Encefalitis de Hashimoto. Presentación de un caso / Hashimoto Encephalitis. Case Report

INTRODUCCIÓN La Encefalitis de Hashimoto (EH) es una encefalopatía de naturaleza autoinmune, con buena respuesta al tratamiento con corticoides, títulos séricos elevados de anticuerpos antitiroideos y de curso subagudo con recaídas-remisiones. Es una enfermedad poco frecuente, con una presentación clínica variable y fisiopatología aún desconocida. PRESENTACIÓN DEL CASO: Paciente femenina de 76 años con antecedentes de hipotiroidismo primario. Ingresó con un síndrome confusional agudo. Al examen físico vigil, Glasgow 13/15, subfebril (37.8°C) desorientada temporoespacialmente, ecolalia, pupilas isocóricas y reactivas, sin focalidad neurológica. Signos meníngeos negativos. Laboratorio: Hipocalcemia leve (7.8mg/dl), hipopotasemia (K 3,2 mmol/l), PCR 221.9 mg/L. Test rápido para VIH negativo. TC de encéfalo sin alteraciones. Punción lumbar líquido cristal de roca, proteínas 1 g/l, glucosa 0.67 g/l, láctico 1.3, leucocitos 77 células/microL (100% mononucleares). Se interpretó inicialmente como Encefalitis de etiología viral y se le indicó aciclovir. Presentó sensorio alternante, excitación psicomotriz y convulsión tónica clónica generalizada. Debido a deterioro súbito del sensorio, se realizó intubación orotraqueal y se trasladó a Unidad de Terapia Intensiva (UTI). Permaneció bajo asistencia mecánica ventilatoria y con vasopresores. Laboratorio: VDRL, p24 y anticuerpos HIV negativos, TSH 27,82, T4 0,41. PCR de LCR: Virus herpes simple 1 y 2, citomegalovirus y JC negativos. Hemocultivos negativos. Ante sospecha clínica de Encefalitis de Hashimoto, se solicitaron anticuerpos antitiroideo peroxidasa (aTPO), antitiroglobulina (aTG) y Anticuerpos Anti-Receptor de TSH (TRABS), que resultaron positivos. Recibió tratamiento con levotiroxina endovenosa e hidrocortisona. Normaliza valores. Por fallo en el weaning, se realizó traqueostomía. Luego de 21 días de internación en Terapia Intensiva pasó a clínica con posterior alta hospitalaria. Discusión: La EH se puede considerar como diagnóstico, solo después de descartar otras causas. En el caso expuesto se llegó al diagnóstico luego de descartar otras causas posibles, con anticuerpos antitiroideos positivos en altas concentraciones y respuesta al tratamiento con corticoides. Conclusión: Se destaca la necesidad de ampliar el conocimiento de esta patología con el fin de disminuir el subdiagnóstico y promover un inicio precoz del tratamiento, mejorando así su progresión y calidad de vida de los pacientes.

INTRODUCTION: Hashimoto's Encephalitis (HE) is an autoimmune encephalopathy, with a good response to treatment with corticosteroids, high serum titers of antithyroid antibodies and a subacute course with relapses-remissions. It is a rare disease, with a variable clinical presentation and still unknown pathophysiology. CASE REPORT: A 76-year-old female patient with a history of primary hypothyroidism. She was admitted with acute confusional syndrome. On physical examination, she was awake, she was Glasgow 13/15, she was subfebrile (37.8°C), disoriented temporally, echolalia, isochoric and reactive pupils, without neurological focality. Negative meningeal signs. Laboratory: Mild hypocalcemia (7.8 mg/dl), hypokalemia (K 3.2 mmol/l), CRP 221.9 mg/L. Rapid test for HIV negative. Brain CT without alterations. Lumbar puncture rock crystal liquid, proteins 1 g/l, glucose 0.67 g/l, lactic acid 1.3, leukocytes 77 cells/microL (100% mononuclear). It was initially interpreted as Encephalitis of viral etiology and acyclovir was prescribed. He presented alternating sensory, psychomotor excitement, and generalized tonic-clonic seizure. Due to sudden deterioration of the sensorium, orotracheal intubation was performed and he was transferred to the Intensive Care Unit. He remained under mechanical ventilatory assistance and with vasopressors. Laboratory: VDRL, p24 and HIV antibodies negative, TSH 27.82, T4 0.41. CSF PCR: Herpes simplex virus 1 and 2, cytomegalovirus and JC negative. Negative blood cultures. Due to clinical suspicion of Hashimoto's Encephalitis, anti-thyroid peroxidase (aTPO), anti-thyroglobulin (aTG) antibodies and Anti-TSH Receptor Antibodies (TRABS) were requested, which were positive. She was treated with intravenous levothyroxine and hydrocortisone. Normalized values. Due to weaning failure, a tracheostomy was performed. After 21 days of hospitalization in the Intensive Care Unit, she was admitted to the clinic and subsequently discharged from the hospital. Discussion: HD can be considered as a diagnosis, only after ruling out other causes. In the case presented, the diagnosis was made after ruling out other possible causes, with positive antithyroid antibodies in high concentrations and response to treatment with corticosteroids. Conclusion: The need to expand knowledge of this pathology is highlighted in order to reduce underdiagnosis and promote early initiation of treatment, thus improving its progression and quality of life of patients.

