Elevated Antithyroid Antibodies Research Articles

Thyroid Function and Morphology in Gaucher Disease: Exploring the Endocrine Implications.

Gaucher disease (GD), the most common ultra-rare metabolic disorder, results from lipid accumulation. Systemic inflammation, cellular stress, and metabolic dysfunction may influence endocrine function, including the thyroid. This study evaluated thyroid function and morphology in 60 GD patients, alongside carbohydrate and lipid metabolism. Anthropometric, biochemical, and hormonal tests were conducted, including thyroid ultrasound and shear-wave elastography (SWE). Clinical data, bone mineral density (BMD), and body composition (BOD POD) analyses were correlated. Healthy controls, matched for age, sex, and body mass index (BMI), were included. GD patients had higher thyroid stimulating hormone (TSH) and free thyroxine (FT4) levels within normal limits. Hypothyroidism occurred in 7%, elevated anti-thyroid antibodies in 8%, and nodular goiter in 23%. Patients with nodular goiter showed lower platelet counts and higher chitotriosidase and glucosylsphingosine (lysoGb-1) levels. Patients with type 3 GD had larger thyroid volumes and greater stiffness on SWE than patients with type 1 GD. GD patients also exhibited increased metabolic risk, including central obesity and elevated glucose levels. GD patients, despite normal thyroid hormone levels, exhibit subtle alterations in thyroid function indicators. Their increased risk of central obesity and glucose metabolism disorders, alongside higher TSH and FT4 levels, underscores the need for closer monitoring and further investigation.

Steroid response encephalopathy associated with autoimmune thyroiditis

IntroductionSteroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare autoimmune disorder affecting the central nervous system, characterized by a spectrum of neurological and psychiatric symptoms.AimThis case study aims to highlight the diagnostic challenges and the successful management of SREAT syndrome in a young woman with autoimmune thyroiditis.Case studyA 21-year-old woman with documented Hashimoto's thyroiditis and polycystic ovary syndrome was admitted to the Clinic of Neurology following a 5-minute tonic seizure and subsequent confusion state lasting several hours. Initial brain MRI showed no abnormalities, and EEG revealed generalized slowness. Comprehensive laboratory assessments, including a complete blood count, biochemical analysis, and electrolyte panels, all yielded normal results. Further investigation revealed a significantly elevated anti-thyroid peroxidase antibody (anti-TPO) titer exceeding 1000 IU/mL. The suspicion of SREAT syndrome was considered. Pulse therapy with methylprednisolone was associated with rapid recovery. The patient was discharged from the hospital with an oral corticosteroid tapering regimen.Results and discussionThe administration of pulse therapy with methylprednisolone resulted in a rapid and very good response in the patient, evidenced by the resolution of seizure activity and improvement in confusion. Laboratory investigations, particularly the markedly elevated anti-TPO titer, supported the diagnosis of SREAT syndrome. The subsequent management with an oral corticosteroid tapering regimen maintained the patient's clinical stability.ConclusionsThis case highlights the importance of considering autoimmune encephalopathy in patients with a history of autoimmune thyroiditis presenting with neurological and psychiatric symptoms. Further research is warranted to better understand the underlying pathomechanisms.

