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Las demencias rápidamente progresivas (DRP) engloban un grupo muy heterogéneo de entidades caracterizadas por la aparición de trastornos cognitivos y conductuales relevantes que evolucionan en pocas semanas o meses. La triada clínica habitual consiste en pérdida de memoria, alteraciones psiquiátricas y crisis epilépticas. Aunque el síndrome de DRP engloba numerosos cuadros clínicos, es habitual pensar en un origen autoinmune paraneoplásico, infeccioso o asociado a enfermedades priónicas. Para su diagnóstico son necesarios estudios de imagen, el examen del líquido cefalorraquídeo y pruebas serológicas como la determinación de antígenos. Es importante establecer un diagnóstico diferencial precoz entre encefalopatías autoinmunes y demencias por trastornos neurodegenerativos, sobre todo en las de origen paraneoplásico, debido a que el tratamiento de la neoplasia es más efectivo en las fases tempranas de la enfermedad y puede evitar el daño neuronal irreversible. Presentamos dos casos de pacientes con deterioro cognitivo de pocos días de evolución debido a una encefalitis límbica no paraneoplásica y una encefalopatía de Hashimoto, causas poco habituales pero reversibles de demencia rápidamente progresiva. Rapidly progressive dementias (RPD) are a very heterogeneous group of diseases characterized by acute cognitive impairment and behavioral disorders in a few weeks or months. Clinically most of times consists of memory loss, psychiatric disorders and epilepsy. Although RPD can be part of multiple clinical conditions, most common causes include autoimmune diseases, infectious or prion diseases and rarely, as a manifestation of a paraneoplastic syndrome. Image studies, cerebral spinal fluid evaluation, and serologic tests such as antigen determination are the most useful in diagnosing a neurological paraneoplastic syndrome. It’s very interesting an early diagnosis because the treatment is more effective in the early stages of illnes and can prevent irreversible neuronal damage. We present two cases of patients with rapidly cognitive impairmen due to limbic encephalitis and Hashimoto encephalopathy, rare but reversible causes of dementia.

RESUMEN Introducción: Las bases fisiopatológicas del Síndrome de ovario poliquístico pueden predisponer a mayor riesgo de autoinmunidad a las mujeres que tienen esta condición y existen evidencias, aunque escasas, de mayor prevalencia de autoinmunidad tiroidea en ellas. Objetivos: Determinar la frecuencia de marcadores serológicos de autoinmunidad tiroidea en mujeres con Síndrome de ovario poliquístico e identificar si existe asociación entre la presencia de ellos y las concentraciones de progesterona y testosterona. Métodos: Se realizó un estudio en 50 mujeres con Síndrome de ovario poliquístico y 50 sin el síndrome. Se realizaron determinaciones de autoanticuerpos tiroideos (anti tiroglobulina (Anti-Tg) y anti peroxidasa (anti-TPO) a las mujeres de ambos grupos de estudio. Se realizaron determinaciones de hormonas (testosterona y progesterona) solo al grupo de estudio de mujeres con SOP. Se crearon categorías por anticuerpos: Positivo si los títulos fueron superior al rango de referencia y negativo dentro del rango. Se consideró respuesta autoinmune positiva, cuando al menos uno de los anticuerpos se encontró elevado. Para la asociación entre la presencia de autoinmunidad y las variables independientes se hicieron análisis bivariados mediante comparación de medias y test no paramétricos. Se consideró un nivel de significancia de α = 0,05. Resultados: En las mujeres con Síndrome de ovario poliquístico, 62 % mostraron anticuerpos positivos y 14 % en las sin el síndrome. En las mujeres sin síndrome, de las 7 mujeres con marcadores de autoinmunidad positivos, en 6 (85,7 %) el anti-Tg fue el que dio positivo. No hubo diferencias significativas en cuanto a la asociación con los niveles de testosterona y progesterona. Conclusiones: Las mujeres con Síndrome de ovario poliquístico tienen mayor frecuencia de desarrollar respuesta autoinmune tiroidea, independiente de los niveles de progesterona y testosterona.

Introduction: Hashimoto's encephalopathy is a condition associated with dysthyroidism and other autoimmune diseases. The pathophysiology is unclear and clinically the disease is characterized by neurological and psychiatric alterations in absence of brain infection. Antithyroid antibodies are high and imaging studies may be normal. Corticosteroid therapy is the treatment of choice. Case report: A 36-year-old woman with monoparesis, behavior and personality changes and hyperthyroidism. Lumbar puncture was done and infectious, metabolic and neoplastic encephalopathy causes were ruled out. MRI skull was requested, it reports compatible images with various brain abscesses of undetermined origin. Considering a probable Hashimoto encephalopathy, anti-peroxidase and thyroglobulin antibodies were requested, detecting high titers. Corticosteroids and immunoglobulin scheme were started with a poor response to the treatment. Conclusions: This case associated Hashimoto's encephalopathy with hyperthyroidism, a rare association reported in the literature. It was suspected a CNS primary vasculitis due to high values of complement; however, brain biopsy was not performed to define a more accurate diagnosis. [ABSTRACT FROM AUTHOR]