Cardiovascular risk in hashimoto thyroiditis patients according to their thyroid state: a cross-sectional study / Riesgo cardiovascular en pacientes con tiroiditis hashimoto según su estada tiroidea: un estudio transversal

Objetivo: investigar la asociación entre los factores de riesgo cardiovascular y el nivel de riesgo cardiovascular con el estado tiroideo de pacientes con Tiroiditis de Hashimoto. Métodos y técnicas: treinta y ocho adultos con tiroiditis de Hashimoto participaron en este estudio descriptivo transversal. Los factores de riesgo cardiovascular considerados incluyeron edad, sexo, presión arterial, índice de masa corporal, glucemia, perfil lipídico, comorbilidades cardiovasculares, Proteína C Reactiva y Eritrosedimentación. Se utilizó la calculadora de riesgo cardiovascular de Framingham. La muestra se clasificó según el estado tiroideo en eutiroidismo (n = 15), hipotiroidismo clínico (n = 9) e hipotiroidismo subclínico (n = 13) e incluyó la presencia de anticuerpos antitiroideos. Se utilizó la prueba exacta de Fisher para determinar la asociación entre las variables estudiadas. Resultados: el 100 % de la muestra fue de sexo femenino; con una edad media entre 39-59 años. La categoría con bajo riesgo fue la mayor muestra (n = 30), equivalente al 78,9 %; riesgo moderado, no se obtuvo ningún paciente; alto riesgo (n = 8) constituyó el 21,1 %. Se encontró significancia estadística entre la edad y el nivel de riesgo cardiovascular en pacientes con hipotiroidismo clínico (p < 1), score in patients with clinical hypothyroidism was found (p < 1), IC 95 %. El nivel de glucosa en el hipotiroidismo subclínico y el hipotiroidismo clínico tuvo significancia estadística. Del mismo modo, se demostró que la presencia de antitiroglobulina (antiTg) está estrechamente relacionada con el nivel de riesgo cardiovascular en pacientes con hipotiroidismo subclínico. Conclusión: la edad, la glucemia, los anti-Tg, el antecedente de diabetes mellitus, la dislipemia y haber padecido algún accidente cerebrovascular se ha relacionado con un aumento del riesgo de desarrollar un evento cardiovascular hasta en 10 años en función de su perfil tiroideo. No se encontró evidencia de una relación directa entre la puntuación de riesgo cardiovascular y el estado de la tiroides en los participantes de este estudio.

Aims: To investigate the association between cardiovascular risk factors and cardiovascular risk score with the thyroid status of patients with Hashimoto's Thyroiditis. Methods: Thirty-eight consenting adults with Hashimoto's Thyroiditis participated in this cross-sectional study. The cardiovascular risk factors considered included age, sex, blood pressure, body mass index, fast blood glucose, lipid profile, cardiovascular comorbidities, C reactive protein, and erythrocyte sedimentation rate. The Framingham CV risk score was performed. The sample was classified into euthyroid (n = 15), clinical hypothyroidism (n = 9), and subclinical hypothyroidism (n = 13), and included the presence of antithyroid antibodies. Fisher's exact test was used to determine the association between the variables studied. Results: 100% of the sample were women; a mean age between 39-59 years old. The category with low risk was the largest (n = 30), equivalent to 78.9%; moderate risk, no patient was obtained; high risk (n = 8) constituted 21.1%. Statistical significance between age and CV risk score in patients with clinical hypothyroidism was found (p < 1), 95% CI. The glucose level in the subclinical hypothyroidism and clinical hypothyroidism had statistical significance. The presence of anti-Thyroglobulin (antiTg) was shown to be closely related to the level of CV risk in patients with subclinical hypothyroidism. Conclusion: Age, glycemia, anti-Tg, history of DM, dyslipidemia, or cerebrovascular accidents have been linked to raising the risk of developing CVD in up to 10 years depending on their thyroid profile. No evidence of a direct relationship between CV risk score and thyroid state was found in the participants of this study.

Asociación entre tiroiditis linfocítica crónica y carcinoma papilar de tiroides: Revisión sistemática y metaanálisis de estudios en especímenes quirúrgicos / Association between chronic lymphocityc thyroiditis and papillary thyroid carcinoma: Systematic review and meta-analysis of studieson surgical specimens