Hashimoto’s Encephalopathy: A Review

The condition "Hashimoto's encephalopathy" (HE) refers to a cerebral dysfunction syndrome with elevated antithyroid peroxidase antibody titers that is thought to have an autoimmune cause. Similar to autoimmune thyroid illness, women are more likely than men to develop HE. It has been documented in adult, geriatric, and pediatric populations worldwide. The clinical appearance may be recurrent and remitting, with myriad symptoms involving myoclonus, seizures, episodes resembling strokes, cognitive deterioration, and neuropsychiatric symptoms. Clinically and biochemically, thyroid function is often normal. Although HE seems to be a rare disorder, it should be taken into consideration when treating "investigation negative encephalopathies" because it responds to corticosteroid treatment. The results of neuroimaging frequently do not help to clarify the diagnosis. High titers of antithyroid antibodies, specifically antithyroid peroxidase antibodies, are diagnosed in the setting of the usual clinical picture. Corticosteroid treatment is nearly always successful, although relapses are common. Other immunomodulatory treatments, like intravenous immunoglobulin and plasma exchange, are often effective as well. Despite a statistical correlation to autoimmune thyroid illness, the cause of HE is not well understood. Research is still needed to fully understand the connections between clinical images, thyroid disease, auto-antibody pattern, and brain pathology. It's possible that over time, a class of nonvasculitic autoimmune inflammatory meningoencephalopathies will include HE. Some writers have proposed severing all connections to Hashimoto and calling the illness, SREAT - "steroid responsive encephalopathy associated with autoimmune thyroiditis".

Use of thyroid hormones in hypothyroid and euthyroid patients: a 2022 THESIS questionnaire survey of members of the Latin American Thyroid Society (LATS)

PurposeInconsistencies in the medical management of hypothyroidism have been reported between endocrinologists in different countries. This study aimed to identify the attitudes of Latin America thyroid specialists towards the use of thyroid hormones.MethodsOnline survey of members of the Latin America Thyroid Society.Results81/446 (18.2%) completed the questionnaire. Levothyroxine (LT4) was the initial treatment of choice for all respondents. 56.8% would consider LT4 use in biochemically euthyroid patients: infertile women with elevated anti-thyroid antibodies (46.9%), resistant depression (17.3%) and growing goiter (12%). Most respondents preferred tablets (39.5%) over liquid formulations (21.0%) or soft gel capsules (22.2%) and would not consider switching formulations in patients with persistent symptoms. 39.5% would never use LT4 + liothyronine (LT3) combination therapy in symptomatic euthyroid patients, due to low quality evidence for benefit. 60.5% reported that persistence of symptoms despite normal TSH is rare (below 5% of patients) and its prevalence has been stable over the last five years. Psychosocial factors (84.0%), comorbidities (86.4%) and the patient unrealistic expectation (72.8%) were considered the top three explanations for this phenomenon.ConclusionLT4 tablets is the treatment of choice for hypothyroidism. A significant proportion of respondents would use LT4 in some groups of euthyroid individuals, contrasting the recommendations of the major clinical practice guideline indications. LT4 + LT3 combination treatment in euthyroid symptomatic patients was considered by nearly 50%. Practices based on weak or absent evidence included use of thyroid hormones for euthyroid subjects by 56.8% of respondents and use of LT4 + LT3 treatment by 60.5% of respondents for patients with persistent symptoms. In contrast to many European countries, LATS respondents report a low and unchanged proportion of dissatisfied patients over the last five years.

About a case: affective psychosis and hyperthyroidism

IntroductionHyperthyroidism due to Graves-Basedow disease is a common cause of neuropsychiatric manifestations, such as anxiety, psychomotor restlessness, mood disturbances, insomnia and psychosis. Hashimoto’s encephalopathy rarely occurs in so-called autoimmune thyroiditis, which can present with hyperthyroidism and neuropsychiatric symptoms similar to Graves’ disease. We add that the mystical-religious beliefs, present in all human cultures, and decisive in the case at hand, make us propose an evolutionary origin of them.ObjectivesClinical case descriptionMethodsA clinical case based on medical reports is describedResultsWe present the case of a 72-year-old woman, a member of the Seventh-day Adventist Church, well adapted to the Community. Known history of elevated antithyroid antibodies since 2019, brought to the emergency room involuntarily due to a mystical-religious delusional condition associated with behavioral disturbance. On examination, cachectic appearance, distal tremor, emotional exaltation and megalomanic speech were highlighted. Laboratory tests revealed primary hyperthyroidism with elevated antibodies. During admission, the differential diagnosis between Graves-Basedow disease and Hashimoto’s encephalopathy was considered. Thyroid scintigraphy oriented the diagnosis to Graves-Basedow disease, not requiring lumbar puncture or corticosteroid treatment. Treatment was based on high-dose antithyroid and antipsychotic drugs, with clinical and analytical remission at 3 weeks. The patient was referred to a Social Health Center for functional recovery. The family refers to a similar episode in 2014, of less intensity and self-limited, which is proposed to be a hashitoxicosis.ConclusionsDifferential diagnosis between Graves-Basedow and Hashimoto disease is essential as they differ in treatment and prognosis. The continuity that the delusion presents with the previous beliefs of the patient, differing mainly in the affective-behavioral implication, makes us consider a predisposition to psychosis in our patient. Religiosity can be adaptive in certain environments, since mystical beliefs have existed throughout the history of the human species and seem to be part of our nature.Disclosure of InterestNone Declared

