SREAT patients (SREAT, 33.3%; sCJD, 6.4%; p = 0.001), as was a pathological increase in protein concentration (SREAT, 68.8%; sCJD, 36.2%; p = 0.001). CONCLUSIONS: In a case of encephalopathy, the diagnosis of SREAT should also be considered in suspected cases of CJD so as to be able to start corticosteroid treatment quickly.

Hashimoto's Encephalopathy: Case Series and Literature Review.

PURPOSE OF REVIEW: To describe the clinical manifestations of Hashimoto's encephalopathy (HE) and discuss its pathogenesis in light of recent research. RECENT FINDINGS: 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.

Neuropsychiatric Manifestations of Thyroid Diseases.

Thyroid disorders are known to cause neuropsychiatric manifestations. Various neuropsychiatric manifestations are depression, dementia, mania, and autoimmune Hashimoto encephalopathy. Numerous investigations carried out in the previous 50-60 years have been evaluated critically. The pathophysiology of neuropsychiatric symptoms of thyroid diseases is described in the current study and its link with autoimmune Hashimoto encephalopathy is also discussed. Furthermore, this paper also describes the association between thyroid-stimulating hormones and cognitive impairment. Hypothyroidism is associated with depression and mania, and hyperthyroidism is linked with dementia and mania. The association between Graves' disease and various mental disorders such as depressive and anxiety disorders is also discussed. The aim of this study is to review the relationship between various neuropsychiatric disorders and thyroid diseases. A literature search from the PubMed database to find various neuropsychiatric manifestations of thyroid disorders in the adult population was conducted. According to the review of the studies, cognitive impairment can result from thyroid disease. It has not been possible to demonstrate how hyperthyroidism can hasten the process of developing dementia. However, subclinical hyperthyroidism, thyroid-stimulating hormone (TSH) levels below the normal range, and high free thyroxine (T4) levels all raise the risk of dementia in the elderly. Additionally, the potential mechanisms underlying this association have been examined. A quick summary of the research on mania as a clinical symptom of hypothyroidism and its likely causes and pathogenesis is also reviewed. There is no dearth of evidence that describes various neuropsychiatric manifestation in thyroid disorders.

Disappearance of anti-thyroglobulin antibodies from the cerebrospinal fluid in parallel with neurological improvement during treatment for Hashimoto's encephalopathy / Disparition des anticorps anti-thyroglobuline dans le liquide céphalo-rachidien après traitement de l'encéphalopathie de Hashimoto

Abnormal elevation of thyroid antibodies in the CSF is observed in 62-75% of Hashimoto's encephalopathy cases. However, the relationship between CSF thyroid antibody levels and response to therapy has been poorly evaluated. We report the case of a 68-year-old man with Hashimoto's encephalopathy, in whom there was a relation between the favorable clinical outcome and the disappearance of antithyroid antibodies from the CSF and a decrease in serum thyroid antibodies.

Une élévation anormale des anticorps thyroïdiens dans le LCR est observée dans 62 à 75 % des cas d'encéphalopathie de Hashimoto. Cependant, la relation entre les niveaux d'anticorps thyroïdiens dans le LCR et la réponse au traitement a été rarement évaluée. Nous rapportons le cas d'un homme de 68 ans atteint d'encéphalopathie de Hashimoto, chez qui l'évolution clinique favorable sous traitement était associée à la disparition des anticorps antithyroïdiens du LCR et une diminution des anticorps thyroïdiens sériques.

Cerebellar Ataxia in the Setting of Hashimoto's Thyroiditis: A Case Report.

Hashimoto's encephalopathy (HE) is a rare diagnosis with a heterogenous presentation. It may not be directly related to thyroid dysfunction as most patients are euthyroid when the symptoms start. There has been a lack of consensus building on the pathophysiology of HE, but most of the evidence points towards autoimmune vasculitis as the underlying process. HE can present as seizures, cognitive dysfunction, tremors, or stroke-like symptoms with focal neurological deficits. Cerebellar ataxia (motor incoordination due to dysfunction of the cerebellum) is seen in HE but is a rare occurrence. The objective of the article was to present a case of cerebellar ataxia in a patient with Hashimoto's thyroiditis. A 30-year-old previously healthy female presented with quickly progressive cerebellar ataxia, bilateral (B/L) limb weakness, and excessive tearing. She was found to have high titers of anti-TPO (anti-thyroid peroxidase) antibodies; a biopsy confirmed Hashimoto's thyroiditis and a battery of negative tests excluding other causes of encephalopathy. Hence, confirming a diagnosis of HE. The patient was given glucocorticoids which relieved her symptoms. After being symptom-free for a few months, she relapsed and was unsuccessfully treated by the steroids. Upon this, she was given IV immunoglobulins, which helped achieve complete resolution. HE can be treated with immunotherapy, and most patients have a good prognosis, but some can have persistent neurological defects if left untreated or treatment is delayed. Relapses are common and may require a more extended treatment regimen.

Multiple system atrophy with anti-NAE antibody refractory to immunotherapy: A case report.

A 51-year-old Japanese man presented with slowly progressing cerebellar ataxia from age 49. Anti-thyroglobulin, anti-thyroid peroxidase, and anti-NAE antibodies were detected. Brain magnetic resonance imaging showed moderate atrophy of the pons, cerebellum, and middle cerebellar peduncle. Dopamine transporter single-photon emission computed tomography showed normal uptake. With the diagnosis of Hashimoto's encephalopathy, repeated steroid pulse therapy and intravenous immunoglobulin therapy were administered; however, they were ineffective for cerebellar ataxia. Afterwards, autonomic failure and parkinsonism became evident. The final diagnosis was MSA of the cerebellar type. Immunotherapies for patients with MSA with anti-NAE antibodies should be carefully considered.

Debut with psychotic symptoms of Hashimoto encephalopathy: A case report. / Debut con síntomas psicóticos de encefalopatía de Hashimoto: reporte de caso.

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.

La encefalopatía de Hashimoto es una enfermedad rara reportada por primera vez en 1966 con una prevalencia de 2,1/1000 habitantes. Se presenta el caso de una mujer de 42 años, sin antecedentes médicos de importancia, quien inició un cuadro de manera súbita con alteración de conciencia, alucinaciones visuales y delusiones. En los exámenes de laboratorio se tuvo anticuerpos antiperoxidasa tiroidea mayor a 600 U/ml, tiroxina 0,93, hormona estimulante de la tiroides 1,60 U/ml, resonancia magnética con lesiones focales subcorticales bilaterales de aspecto desmielinizante inespecífico, electroencefalograma sin particularidades. Se realizó el diagnóstico de encefalopatía de Hashimoto y el cuadro remitió luego del tratamiento con corticoides. El artículo resalta la importancia de realizar una evaluación integral de los pacientes con sintomatología psiquiátrica atípica y ahondar en el diagnóstico de exclusión.