Introducción. Existen resultados inconsistentes con relación al planteamiento de la hipótesis que sugiere una mayor probabilidad de documentar un carcinoma papilar de tiroides en especímenes quirúrgicos con cambios compatibles con tiroiditis linfocítica crónica. En los metaanálisis existentes se han incluido estudios no comparables metodológicamente y no se proponen claras fuentes de sesgo, justificación para la realización del presente metaanálisis. Métodos. Se realizó una búsqueda bibliográfica en Pubmed y Embase. Fueron obtenidos estudios retrospectivos donde se comparaba la prevalencia de carcinoma papilar de tiroides en especímenes con y sin cambios por tiroiditis linfocítica crónica. La evidencia recolectada fue sintetizada estadísticamente. Resultados. Un total de 22 artículos fueron incluidos. La población estuvo conformada por 63.548 especímenes. El OR combinado fue 1,81 (IC95%: 1,51-2,21). Hubo heterogeneidad entre la distribución de las razones de oportunidad entre los estudios (I2= 91 %; p>0,00001). La forma del gráfico en embudo de los estudios incluidos en el análisis parece estar simétrica, lo que indica la ausencia del sesgo atribuible a los estudios pequeños. Conclusiones. La literatura actual sugiere que existe un mayor riesgo de documentar un carcinoma papilar de tiroides en especímenes quirúrgicos en los que se observan cambios compatibles con tiroiditis linfocítica crónica; sin embargo, existen fuentes de sesgo que no será posible controlar en estudios retrospectivos, por lo que recomendamos estudiar la hipótesis que sugiere una mayor probabilidad de diagnosticar un carcinoma papilar de tiroides en especímenes con cambios compatibles con tiroiditis linfocítica crónica mediante metodologías prospectivas

Introduction. Inconsistent results exist in the literature regarding the hypothesis statement suggesting an increased likelihood of documenting papillary thyroid carcinoma (PTC) in surgical specimens with changes compatible with chronic lymphocytic thyroiditis. Existing meta-analyses have included studies that are not methodologically comparable and do not propose clear sources of bias, thus, this is justification for the present meta-analysis. Methods. A literature search in Pubmed and Embase was performed from January 1, 1950 to December 31, 2020. Retrospective studies comparing the prevalence of papillary thyroid carcinoma in specimens with and without chronic lymphocytic thyroiditis changes were obtained. The collected evidence was statistically analyzed. Results. A total of 22 articles were included. The study population consisted of 63,548 surgical specimens. The pooled OR, based on the studies, was 1.81 (95% CI: 1.51-2.21). There was heterogeneity between the distribution of prevalence ratios and opportunity ratios across studies (I²= 91%; p>0.00001). The funnel plot shape of the studies included in the analysis appears to be symmetrical, indicating the absence of bias attributable to small studies. Conclusions. The current literature suggests that there is an increased risk of documenting papillary thyroid carcinoma in surgical specimens in which chronic lymphocytic thyroiditis-compatible changes are observed; however, there are sources of bias that will not be possible to control for in retrospective studies, so we recommend studying the hypothesis suggesting an increased likelihood of diagnosing PTC in specimens with chronic lymphocytic thyroiditis-compatible changes using prospective methodologies

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

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.

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.

Linfoma del tejido linfoide asociado a las mucosas en paciente con tiroiditis de Hashimoto / Mucosa-associated lymphoid tissue lymphoma in a patient with Hashimoto's thyroiditis

RESUMEN El linfoma del tejido linfoide asociado a las mucosas es una variedad poco común y recientemente descubierta del linfoma no Hodgkin. Suele presentarse en la sexta década de la vida, con un predominio del sexo femenino y en sitios como el tracto digestivo, pulmón, riñón, hígado, piel, y solo en el 2 % de los casos, en la glándula tiroides, donde en muchas ocasiones se asocia a la tiroiditis autoinmune de Hashimoto. Su evolución es favorable cuando se diagnostica en estadios iniciales de la enfermedad. Se presenta una paciente de 22 años, con una historia de trastornos endocrinos, perceptibles desde la adolescencia, a quien se le diagnosticó una tiroiditis de Hashimoto, sobre la que subyacía un linfoma del tejido linfoide asociado a las mucosas, y que evolucionó satisfactoriamente luego del tratamiento quirúrgico.

ABSTRACT Mucosa-associated lymphoid tissue lymphoma is a rare and recently discovered variant of non-Hodgkin's lymphoma. It usually occurs in the sixth decade of life, with a predominance of females and may be observed in sites such as the digestive tract, lung, kidney, liver, skin, and only in 2% of cases, in the thyroid gland, where in many occasions it is associated with Hashimoto's autoimmune thyroiditis. Its evolution is favorable when it is diagnosed in the initial stages of the disease. We present a 22-year-old female patient with a history of perceptible endocrine disorders since adolescence, who was diagnosed with Hashimoto's thyroiditis, underlying mucosa-associated lymphoid tissue lymphoma, and who evolved satisfactorily after surgical treatment.

Encefalopatía asociada a enfermedad tiroidea autoinmune: reporte de caso y revisión de la literatura / Encephalopathy associated with autoimmune thyroid disease: Case report and literature review

RESUMEN INTRODUCCIÓN: La encefalopatía asociada a enfermedad tiroidea autoinmune se caracteriza por un inicio insidioso de síntomas neuropsiquiátricos que incluyen alteración de la función cognoscitiva, cambios del comportamiento, crisis convulsivas y trastornos del movimiento. REPORTE DE CASO: Hombre de 69 años con antecedente de hipotiroidismo primario que consultó por dos semanas de alteración de la memoria, confusión y trastorno del lenguaje, asociados a hiperreflexia y temblor generalizado. Los estudios mostraron nivel de hormona tiroidea y títulos de anticuerpos antitiroideos elevados, líquido cefalorraquídeo con aumento de proteínas y anticuerpos antineuronales negativos, neuroimagen normal y electroencefalograma con alteraciónes inespecíficas. Posterior a descartar otras etiologías, se hizo el diagnóstico de encefalopatía asociada a enfermedad tiroidea autoinmune y se inició manejo con esteroides, con los que presentó una mejoría clínica significativa. DISCUSIÓN: La encefalopatía asociada a enfermedad tiroidea autoinmune es un trastorno complejo que requiere un diagnóstico oportuno y rápido. En todos los pacientes con síntomas neuropsiquiátricos agudos o subagudos, y sin causa clara, es pertinente solicitar un perfil de anticuerpos antitiroideos independiente de la función tiroidea basal.