Nivoi vitamina D i genetski variant VDR rs2228570 u autoimunom tireoditisu

Autoimmune Thyroiditis (AIT) is a prevalent autoimmune disorder characterized by an immune response targeting the thyroid gland. Genetic factors play a significant role in AIT susceptibility, with immune-related genes, especially the vitamin D receptor (VDR) gene, potentially influencing AIT development. This comprehensive review delves into the intricate association between VDR gene polymorphisms, particularly rs2228570 (FokI), and AIT susceptibility, exploring various populations. Research has unveiled contrasting outcomes regarding the link between the VDR rs2228570 polymorphism and AIT risk across diverse ethnic groups. Certain populations have exhibited a noteworthy correlation, suggesting that population-specific genetic factors contribute to AIT risk. A recurring observation of vitamin D deficiency in AIT patients has correlated with elevated anti-thyroid antibodies, abnormal thyroid function , and thyroid volume. The results emphasize the possible role of vitamin D in the development of AIT, suggesting the importance of vitamin D supplementation to address deficiencies associated with AIT. In addition to VDR rs2228570, other genetic variants have also shown associations with AIT susceptibility, displaying varying results across different populations. Ethnicity emerges as a pivotal factor influencing these associations, underscoring the need to consider genetic variations in diverse populations. We emphasize the intricate interplay between VDR gene polymorphisms, vitamin D, and AIT susceptibility. Extensive research is essential to unveil the clinical significance of these genetic variations, offering prospects for enhanced diagnostic and therapeutic strategies for individuals with AIT.

Lymphocytic Thyroiditis- Association between Cytology and Biochemical Findings

Introduction: Hashimoto’s thyroiditis otherwise called as chronic lymphocytic thyroiditis is the most common cause of hypothyroidism and one of the most common thyroid lesions in Fine Needle Aspiration Cytology (FNAC). Diagnosis depends on clinical findings, ultrasonographic findings, thyroid profile and antithyroid antibody levels along with the FNAC findings. Not much studies are there which have assessed the association of the clinical and biochemical parameters with the cytological findings. Aim: To grade the cytological features of lymphocytic thyroiditis and to study the association of the grades with Thyroid Function Test (TFT) and antithyroid antibody levels. Materials and Methods: A prospective time bound analytical observational study of one year was carried out in the Department of Pathology at Government Medical College, Palakkad, Kerala, India after obtaining approval from the Institutional Ethics Committee (IEC). Out of the total of 462 cases of thyroid lesions who underwent Fine Needle Aspiration (FNA), 147 cases were lymphocytic thyroiditis as per cytology. Of these, 40 cases had Thyroid Stimulating Hormone (TSH), Antithyroglobulin Antibody (ATG) and Antithyroid Peroxidase Antibody (ATPO) levels known which were included in the study. Cytological grading of those were done and the association of the same with the above mentioned parameters was studied. Data was analysed using Statistical Package for the Social Sciences (SPSS) software. Descriptive statistics were calculated and presented as percentages, mean and Standard Deviation (SD). Chi-square test was used to find the association between the categorical variables. The p-value <0.05 was taken as statistically significant. Results: Cytomorphology was diagnostic of thyroiditis in all the cases. Out of the 40 cases, 32 were females.The most common age group affected was 31-40 years, though it ranged from 18-68 years. Most common clinical presentation was diffusely enlarged thyroid, though a few presented as nodular disease. Most of the patients had grade 1 thyroiditis (23 cases) followed by grade 2 (13 cases). Elevated antithyroid antibody levels - ATPO elevated in 32 cases and ATG in 20 cases. TSH was seen elevated in 28 cases of thyroiditis. No association was observed with the antithyroid antibody levels and the cytological grade. Conclusion: Lymphocytic infiltration into the follicles is the diagnostic cytological feature of chronic lymphocytic thyroiditis. TSH elevation and positive ATPO levels strongly suggest Hashimoto’s thyroiditis. No association was found between the cytological grade and TSH and antibody levels.

