Hashimoto Encephalopathy also Known as Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT)

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.

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Episodic Hemiparesis, Cognitive Decline, and Seizures in a Woman With Pernicious Anemia

10.1093/med/9780197583425.003.0027 ◽

2021 ◽

pp. 90-92

Author(s):

Cristina Valencia-Sanchez ◽

Andrew McKeon

Keyword(s):

Pernicious Anemia ◽

Autoimmune Thyroiditis ◽

Oral Prednisone ◽

Hashimoto Thyroiditis ◽

Vitamin D Supplementation ◽

Her Family ◽

Migraine Headaches ◽

Fluid Analysis ◽

Hashimoto Encephalopathy ◽

History Of

A 46-year-old woman with a history of pernicious anemia, sought care for intermittent episodes of weakness in her right upper extremity and speech difficulties followed by a headache. It was initially thought that she had migraine headaches. Blood tests showed increased thyrotropin and low free thyroxine levels, and she was diagnosed with Hashimoto thyroiditis. She initiated treatment with levothyroxine. One month later, the patient had a confusional episode and over the course of the following 2 months, the cognitive difficulties progressed. She was brought to the emergency department after a generalized tonic-clonic seizure. On examination, she was profoundly encephalopathic. Formal bedside cognitive testing could not be obtained. Findings of magnetic resonance imaging of the brain were normal. Electroencephalography revealed diffuse slowing. Cerebrospinal fluid analysis showed increased protein concentration and lymphocytic pleocytosis. Thyroid peroxidase antibody value was markedly increased. Thyroglobulin antibody value was also increased. Consistent with her history of pernicious anemia, she was positive for gastric parietal cell antibodies. The clinical presentation was compatible with steroid-responsive encephalopathy associated with autoimmune thyroiditis, also known as Hashimoto encephalopathy. The patient started levetiracetam therapy and had no further seizures. She received intravenous methylprednisolone. At the completion of treatment, her confusion rapidly and substantially improved. She was discharged home on oral prednisone. She also started pantoprazole, calcium, and vitamin D supplementation and Pneumocystis jirovecii prophylaxis. For long-term immunotherapy, she initiated methotrexate. Three months later, the patient and her family reported 90% improvement in her cognitive functioning and resolution of the episodes of hemiparesis. The patient continued tapering prednisone. It was recommended that she continue methotrexate for 5 years before discontinuing. Steroid-responsive encephalopathy associated with autoimmune thyroiditis, also known as Hashimoto encephalopathy, was initially described as strokelike episodes and subacute encephalopathy months after the onset of autoimmune (Hashimoto) thyroiditis.

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Hashimoto Encephalopathy as a Complication of Autoimmune Thyroiditis

Medical Principles and Practice ◽

10.1159/000494800 ◽

2018 ◽

Vol 28 (1) ◽

pp. 91-95 ◽

Cited By ~ 2

Author(s):

Alixandria A. Fiore ◽

Wilson B. Pfeiffer ◽

Syed A.A. Rizvi ◽

Anais Cortes ◽

Conrad Ziembinski ◽

...

Keyword(s):

Case Report ◽

Cognitive Decline ◽

Autoimmune Thyroiditis ◽

Initial Treatment ◽

Clinical Presentation ◽

Diagnostic Workup ◽

Visual Hallucinations ◽

Intravenous Methylprednisolone ◽

Hashimoto Encephalopathy ◽

Rapid Cognitive Decline

Objective: To present a case of Hashimoto encephalopathy as a complication of autoimmune thyroiditis. Clinical Presentation and Intervention: A previously healthy 56-year-old female presented with rapidly progressive cognitive decline and visual hallucinations. Being a diagnosis of exclusion, Hashimoto encephalopathy required an extensive laboratory and diagnostic workup, which was done over the course of a 15-day hospitalization. The patient recovered after initial treatment with intravenous methylprednisolone and was then switched to prednisone p.o. Conclusion: This case report illustrates the importance of awareness for Hashimoto encephalopathy, as it remains one of the few easily treatable and reversible causes of rapid cognitive decline.

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Anti-Thyroid Peroxidase and Anti-Thyroglobulin Autoantibodies in the Cerebrospinal Fluid of Patients with Unipolar Depression

Journal of Clinical Medicine ◽

10.3390/jcm9082391 ◽

2020 ◽

Vol 9 (8) ◽

pp. 2391

Author(s):

Rick Dersch ◽

Ludger Tebartz van Elst ◽

Benedikt Hochstuhl ◽

Bernd L. Fiebich ◽

Oliver Stich ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Autoimmune Thyroiditis ◽