ABSTRACT INTRODUCTION: Thyroid autoimmune disease-associated encephalopathy is characterized by an insidious onset of neuropsychiatric symptoms which may include impaired cognitive function, behavioral changes, seizures, and movement disorders. CASE REPORT: A 69-year-old man with a history of primary hypothyroidism who consulted for two weeks of memory impairment, confusion, and language disorder, associated with hyperreflexia and generalized tremor. Studies showed elevated thyroid hormone levels and antithyroid antibody titers, cerebrospinal fluid with increased protein and negative antineuronal antibodies, normal neuroimaging, and electroencephalogram with nonspecific abnormalities. After ruling out other etiologies, a diagnosis of encephalopathy associated with autoimmune thyroid disease was made and management with steroids was started, with which he presented significant clinical improvement. DISCUSSION: Encephalopathy associated with autoimmune thyroid disease is a complex disorder that requires timely and rapid diagnosis. In all patients with acute or subacute neuropsychiatric symptoms, and without a clear cause, it is pertinent to request an antithyroid antibody profile independent of baseline thyroid function

Factores clínicos y ambientales asociados a la tiroiditis de Hashimoto / Clinical and environmental factors associated with Hashimoto's thyroiditis

Introducción: La tiroiditis de Hashimoto es una enfermedad tiroidea autoinmune poligénica y multifactorial resultante de una interacción compleja de factores genéticos y ambientales. Objetivo: Determinar la posible asociación de los factores clínicos y ambientales con los niveles de anticuerpos antitiroideos y las pruebas de función tiroidea en la tiroiditis de Hashimoto. Métodos: Estudio observacional, descriptivo y transversal con 120 personas con diagnóstico de tiroiditis de Hashimoto. Variables estudiadas: edad, sexo, color de la piel, estado nutricional, paridad, hábito de fumar, consumo de alcohol, preparados estrogénicos, antecedentes familiares de enfermedad autoinmune tiroidea y personales de otras enfermedades autoinmunes. Se realizaron determinaciones de anticuerpos AbTPO, TSH, T3 y T4. Resultados: Predominio del sexo femenino (92,5 por ciento), de pacientes de piel blanca (50,8 por ciento) y con sobrepeso corporal (40 por ciento). El 73 por ciento no consumían preparados estrogénicos. El 20 por ciento tenían antecedentes familiares de enfermedad tiroidea y personales de diabetes mellitus tipo 1 (7,5 por ciento). La media del anticuerpo en pacientes con antecedentes de infecciones virales fue superior a los que no tuvieron este antecedente (732,6 vs. 624,6). El resto de las variables no mostraron diferencias entre las medias del anticuerpo. Ninguno de los factores estudiados mostró asociación con el estado de la función tiroidea. (p>0,05). Conclusiones: No existió asociación entre los factores clínicos y ambientales en relación a los niveles de Ac TPO y el estado de la función tiroidea, con predominio del hipotiroidismo manifiesto al diagnóstico de la TH(AU)

Introduction: Hashimoto's thyroiditis is a polygenic and multifactorial autoimmune thyroid disease, resulting from a complex interaction of genetic and environmental factors. Objective: To determine the possible association of clinical and environmental factors with antithyroid antibody levels and thyroid function tests in HT. Methods: An observational, descriptive, cross-sectional study was carried out with 120 subjects diagnosed with Hashimoto's thyroiditis. We studied variables such as age, sex, skin color, nutritional status, parity, smoking, alcohol consumption, estrogen preparations, family history of autoimmune thyroid disease and personal history of other autoimmune diseases. Additionally, AbTPO, TSH, T3 and T4 antibody determinations were made. Results: Predominance of the female sex (92.5 percent), white skin (50.8 percent) and body overweight (40 percent). 73 percent did not consume estrogenic preparations. Twenty percent had family history of thyroid disease and personal history of type 1 diabetes mellitus (7.5 percent). The mean antibody in patients with history of viral infections was higher than those without this history (732.6 vs. 624.6). The rest of the variables did not show differences between the means of the antibody. None of the factors studied showed association with the state of thyroid function. (p > 0.05). Conclusions: There was no association between clinical and environmental factors in relation to Ac TPO levels and the state of thyroid function, with a predominance of overt hypothyroidism at diagnosis of HT(AU)

Perfil clínico y epidemiológico de los pacientes con diagnóstico de neuromielitis óptica / Clinical and epidemiological profile of patients with neuromyelitis optica diagnosis