Effects of animal protein intake during pregnancy on autoimmune thyroiditis in pregnant women with mild iodine deficiency.

Iodine and animal protein may affect thyroid function. In the present study, we explored the association between animal protein intake and thyroid antibody status in pregnant women following universal salt iodisation. Pregnant women were enrolled using a multistage, stratified random sampling method in Shanghai. In total, 4646 eligible women were interviewed in person. We used a validated food frequency questionnaire and food composition tables to calculate the daily intakes of protein and iodine. We collected urine samples and performed thyroid antibody tests. Positive thyrotropin receptor antibody (TR-Ab) rates were different among animal protein intake groups (p < 0.05). Median urinary iodine concentration (UIC) was higher in the thyroid peroxidase antibody (TPO-Ab) positive group than in the negative group (p < 0.05). The median of total protein intake, animal protein intake and UIC was higher in the TR-Ab positive group than in the negative group (p < 0.05). The median of total protein intake and UIC was higher in the TPO-Ab/TG-Ab/TR-Ab positive group than in the negative group (p < 0.05). Multivariable logistic regression results showed that insufficient iodine had a negative correlation with positive TPO-Ab and positive TR-Ab (p < 0.05). The middle third and top third animal protein intakes served as protective factors for TR-Ab (coefficient = 0.559, 95% confidence interval [CI] = 0.415-0.752, p < 0.001; coefficient = 0.0.406, 95% CI = 0.266-0.621, p < 0.001) and positive TPO-Ab/TR-Ab/TG-Ab (coefficient = 0.817, 95% CI = 0.687-0.971, p = 0.022; coefficient = 0.805, 95% CI = 0.672-0.964, p = 0.018). Adequate animal protein intake protects against elevated anti-thyroid antibody levels in pregnant women with mild iodine deficiency.

Steroid-Responsive Encephalopathy Associated with Thyroiditis

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

Atypical euthyroid presentation of Steroid Responsive Encephalopathy with Associated Thyroiditis

Introduction&#x0D; Steroid-responsive encephalopathy with associated thyroiditis (SREAT) is an autoimmune disease associated with antithyroid antibodies. Its clinical features are variable, ranging from sudden focal neurologic deficits to progressive sub-acute cognitive decline and thus, can mimic a host of other neurological and psychiatric conditions.&#x0D; Case Presentation&#x0D; We present the case of a previously healthy 62-year-old female with rapid onset neurocognitive and functional decline. EEG and MRI findings were consistent with an encephalopathy of unknown origin. Serologic findings revealed elevated antithyroid antibodies but were otherwise insignificant.&#x0D; Conclusion&#x0D; Steroid-responsive encephalopathy with associated thyroiditis is typically a diagnosis of exclusion but should always be considered in cases of encephalopathy of unknown origin. The disorder is often underdiagnosed due to its variable presentation and poorly understood pathophysiology, but prognosis can be significantly improved with greater physician awareness and prompt management with corticosteroids.

Overt Thyroid Dysfunction and Anti-Thyroid Antibodies Predict Response to Anti-PD-1 Immunotherapy in Cancer Patients.