Unipolar Depression ◽

Hashimoto Thyroiditis ◽

Serum Markers ◽

Thyroid Peroxidase ◽

Hashimoto Encephalopathy ◽

Thyroglobulin Autoantibodies ◽

Brain Involvement ◽

Paired Serum

Introduction: The risk of developing depression is increased in patients with autoimmune thyroiditis. Autoimmune Hashimoto thyroiditis is diagnosed using the serum markers anti-thyroid peroxidase (TPO) and anti-thyroglobulin (TG) antibodies. In rare cases, patients with autoimmune thyroiditis can also suffer from the heterogeneous and ill-defined syndrome of Hashimoto encephalopathy. Biomarkers for Hashimoto encephalopathy or for any brain involvement of autoimmune thyroiditis are currently lacking. The aim of the present descriptive study was therefore to determine whether a subgroup of seropositive patients shows intrathecal anti-thyroid antibody synthesis in the cerebrospinal fluid (CSF). Participants and methods: Paired serum and CSF samples from 100 patients with unipolar depression were examined for anti-TPO and anti-TG antibodies using enzyme-linked immunosorbent assays. Antibody-specific indices (ASIs) were calculated for seropositive samples. These ASIs allow the differentiation between the brain-derived fraction of antibodies and antibodies which are passively diffused from the serum. ASIs >1.4 were assessed as positive for brain-derived antibodies. Additionally, for explorative evaluations, a stricter ASI limit of >2 was applied. Results: Anti-TPO antibodies were increased in the serum of 16 patients (16%); increased anti-TPO ASIs (>1.4) were detected in 11 of these patients (69%). Anti-TG antibodies in the serum were detected in three patients (3%), with two of them (67%) showing increased ASIs (>1.4). Overall, the authors found increased anti-thyroid antibodies in 17 of 100 patients (17%), with 13 out of 17 patients showing increased ASIs (76%; range 1.4–4.1). Choosing ASI levels of >2 led to positive findings in six out of 16 patients (38%) with anti-TPO antibodies in their serum but no increase in ASIs in three patients (0%) who were seropositive for anti-TG antibodies. The patients with elevated ASIs (N = 13) were younger than the ASI-negative patients (N = 87; p = 0.009); no differences were noted in the frequency of CSF, electroencephalography, and/or magnetic resonance imaging alterations. Discussion: A subgroup of seropositive patients showed intrathecal synthesis of anti-TPO and, more rarely, of anti-TG antibodies, which might be an indication of central autoimmunity in a subgroup of patients with unipolar depression. The confirmation of elevated ASIs as a biomarker for Hashimoto encephalopathy must await further studies. The relevance of the findings is limited by the study’s retrospective and uncontrolled design.

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The systemic disease: autoimmune thyroiditis – interdisciplinary collaboration: Homeopathy – micro-immunotherapy – orthomolecular therapy

Allgemeine Homöopathische Zeitung ◽

10.1055/s-0037-1601096 ◽

2017 ◽

Vol 262 (02) ◽

pp. 2-76

Author(s):

I Chammah ◽

H Orfanos-Boeckel ◽

G Schlegel

Keyword(s):

Autoimmune Thyroiditis ◽

Interdisciplinary Collaboration ◽

Systemic Disease

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SERUM THYROTROPHIN AND THE RESPONSE TO THYROTROPHIN RELEASING HORMONE IN SYMPTOMLESS AUTOIMMUNE THYROIDITIS AND IN BORDERLINE AND OVERT HYPOTHYROIDISM

Acta Endocrinologica ◽

10.1530/acta.0.0750274 ◽

1974 ◽

Vol 75 (2) ◽

pp. 274-285 ◽

Cited By ~ 42

Author(s):

A. Gordin ◽

P. Saarinen ◽

R. Pelkonen ◽

B.-A. Lamberg

Keyword(s):

Autoimmune Thyroiditis ◽

Elevated Serum ◽

Mean Value ◽

Primary Hypothyroidism ◽

Overt Hypothyroidism ◽

Thyrotrophin Releasing Hormone ◽

Releasing Hormone ◽

The Mean ◽

The Individual ◽

Serum Tsh

ABSTRACT Serum thyrotrophin (TSH) was determined by the double-antibody radioimmunoassay in 58 patients with primary hypothyroidism and was found to be elevated in all but 2 patients, one of whom had overt and one clinically borderline hypothyroidism. Six (29%) out of 21 subjects with symptomless autoimmune thyroiditis (SAT) had an elevated serum TSH level. There was little correlation between the severity of the disease and the serum TSH values in individual cases. However, the mean serum TSH value in overt hypothyroidism (93.4 μU/ml) was significantly higher than the mean value both in clinically borderline hypothyroidism (34.4 μU/ml) and in SAT (8.8 μU/ml). The response to the thyrotrophin-releasing hormone (TRH) was increased in all 39 patients with overt or borderline hypothyroidism and in 9 (43 %) of the 21 subjects with SAT. The individual TRH response in these two groups showed a marked overlap, but the mean response was significantly higher in overt (149.5 μU/ml) or clinically borderline hypothyroidism (99.9 μU/ml) than in SAT (35.3 μU/ml). Thus a normal basal TSH level in connection with a normal response to TRH excludes primary hypothyroidism, but nevertheless not all patients with elevated TSH values or increased responses to TRH are clinically hypothyroid.

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