INTRODUCCIÓN. El trastorno del espectro de neuromielitis óptica, enfermedad inflamatoria, desmielinizante, afecta al sistema nervioso central, frecuente en poblaciones no caucásicas como la ecuatoriana. El retraso en su diagnóstico y tratamiento provoca discapacidad que se puede prevenir. OBJETIVO. Determinar el perfil clínico y epidemiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 45 Historias Clínicas y una muestra de 41 de pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica atendidos en la Unidad de Neurología del Hospital de Especialida-des Carlos Andrade Marín, período enero 2005 a diciembre 2019. Se realizó análisis univarial. Se aplicó el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 25. RESULTADOS. El 76,0% (31; 41) fueron mujeres. Datos promedios: edad 48,9 años; diagnóstico definitivo demoró 4,12 años, desde el inicio de los síntomas; tiempo de diagnóstico fue 3,17 años; 3,7 brotes en total; el 87,8% (36; 41) con un fenotipo recurrente. La media de duración de la enfermedad fue de 6,8 años. En el 70,7% (29; 41), se identificaron anticuerpos anti-AQP4 en suero mediante inmunofluorescencia directa, el 51,2% requirieron para la marcha apoyo uni o bilateral. El 43,9% (18; 41) debutó con neuritis óptica; el 31,7% (13; 41) presentaron mielitis como primer síntoma y el 24,4% (10; 41) la combinación de neuritis óptica y mielitis fueron los síntomas iniciales. CONCLUSIÓN. Se determinó el perfil clínico y epi-demiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. Existió demora en el diagnóstico definitivo de los pacientes desde el inicio de los síntomas, lo que se tradujo en un aumento de la discapacidad.

INTRODUCTION. Neuromyelitis optica spectrum disorder, an inflammatory, demyelinating disease, affects the central nervous system, common in non-Caucasian popu-lations such as Ecuadorians. The delay in its diagnosis and treatment causes disabi-lity that can be prevented. OBJECTIVE. To determine the clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 45 Medical Records and a sample of 41 patients with a diagnosis of neuromyelitis optica spectrum disor-der seen at the Neurology Unit of the Carlos Andrade Marín Specialties Hospital, period from January 2005 to December 2019. Univariate analysis was performed. The statistical program International Business Machines Statistical Package for the Social Sciences, version 25 was used. RESULTS. 76,0% (31; 41) were women. Average data: age 48,9 years; definitive diagnosis took 4,12 years from the onset of symptoms; time to diagnosis was 3,17 years; 3,7 outbreaks in total; 87,8% (36; 41) with a recurrent phenotype. The average disease duration was 6,8 years. In 70,7% (29; 41), anti-AQP4 antibodies were identified in serum by direct immunofluorescence, 51,2% required uni- or bilateral su-pport for walking. Optic neuritis started in 43,9% (18; 41); 31,7% (13; 41) had myelitis as the first symptom and 24,4% (10; 41) the combination of optic neuritis and myelitis were the initial symptoms. CONCLUSION. The clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder was determined. There was delay in the conclusive diagnosis of patients from the beginning of symptoms, which resulted in increased disability.

Alteraciones tiroideas en la infancia y en la adolescencia. Parte 2: hipotiroidismo / Thyroid disorders in childhood and adolescence. Part 2: Hypothyroidism

El hipotiroidismo es la disfunción tiroidea más frecuente, resultante de una disminución de la actividad biológica de las hormonas tiroideas en los tejidos. El objetivo es realizar una revisión y actualización del hipotiroidismo adquirido en la infancia y adolescencia con énfasis en el hipotiroidismo primario. La causa más común es la tiroiditis de Hashimoto o tiroiditis linfocitaria crónica. La característica distintiva es el impacto profundo en el crecimiento esquelético, maduración y desarrollo puberal, con potencial repercusión en la talla adulta. Los signos y síntomas del hipotiroidismo adquirido son similares a los adultos y, en general, no se asocia con compromiso del desarrollo neuromadurativo.La presunción clínica se confirma con niveles elevados de tirotrofina y disminuidos de tiroxina libre. Las metas del tratamiento incluyen lograr adecuado crecimiento, maduración sexual, desarrollo neuromadurativo y cognitivo óptimo. En la mayoría de los pacientes, el tratamiento de reemplazo revierte los signos y síntomas.

Hypothyroidism is the most frequent thyroid dysfunction. It is the consequence of a decrease in the biological activity of thyroid hormones in target tissues. The aim of this paper is to review and update acquired hypothyroidism in childhood and adolescence with emphasis on primary hypothyroidism due to its greater frequency. Hashimoto's thyroiditis, also known as chronic lymphocytic thyroiditis, is the most common cause of primary acquired hypothyroidism. The distinctive feature is the profound impact on skeletal growth, maturation, and pubertal development, with potential implications on adult height. Signs and symptoms of acquired hypothyroidism are similar to those reported in adults and are generally not associated with neurodevelopmental impairment. Biochemi confirmation of primary hypothyroidism requires the finding of elevated thyrophine and decreased free thyroxine levels. Treatment goals are to achieve normal growth and maturation as well as cognitive development. In most of the patients, replacement treatment reverses symptoms and signs of hypothyroidism and may decrease goiter size.