Background: Thyroid dysfunction is among the most common adverse effects during anti-programmed cell death 1 (PD-1) immunotherapy, and alongside correlations with elevated anti-thyroid antibodies (ATAb), studies have found correlations with survival. However, the exact relations remain to be clarified. We, therefore, aimed at clarifying the relationship between thyroid dysfunction, ATAbs, and survival in anti-PD-1 treated cancer patients. Methods: We included 168 patients with nonsmall-cell lung carcinoma, renal cell carcinoma, and metastatic melanoma treated with nivolumab or pembrolizumab. Thyrotropin and free T4 (fT4) levels were measured before each anti-PD-1 infusion. ATAb levels (anti-thyroid peroxidase [TPO] and anti-thyroglobulin [Tg]) were measured at baseline and after two months of treatment. Although the vast majority of patients had detectable levels of ATABs, only a few patients had positive ATAbs when using conventional cut-offs. To study the consequences of detectable ATABs, the cut-off levels were a priori set at the median concentrations at baseline in the study population. Tumor progression was classified according to RECIST v1.1. Results: Patients who acquired overt thyroid dysfunction during treatment had significantly higher overall survival (OS) (hazard ratio [HR] = 0.18 confidence interval [CI: 0.04-0.76]; p = 0.020) and progression-free survival (PFS) (HR = 0.39 [0.15-0.998]; p = 0.050) than patients without thyroid dysfunction with 1-year OS rates of 94% vs. 59% and 1-year PFS rates of 64% vs. 34%. During treatment, patients with ATAb levels above the median had a higher OS (HR = 0.39 [0.21-0.72]; p = 0.003) and PFS (HR = 0.52 [0.33-0.81]; p = 0.004) than patients with ATAb levels below the median, with 1-year OS rates of 83% vs. 49% and PFS rates of 54% vs. 20%, respectively. When analyzing ATAb levels over time, patients with a persistent ATAb level above the median had a higher OS (HR = 0.41 [0.19-0.89], p = 0.025) and PFS (HR = 0.54 [0.31-0.95], p = 0.032) compared with patients with a persistent ATAb level below the median. Patients whose ATAb levels increased above the median during treatment had an improved OS (HR = 0.27 [0.06-1.22], p = 0.088) and PFS (HR = 0.24 [0.07-0.77], p = 0.017) compared with patients whose ATAb levels decreased below the median. Conclusions: Acquired overt thyroid toxicity and above median ATAb levels during anti-PD-1 treatment are associated with improved PFS and OS. In addition, our results suggest that ATAb levels at baseline are of clinical relevance for PFS and OS.

1295P - Thyroid toxicity and anti-thyroid antibodies as predictive markers for patients treated with anti-PD1 checkpoint therapy