Las enfermedades tiroideas son un importante problema de salud que afecta a un gran porcentaje de la población. Las pruebas bioquímicas constituyen el pilar fundamental para su diagnóstico y seguimiento. El desarrollo de ensayos de segunda y tercera generación ha supuesto un gran avance en el diagnóstico de estas enfermedades. El texto incluye los ensayos utilizados para diagnosticar y tratar las diferentes enfermedades tiroideas, provee información bioquímica y clínica actualizada contenida en secciones referidas a la utilidad clínica de las determinaciones de hormonas tiroideas totales y libres, anticuerpos antitiroideos, tirotropina humana y tiroglobulina, de manera que pueda ofrecer, tanto al laboratorio como al médico, un panorama general de la utilidad y la capacidad actual de estas pruebas.Thyroid diseases are a significant health problem affecting a high percentage of the population. The biochemical tests are the fundamental pillar for diagnosis and follow-up. The development of second and third-generation assays has represented a great advance in diagnosing these diseases. The text covers the tests to diagnose and treat a number of thyroid diseases, and provides the reader with updated biochemical and clinical information in sections about the clinical usefulness of total and free thyroid hormone determinations, antithyroid antibodies, human thyrotropin and thyroglobulin. In this way, it can offer both the lab and the physician a general overview of the usefulness and the current capability of these tests.

Introduction: Hashimoto’s encephalopathy is a condition associated with dysthyroidism and other autoimmune diseases. The pathophysiology is unclear and clinically the disease is characterized by neurological and psychiatric alterations in absence of brain infection. Antithyroid antibodies are high and imaging studies may be normal. Corticosteroid therapy is the treatment of choice. Case report: A 36-year-old woman with monoparesis, behavior and personality changes and hyperthyroidism. Lumbar puncture was done and infectious, metabolic and neoplastic encephalopathy causes were ruled out. MRI skull was requested, it reports compatible images with various brain abscesses of undetermined origin. Considering a probable Hashimoto encephalopathy, anti-peroxidase and thyroglobulin antibodies were requested, detecting high titers. Corticosteroids and immunoglobulin scheme were started with a poor response to the treatment. Conclusions:This case associated Hashimoto’s encephalopathy with hyperthyroidism, a rare association reported in the literature. It was suspected a CNS primary vasculitis due to high values of complement, however, brain biopsy was not performed to define a more accurate diagnosis. [ABSTRACT FROM AUTHOR]

Background and Aim: Although sensorineural hearing loss may have different aetiologies, we focused on autoimmune hearing loss since it may be reversible with corticosteroid therapy; this entity is sometimes associated with systemic autoimmune diseases. Hashimoto's thyroiditis or chronic autoimmune thyroiditis shows antibodies and may be harmful to hearing thresholds regardless of hypothyroidism effect. To date this effect has not been sufficiently studied and never with extended high frequencies. The aim of this work is to study by age groups whether hearing thresholds in the human auditory range (128-20,000Hz) are affected in Hashimoto's disease., Materials and Methods: Two groups of 128 patients affected by Hashimoto's thyroiditis were included. First group: patients with pathological antithyroid antibodies who do not need L-thyroxine treatment. Second group: patients controlled with L-thyroxine substitutive treatment. Audiometric threshold study comparing between the groups of patients and a group of 209 controls was performed. All patients underwent complete otorhinolaryngological examination, antithyroid antibodies, TSH, T 3 and T 4 blood levels, tympanometry, conventional pure-tone audiometry, and extended-high-frequency audiometry., Results: All patients were women. Both groups showed worst audiometric thresholds than the control group; both study groups showed worse hearing than controls, this difference was statistically significant in all frequencies. In the 8-20kHz frequency range, this difference was more than 10dB, and in the 9-16kHz and 20kHz range this difference was more than 20dB. When separated by age groups, in younger subjects (20-29 years) these differences were found in all frequencies, except for conversational frequencies (500-4,000Hz); between 30 and 49 years the difference is statistically significant in all frequencies; and from 50 to 69 years differences are found, especially in the conversational frequencies., Conclusions: This first work studying the human auditory range in the chronic autoimmune thyroiditis or Hashimoto's thyroiditis confirms that hearing loss related to the autoimmune disorder predominates at extended-high-frequencies initially. But ends up involving all frequencies in pure-tone conventional audiometry, then it may be detected in routine clinical tests. These results support the role of extended-high-frequencies audiometry to diagnose subclinical hearing loss in patients affected by Hashimoto's thyroiditis., (Copyright © 2022 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Published by Elsevier España, S.L.U. All rights reserved.)