Prevalence of Hashimoto's thyroiditis in psoriasis patients

SUMMARY OBJECTIVE: To study the prevalence of Hashimoto's Thyroiditis in a sample of psoriasis patients. METHODS: Cross-sectional observational study of 120 individuals (60 with psoriasis and 60 control subjects) paired by gender and age for thyroid function (thyroid stimulating hormone, free T4), antithyroperoxidase and antithyroglobulin tests and physical examination. Psoriasis Area and Severity Index and Nail Psoriasis Severity Index were evaluated simultaneously. Epidemiological, clinical and treatment data was collected from medical records. Patients with documented hypothyroidism and/or goiter associated with positive antithyroperoxidase antibody were considered to have Hashimoto's Thyroiditis. RESULTS: The prevalence of Hashimoto's Thyroiditis in the group with psoriasis was 21.6%; in the control group, it was 4/60 (6.6%) with p=0.03 (OR=3.8; 95%CI 1.18-12.6). In the group of patients with psoriasis, Hashimoto's Thyroiditis was more common in women (p=0.002) and less common in those who had polyarticular arthropathic psoriasis (p=0.05) and plaque psoriasis (p=0.005). A logistic regression showed that the only independent variable associated with Hashimoto's thyroiditis was plaque psoriasis. CONCLUSIONS: There is a high prevalence of Hashimoto's Thyroiditis in psoriatic patients, especially in women. Hashimoto's Thyroiditis is less common in patients with the plaque form of psoriasis.

Decreased serum levels of IL-27and IL-35 in patients with Graves disease

ABSTRACT Objectives Graves' disease (GD) is an autoimmune disease causing the overproduction of the thyroid hormone from thyroid gland. This disease is mainly the result of the production of antibodies against TSH receptors. Cytokines play an important role in orchestrating the pathophysiology in autoimmune thyroid disease. The regulatory role of IL-12 on TH1 cells has been proven. IL-27 and IL-35, members of IL-12 cytokine family, are two cytokines that have been newly discovered. IL-35 has been identified as a novel immunosuppressive and anti-inflammatory cytokine while IL-27 has both inflammatory and anti-inflammatory functions. The objective of the current study was to examine the changes in the serum level of the foregoing cytokines in GD patients in comparison to healthy controls. Materials and methods In this study, serum levels of IL-27 and IL-35 were determined by an ELISA method; anti TPO and anti Tg were measured by an RIA method in 40 new cases of Graves's disease. The findings were compared with 40 healthy controls. Results The results showed a significant difference between IL-27 and IL-35 regarding their serum levels with P values of 0.0001 and 0.024, respectively; anti TPO and anti Tg levels of the cases were also significantly different from controls (p < 0.001). Conclusion The reduction in the serum levels of IL-27 and IL-35 in GD patients compared to normal subjects suggests the possible anti-inflammatory role of these cytokines in GD.

Autoimmune encephalitis (AIE)

The Guidelines Project, an initiative of the Brazilian Medical Association, aims to combine information from the medical field in order to standardize producers to assist the reasoning and decision-making of doctors. The information provided through this project must be assessed and criticized by the physician responsible for the conduct that will be adopted, depending on the conditions and the clinical status of each patient.

Variables predictivas de talla baja adulta en pacientes con hipotiroidismo adquirido grave de origen autoinmune / Predictive outcome measures of adult short stature in patients with severe acquired autoimmune hypothyroidism

El hipotiroidismo por tiroiditis de Hashimoto es la causa más frecuente de disfunción tiroidea en niños.Nuestro objetivo fue analizar el impacto en la talla final según la talla y el estadio puberal al momento del diagnóstico en menores de 18 años con hipotiroidismo grave de origen autoinmune. De los 79 pacientes, el 78,5 % fueron mujeres. Los que presentaron bocio (el 56 %) mostraron mejor talla en el diagnóstico que los que no lo tenían (puntaje de desvío estándar de media de talla: 0,2 vs. -2,42; p < 0,0001). Cinco niñas (el 6,3 %) presentaron pubertad precoz. De los pacientes con talla final (n: 33), dentro de los que presentaron talla baja al momento del diagnóstico, los púberes tuvieron una talla final significativamente menor que los prepúberes (puntaje de desvío estándar media: -2,82 vs. -1,52; p = 0,0311).El diagnóstico tardío de hipotiroidismo grave en pediatría tiene un impacto negativo en la talla final, especialmente, en los pacientes puberales al momento del diagnóstico.

Hypothyroidism caused by Hashimoto's thyroiditis is the most common reason for thyroid dysfunction in children. Our objective was to analyze its impact on final stature in relation to height and pubertal stage at the time of diagnosis in children younger than 18 years with severe autoimmune hypothyroidism. Out of 79 patients, 78.5 % were girls. Those with goiter (56 %) had a better height at diagnosis than those without goiter (mean standard deviation score for height: 0.2 versus −2.42; p < 0.0001). Five girls (6.3 %) had precocious puberty. When considering the final stature of patients (n: 33), among those with short stature at the time of diagnosis, pubertal children had a significantly shorter final stature than prepubertal children (mean standard deviation score for height: −2.82 versus −1.52; p = 0.0311). The late diagnosis of severe hypothyroidism in pediatrics has a negative impact on final stature, especially in those who were pubertal patients at the time of diagnosis

Síndrome autoinmune tirogástrico (SAT): serie de casos / Thyrogastric syndrome: Case Series