BackgroundThyroid dysfunction is one of the most common adverse effects during anti-PD-1 therapy, and alongside elevated anti-thyroid antibodies (ATAb) it is correlated with overall (OS) and progression free survival (PFS). The objective and novelty of our study was the simultaneous investigation of thyroid dysfunction and ATAbs on survival. MethodsWe included 168 patients with non-small cell lung carcinoma (n=93), renal cell carcinoma (n=12), and advanced and metastatic melanoma (n=63) treated with nivolumab or pembrolizumab. TSH and fT4 serum levels were measured prior to each anti-PD-1 infusion and ATAb titers in sera, i.e. anti-TPO and anti-Tg (cut-off: 3.05IU/ml and 22.35IU/ml, respectively; based on median value at baseline), at baseline, and after 2 months of treatment. Thyroid dysfunction was based on TSH and fT4 and classified as subclinical or overt. Tumor progression was classified according to RECIST v1.1 and was monitored until progression, death or withdrawal of the study. Cox regression was used with correction for tumor type. ResultsPatients who acquired overt thyroid toxicity during anti-PD-1 treatment had significantly higher OS (HR=0.17 [95% CI: 0.04-0.74]; p=0.018) and PFS (HR=0.38 [0.15-0.98]; p=0.05) than patients without thyroid toxicity with one-year OS rates of 95% vs 64% and one-year PFS rates of 65% vs 33%. Moreover, patients with positive ATAb status during treatment had higher OS (HR=0.39 [0.21-0.72]; p=0.003) and PFS (HR=0.52 [0.33-0.81]; p=0.004) than patients with negative ATAb status with one-year OS rates of 83% vs 49% and PFS rates of 54% vs 20%, respectively. For 84% of patients the ATAb status at baseline was the same as during treatment. Patients with persistent positive ATAb status (48%) had higher OS (HR=0.41 [0.19-0.89], p=0.03) and PFS (HR=0.54 [0.31-0.95], p=0.03) compared to patients with persistent negative ATAb status (36%). ConclusionsAcquired overt thyroid toxicity and positive ATAb status during anti-PD-1 treatment are associated with improved PFS and OS. Moreover, our results indicate that ATAb status at baseline is of clinical relevance for PFS and OS. If validated, these parameters may serve as a novel positive predictive markers. Clinical trial identificationDutch trial register: MULTOMAB, NL6828. Legal entity responsible for the studyErasmus Medical Center. FundingErasmus Medical Center. DisclosureA.A.M. Van der Veldt: Advisory / Consultancy: Bristol-Myers Squibb; Advisory / Consultancy: Merck Sharp & Dohme. R.H. Mathijssen: Research grant / Funding (institution), Travel / Accommodation / Expenses: Astellas; Research grant / Funding (institution): Bayer; Research grant / Funding (institution): Boekringer; Research grant / Funding (institution): Cristal Therapeutics; Honoraria (self), Research grant / Funding (institution): Novartis; Research grant / Funding (institution): Pamgene; Research grant / Funding (institution), Travel / Accommodation / Expenses: Pfizer; Research grant / Funding (institution): Roche; Research grant / Funding (institution): Sanofi; Honoraria (self): Servier. All other authors have declared no conflicts of interest.

SUN-LB099 Hashimoto's Encephalopathy: The Needle in the Haystack

Background: Hashimoto’s encephalopathy (HE) is a rare heterogeneous disorder first described in 1966 and presents with variable neuropsychiatric dysfunction, including cognitive decline, seizures, myoclonus, and psychiatric manifestations. HE is likely an autoimmune vasculitis rather than a thyroid dysfunction, and is steroid responsive. Clinical case: A 64-year-old Caucasian female with a past history of tobacco abuse presents with altered level of consciousness, left frontal headache, and a mechanical fall resulting in a radial fracture. No chest pain or syncopal episode. She was noted to have persistent confusion and flu-like symptoms for three days. Review of systems were otherwise negative. Patient does not take any medications. Surgical and social histories were unremarkable. Vital signs: BP 131/59, HR 97, RR 16, Tmax 103.4, Sat 97%. Neurologic examination revealed episodes of confusion with paraphasic speech errors. She was unable to correctly recall her name. No dysarthria, facial asymmetry, or motor deficits. Laboratories: WBC 19,100uL (4,000-10,000uL). Chemistries revealed normal electrolytes and hepatic function, coagulation studies, urinalysis, B12, TSH, and toxicology. Autoimmune and paraneoplastic etiologies were excluded. Lumbar puncture revealed elevated protein 102.7mg/dL (15-45mg/dL) and WBC 54/uL (0-10/uL) with 100% mononuclear predominance. Head CT revealed low attenuation in the right posterior temporal lobe. MRI brain without/with contrast revealed chronic ischemic vessel disease. EEG noted mild generalized slowing and disorganization. Patient was initially treated for viral encephalitis until CSF PCR analyses for a broad spectrum of pathogens was negative. Further serum studies including thyroglobulin antibody and thyroid peroxidase antibody were sent, noting values of 63.8U/mL (<60U/mL) and >1300U/mL (<60U/mL), respectively. She was treated with Methylprednisolone 250mg IV daily for three days and transitioned to a Prednisone 60mg daily taper. She had immediate and complete resolution of her symptoms within the first 24 hours of corticosteroid therapy. Conclusion: Hashimoto's encephalopathy is a diagnosis of exclusion as it can mimic meningitis, encephalitis, psychiatric disorders, stroke, vasculitis, and migraines. Persistent confusion and fevers may cause a delay in diagnosis. HE affects women predominantly in the 5-6th decade. Diagnosis can be made with an elevated anti-thyroid peroxidase antibody and/or antithyroglobulin antibody. Severity does not correspond with serum levels. HE responds well to corticosteroids, including 50-150mg Prednisone daily, with 90-98% response rate, and may take months for complete resolution of symptoms. Refractory cases may require immunosuppressive agents. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

P.006 Role of repeat antithyroid antibody testing in the diagnosis of Hashimoto’s Encephalopathy

Background: We present a case of a previously well 71-year-old woman who developed rapidly progressive dementia. She had several prolonged hospital admissions and extensive investigations were performed. Her illness was steroid-responsive with clinical features suggestive of Hashimoto’s Encephalopathy (HE). However, multiple thyroid antibody panels acquired were initially normal, becoming elevated during subsequent relapses that occurred 3 years after initial presentation. The case signifies the importance of repeating antithyroid antibody levels in a patient with a clinical picture suggestive of HE. Methods: Case report. Results: The diagnosis of HE was established because of a compatible clinical picture including a relapsing encephalopathy with motor involvement and steroid responsiveness. In addition, although initial antithyroid antibody testing was negative, subsequent attacks were associated with significant elevations and reverted to normal with resolution of the attacks. Conclusions: In a patient with normal levels of antithyroid antibodies and a clinical presentation suggestive of HE, we recommend repeat antithyroid antibody testing to confirm diagnosis. Further studies are necessary to clarify the pathogenic role of elevated antithyroid antibodies in the mechanism of HE.

A Rare Case of Creutzfeldt-Jakob Disease with Elevated Anti-Thyroid Antibodies (P5.140)

A Rare Case of Creutzfeldt-Jakob Disease with Elevated Anti-Thyroid Antibodies (P5.140)

Hashimoto Encephalopathy: Encephalitis versus Vasculopathy and the Impact of Concussion

Patients with Hashimoto encephalitis may present with seizures, stroke-like episodes, transient focal and global neurological deficits, and a variety of neuropsychiatric disturbances. The encephalopathy evolves with elevated anti-thyroid peroxidase antibodies, independent of hormonal thyroid function. A vasculitic basis is considered in cases that show inflammatory vasculopathy in brain biopsy and postmortem tissues. A relation to autoimmune encephalitis is supported by etiologic localization of the disturbance to vulnerable areas of the mesial temporal lobe and the association of potentially pathogenic antibodies. Both mechanisms presume disruption of the blood-brain barrier. We report a patient with Hashimoto encephalopathy in whom repeated concussions antedated onset of the disorder with overlapping areas of altered brain metabolism and vascular perfusion in the hippocampus, deemed to be most vulnerable in autoimmune encephalopathy and concussive brain injury.

Hashimoto Encephalopathy: Impact of Concussion

Patients with Hashimoto encephalitis may present with seizures, stroke-like episodes, transient focal and global neurological deficits, and a variety of neuropsychiatric disturbances. The encephalopathy evolves with elevated anti-thyroid peroxidase antibodies, independent of hormonal thyroid function. A teenager experienced repeated concussions antedating onset of Hashimoto encephalopathy. Neuroradiological studies showed overlapping areas of altered brain metabolism and vascular perfusion in the hippocampus, deemed most vulnerable in autoimmune encephalopathy. Concussive brain injury may be a contributing factor in the development of encephalopathy due to its capacity to alter the blood-brain barrier.