Resumen Un porcentaje importante de pacientes con gastritis crónica atrófica corporal autoinmune, o gastritis tipo A, desarrollan enfermedad autoinmune tiroidea (enfermedad de Graves o de Hashimoto) y viceversa, situación conocida como síndrome autoinmune tirogástrico (SAT), pero no se conoce su prevalencia, por lo que puede pasarse sin el diagnóstico completo. El desarrollo de la gastritis atrófica limita la absorción de la vitamina B12, lo que lleva a alteraciones hematológicas, neurológicas y metabólicas, por tanto, es importante realizar las pruebas necesarias para su diagnóstico y seguir de cerca la evolución de los pacientes. La detección serológica de los autoanticuerpos contra la glándula tiroides y el cuerpo gástrico muestran la etiología autoinmune y un estado inflamatorio con daño tisular. Todo paciente con enfermedad autoinmune debe ser valorado para descartar la presencia de otras patologías de etiología inmunológica.

Abstract A significant percentage of patients with chronic autoimmune atrophic body gastritis (type A gastritis) develop thyroid autoimmune disease (Graves' disease or Hashimoto's disease) and vice versa. This situation is known as thyrogastric syndrome. Its prevalence is unknown, due to incomplete diagnoses. Since the development of atrophic gastritis limits the absorption of vitamin B12 leading to hematological, neurological and metabolic alterations, it is important to perform necessary diagnostic tests and to closely monitor the evolution of patients. Serological detection of autoantibodies against the thyroid gland and the gastric body show the autoimmune etiology and an inflammatory state with tissue damage. Every patient with autoimmune disease should be evaluated to rule out the presence of other pathologies of immunological etiology.

Human herpesvirus 6A active infection in patients with autoimmune Hashimoto's thyroiditis

ABSTRACT Background: Hypothyroidism due to Hashimoto's thyroiditis (HT) is the commonest autoimmune endocrine illness in which antibodies against thyroid organ result in inflammation. The disease has a complex etiology that involves genetic and environmental influences. Viral infections may be involved in triggering of the disease as their molecular mimicry enhance autoimmune responses. Human herpesvirus-6 (HHV-6) is recognized for its contribution to some autoimmune diseases. Objective: In the current study, the prevalence of HHV-6 active infection in patients with HT and with non-autoimmune thyroid disorders were compared with patients with euthyroidism. In addition, a correlation between presence of HHV-6 infections and HT was investigated. Methods: A total of 151 patients with clinically and laboratory confirmed HT, 59 patients with non-autoimmune thyroid disorders, and 32 patients with normal thyroid function were included in the study. For further confirmation of HT disease, all the precipitants were tested for anti-thyroid peroxidase (TPO), and anti-thyroglobulin (TG) antibodies. For detection of both HHV-6 types A and B, nested PCR and restriction enzyme digestion were used. HHV-6 DNA positive samples were further investigated by DNA sequencing analysis. Results: HHV-6A DNA was found in serum sample of 57 out of 151 patients (38%) with HT, which was significantly more often than in patients with non-autoimmune thyroid disorders (p = 0.001). However, HHV-6 DNA was not detected in serum samples of euthyroid subjects. Conclusions: The results support a possible role for active HHV-6A infection, demonstrated by the presence of HHV-6 DNA in sera, in the development of HT.

Encefalitis autoinmunes: Receptor anti-NMDA y nuevos inmunofenotipos / Autoimmune encephalitis. Anti-NMDA receptor and new immunophenotypes

Las encefalitis autoinmunes (EA) se definen como síndromes neurológicos de instalación subaguda de compromiso de conciencia, alteración de la memoria de trabajo y trastornos psiquiátricos frecuentemente asociados a movimientos anormales y crisis epilépticas y que se producen por la acción de anticuerpos anti neuronales específicos que se fijan a receptores de neurotransmisores o proteínas de membrana. El anticuerpo anti NMDAR es el que origina la mayoría de los casos de EA en niños y adultos jóvenes, seguido por el anticuerpo anti LGI1 de presentación en el adulto. Las EA han aumentado en la última década, en la que se ha descrito un gran número de nuevos anticuerpos que producen en su mayoría síndromes neurológicos que involucran al sistema nervioso central, con predominio de signología psiquiátrica, excepto en niños en los que predominan movimientos anormales, crisis epilépticas y compromiso de conciencia. Se asocian frecuentemente a tumores en el adulto pero en los niños esta asociación es más rara. Todas las EA responden a terapia inmunomoduladora aunque en diferente medida según el tipo de anticuerpo involucrado. Generalmente la evolución a la mejoría es lenta y puede completarse en meses o incluso en un año o más. En esta revisión se destaca los principales cuadros de EA relacionados con anticuerpos específicos mencionando también los inmunofenotipos descubiertos recientemente.