Physiological serum 25-hydroxyvitamin D concentrations are associated with improved thyroid function\u2014observations from a community-based program

PurposeVitamin D deficiency has been associated with an increased risk of hypothyroidism and autoimmune thyroid disease. Our aim was to investigate the influence of vitamin D supplementation on thyroid function and anti-thyroid antibody levels.MethodsWe constructed a database that included 11,017 participants in a health and wellness program that provided vitamin D supplementation to target physiological serum 25-hydroxyvitmain D [25(OH)D] concentrations (>100 nmol/L). Participant measures were compared between entry to the program (baseline) and follow-up (12 ± 3 months later) using an intent-to-treat analysis. Further, a nested case-control design was utilized to examine differences in thyroid function over 1 year in hypothyroid individuals and euthyroid controls.ResultsMore than 72% of participants achieved serum 25(OH)D concentrations >100 nmol/L at follow-up, with 20% above 125 nmol/L. Hypothyroidism was detected in 2% (23% including subclinical hypothyroidism) of participants at baseline and 0.4% (or 6% with subclinical) at follow-up. Serum 25(OH)D concentrations ≥125 nmol/L were associated with a 30% reduced risk of hypothyroidism and a 32% reduced risk of elevated anti-thyroid antibodies. Hypothyroid cases were found to have higher mean serum 25(OH)D concentrations at follow-up, which was a significant positive predictor of improved thyroid function.ConclusionThe results of the current study suggest that optimal thyroid function might require serum 25(OH)D concentrations above 125 nmol/L. Vitamin D supplementation may offer a safe and economical approach to improve thyroid function and may provide protection from developing thyroid disease.

Hashimoto encephalopathy in pediatric patients: Homogeneity in clinical presentation and heterogeneity in antibody titers

ObjectiveHashimoto encephalopathy is an autoimmune encephalopathy characterized by elevated antithyroid antibodies and a favorable response to corticosteroid. This study delineated the clinical characteristics of pediatric Hashimoto encephalopathy and the significance of low antithyroid antibody titers in diagnosis and treatment. Subjects and methodsClinical manifestations, antibody titers, and treatment responses were retrospectively reviewed in six consecutive children diagnosed with Hashimoto encephalopathy between August 2008 and July 2016. ResultsAge at diagnosis was 10–17years. Presenting symptoms were seizures, altered consciousness, behavioral changes, psychosis, tremor, and dystonia. Thyroid function was normal in five patients, and one had hypothyroidism prior to the encephalopathy. Antithyroid antibody titer was increased at presentation in five patients and one week later in the other. Antibody levels were extremely varied (anti-thyroglobulin, 20.5–2318.0U/ml; anti-thyroid peroxidase, 12.5–2231.0U/ml; reference range, <60U/ml) and <180U/ml in two patients. Electroencephalogram was abnormal in five patients. Brain magnetic resonance imaging was unremarkable. Four patients responded to high-dose corticosteroid and one improved with additional intravenous immunoglobulin. The remaining patient did not respond to both treatments and normalized after plasmapheresis. Autoantibody titers decreased with treatment response in the acute stage. Two patients with low antibody titers showed similar clinical presentations and responses. ConclusionsThe clinical presentations and treatment responses in Hashimoto encephalopathy were similar, irrespective of antithyroid antibody titer. Because the initial antithyroid antibody titers can be normal or mildly-elevated, follow-up testing of antithyroid antibodies is required in patients who are clinically suspect for Hashimoto encephalopathy.

Hashimoto’s Thyroiditis and Encephalopathy

Although very rare, the literature describes cases of Hashimoto encephalopathy attesting to its occurrence. Affected patients present with seizures, stroke-like episodes, transient focal and global neurological deficits, and a variety of neuropsychiatric disturbance from dementia to hallucinations and psychosis. The encephalopathy evolves with concomitantly elevated anti-thyroid peroxidase antibodies, independent from hormonal thyroid function setting it apart from thyrotoxicosis and myxedema. A new patient reported with Hashimoto’s encephalopathy, in whom neurocognitive impairment and stroke-like onset of hemiparesis and hemiparkinsonism correlated with brain magnetic resonance imaging fused with positron emission tomography.