Autoimmune encephalitis (AE) is defined as neurological syndromes of subacute installation of compromise of consciousness, alteration of working memory and psychiatric disorders associated with abnormal movements and epileptic seizures and that are produced by the action of anti-neuronal antibodies. They bind to neurotransmitter receptors or membrane proteins. Antibody to NMDAR is the origin of the majority of cases of AD in children and young adults, followed by anti-LGI1 antibody for presentation in adults. The AE has increased in the last decade, with a large number of new agents described that produce mostly neurological syndromes that involve the central nervous system, with predominance of psychiatric signaling, except in children and the predominant abnormal movements, epileptic seizures and compromise of conscience. They are frequently associated with tumors in adults but in children this association is more infrecuent. All AEs respond to immunomodulatory therapy although in different measures depending on the type of antibody involved. In general, the evolution to improvement is slow and can be completed in months or even in one year or more. In this review, the main EA clinical pictures related to specific antibodies are highlighted, also mentioning recently discovered immunophenotypes.

Ocular surface evaluation in pediatric Hashimoto's thyroiditis without thyroid-associated ophthalmopathy / Avaliação da superfície ocular na tireoidite de Hashimoto pediátrica sem oftalmopatia associada à tireoide

ABSTRACT Purpose: We aimed to assess ocular surface characteristics in children with Hashimoto's thyroiditis without thyroid-associated ophthalmopathy and compare the results with those of healthy children. Methods: Twenty-two children with Hashimoto's thyroiditis (Group 1) and 20 healthy children without any ocular and/or systemic disorder (Group 2) were enrolled in the study. Ocular Surface Disease Index questionnaire, tear film osmolarity measurement (TearLab Osmolarity System, San Diego, CA, USA), Schirmer and tear film breakup time tests, meibography, and conjunctival brush cytology were performed and compared the results between the groups. Results: The study group included 19 girls and 3 boys in Group 1 and 12 girls and 8 boys in Group 2 (p=0.081). Thyroid-associated ophthalmopathy was not identified in any of the patients. Mean tear film osmolarity was 310.23 ± 11.98 mOsm/l in Group 1 and 313.60 ± 15.03 mOsm/l in Group 2 (p=0.424). Mean Schirmer test score was lower in Group 1 (14.91 ± 6.27) compared with Group 2 (23.60 ± 5.63) (p=0.001). Mean tear film breakup time was lower in Group 1 (11.78 ± 4.07) compared with Group 2 (15.1 ± 1.6) (p=0.013). Moreover, mean meibomian gland area loss was 25.01% ± 10.04% in Group 1 and 16.54% ± 6.02% in Group 2 (p=0.002). Conjunctival cytologic analysis in Group 1 revealed grade 0 changes in 6 patients (27.3%), grade 1 changes in 14 patients (63.6%), and grade 2 changes in 2 patients (9.1%), whereas 18 patients (90%) had grade 0 changes and 2 patients (10%) had grade 1 changes (p=0.001) in Group 2. Conclusions: The study demonstrates several ocular surface changes in children with Hashimoto's thyroiditis. These findings may indicate a tendency for dry eye in pediatric Hashimoto's thyroiditis patients without clinical evidence of thyroid-associated ophthalmopathy.

RESUMO Objetivo: Avaliar as características da superfície ocular em crianças com tireoidite de Hashimoto sem oftalmopatia associada à tireoide e comparar os resultados com aqueles de crianças saudáveis. Métodos: Vinte e duas crianças com tireoidite de Hashimoto (Grupo 1) e 20 crianças saudáveis sem qualquer distúrbio ocular e/ou sistêmico (Grupo 2) participaram do estudo. Utilizou-se o questionário Índice da Doença da Superfície Ocular, medida de osmolaridade do filme lacrimal (Tearlab Osmolarity System, San Diego, CA, EUA), teste de Schirmer e tempo de ruptura do filme lacrimal, meibografia e citologia do raspado conjuntival e comparação dos resultados entre os grupos. Resultados: O grupo de estudo incluiu 19 meninas e 3 meninos no Grupo 1 e 12 meninas e 8 meninos no Grupo 2 (p=0,081). A oftalmopatia associada à tireoide não foi identificada em nenhum dos pacientes. A média da osmolari­dade do filme lacrimal foi 310,23 ± 11,98 mOsm/l no Grupo 1 e 313,60 ± 15,03 mOsm/l no Grupo 2 (p=0,424). A média do escore do teste de Schirmer foi menor no Grupo 1 (14,91 ± 6,27) do que no Grupo 2 (23,60 ± 5,63) (p=0,001). A média do tempo de ruptura do filme lacrimal foi menor no Grupo 1 (11,78 ± 4,07) em comparação com o Grupo 2 (15,1 ± 1,6) (p=0,013). Além disso, a média da perda de área da glândula meibomiana foi 25,01% ± 10,04% no Grupo 1 e 16,54% ± 6,02% no Grupo 2 (p=0,002). A análise da citologia conjuntival no Grupo 1 revelou alterações de grau 0 em 6 pacientes (27,3%), alterações de grau 1 em 14 pacientes (63,6%) e alterações de grau 2 em 2 pacientes (9,1%), enquanto 18 pacientes (90%) com alteração de grau 0 e 2 pacientes (10%) com alteração de grau 1 (p=0,001) no Grupo 2. Conclusões: O estudo demonstra várias alterações da superfície ocular em crianças com tireoidite de Hashimoto. Esses achados podem indicar uma tendência para olho seco em pacientes pediátricos com tireoidite de Hashimoto, sem evidências clínicas de oftalmopatia associada à tireoide.