Encefalopatía de Hashimoto. Un diagnóstico por exclusión y revisión de la literatura a partir de un reporte de caso

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

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

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.

Hashimoto‘S Encephalopathy Case Reports Of Hashimoto‘S Encephalopathy In Tertiary Care Hospital

Hashimoto's encephalopathy (HE) is an uncommon syndrome and rare disease, associated with Hashimoto thyroiditis. It is characterized by a acute to chronic loss of cognitive dysfunction ,subacute onset of confusion with altered level of consciousness, stroke like episodes , neuropsychiatric manifestations ,seizures, and myoclonus. HE is believed to be an immune-mediated disorder rather than representing the direct effect of an altered thyroid state on the central nervous system. Hashimoto encephalopathy or Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) and a more general term, nonvasculitic autoimmune meningoencephalitis, are also used to describe this condition. Here we are reporting 3 cases of hashimoto encephalopathy in the tertiary care centre who presented with acute to chronic memory loss , neuropsychiatric disturbances, complex parital seizures visual hallucinations and myoclonus and responded to steroids. A negative microbiological screen of the CSF and serum along with raised CSF protein, elevated serum antithyroid antibodies, characteristic EEG and neuroimaging findings yielded the diagnosis

Psychotic Symptoms of Hashimoto's Encephalopathy: A Diagnostic Challenge

Hashimoto's encephalopathy (HE) is a rare and underdiagnosed neuropsychiatric illness. We present the case of a 17-year-old girl who was admitted to a tertiary-care psychiatric center with acute onset psychosis and fever. Her psychotic symptoms were characterized by persecutory and referential delusions, as well as tactile and visual hallucinations. Her acute behavioral disturbance warranted admission and treatment in a psychiatric setting (risperidone tablets, 3 mg/day). She had experienced an episode of fever with a unilateral visual acuity defect approximately 3 years before admission, which was resolved with treatment. Focused clinical examination revealed an enlarged thyroid, and baseline blood investigations, including thyroid function test results were normal. Abnormal laboratory investigations revealed elevated anti-thyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) levels (anti-TPO of 480 IU/mL; anti-TG of 287 IU/mL). Results of other investigations for infection, including cerebrospinal fluid examination, electroencephalography, and brain magnetic resonance imaging were normal. She was diagnosed with HE and was treated with intravenous corticosteroids (methylprednisolone up to 1 g/day; tapered and discontinued after a month). The patient achieved complete remission of psychotic symptoms and normalization of the anti-thyroid antibody titers. Currently, at the seventh month of follow-up, the patient is doing well. This case highlights the fact that in the absence of well-defined clinical diagnostic criteria, a high index of suspicion is required for early diagnosis of HE. Psychiatrists need to explore for organic etiologies when dealing with acute psychiatric symptoms in a younger age group.

Conocimientos actuales en encefalopatía de Hashimoto: revisión de la literatura / Current knowledge on Hashimoto's encephalopathy: a literature review

Resumen La encefalopatía de Hashimoto es una enfermedad rara. Se reporta una prevalencia de 2,1 por cada 100 000 habitantes. Entre las manifestaciones clínicas se describen confusión, disminución del estado de consciencia, déficit cognitivo, convulsiones, mioclonus, ataxia y/o déficits neurológicos focales. Debido a la amplia variedad de signos y síntomas, la sospecha clínica diagnóstica es fundamental. El diagnóstico se basa en tres pilares: la presencia de manifestaciones clínicas neurológicas, con la exclusión de otras causas de encefalopatía; presencia de anticuerpos antitiroideos aumentados; una mejoría clínica notable luego de la administración de inmunomoduladores. El tratamiento de la encefalopatía de Hashimoto tiene dos objetivos: controlar el proceso autoinmune y controlar las complicaciones de la enfermedad. Aunque en la mayoría de los casos la recuperación es completa con el tratamiento, el riesgo de recaídas puede oscilar entre 12,5 a 40% en seguimientos a dos años.

Abstract Hashimoto's encephalopathy is a rare disease, with a reported prevalence of 2.1 per 100 000. Clinical manifestations include confusion, decreased state of consciousness, cognitive deficit, seizures, myoclonus, ataxia, and focal neurological deficits. Due to the wide variety of signs and symptoms, clinical diagnostic suspicion is essential. Diagnosis is based on three pillars: the presence of neurological clinical manifestations after ruling out other causes of encephalopathy. 2) Presence of increased antithyroid antibodies. 3) Significant clinical improvement after the administration of immunomodulation. The treatment of Hashimoto's encephalopathy pursues two objectives: to control the autoimmune process and to control the complications of the disease. Although in most cases recovery is complete with treatment, the risk of relapse can range from 12.5 to 40% in follow-ups to 2 years.

Clinical characteristics and follow-up of Hashimoto encephalopathy in children / 临床儿科杂志

Objective To explore the clinical characteristics of Hashimoto encephalopathy (HE) in children. Methods The clinical data of 4 children with HE were analyzed retrospectively. Results All the 4 cases were school-age children and 3 of them were girls. They were physically healthy before onset. The main clinical manifestations were epileptic seizures in 3 cases, mental symptoms in 2 cases, disturbance of consciousness in 2 cases, stroke like symptoms in 1 case, decreased memory and decreased sleep in 1 case. Electroencephalogram showed that the background activity was decreased in 4 cases, and MRI showed abnormal in 3 cases. Serum thyroid antibodies were significantly increased in 4 cases, and were returned to normal in 2 cases when clinical symptoms disappeared, while they were significantly reduced, but not completely back to normal in another 2 cases. Only one out of 4 cases had abnormal thyroid function. All the 4 cases responded well to corticosteroid therapy. One of them relapsed after discontinuation of the therapy, but it was still effective when the therapy was reassumed. Conclusions HE is rare in children. When there are manifestations of unknown cause, such as epileptic seizures, mental disorders, cognitive impairment, movement disorders and disturbance of consciousness, HE should be considered. In addition, the increase of serum thyroid antibody should be considered as a necessary condition for diagnosis.

Thyroid Autoantibody Positive Anti-N-Methyl-D-Aspartate Receptor Encephalitis

BACKGROUND: Hashimoto's encephalopathy (HE) and anti N-methyl-D-aspartate receptor (NMDAR) encephalitis have clinical overlaps. CASE REPORT: A 70-year-old woman presented with acutely developed confusion, disorientations and psychosis. HE was suspected based on goiter, markedly elevated anti-thyroglobulin and anti-thyroid peroxidase antibody. She was placed on high dose steroid and intravenous immunoglobulins administration, which did not ameliorate her symptoms. After the antibodies to the NMDAR were identified, weekly 500 mg of rituximab with 4 cycles were started. The current followed up indicated a complete recovery. CONCLUSIONS: The possible associations between NMDAR antibody and autoimmune thyroid antibodies in anti-NMDAR encephalitis with positive thyroid autoantibodies remain unclear. However, a trend toward a higher incidence of NMDAR antibody in patients with autoimmune thyroid antibodies than without has been observed. Cases of encephalitis with only NMDAR antibody (pure anti-NMDAR encephalitis) also occur. Therefore, it is important for clinicians to know the clinical and pathogenic differences between anti-NMDAR encephalitis with positive thyroid autoantibody and pure anti-NMDAR encephalitis for relevant treatment, predicting prognosis, and future follow-up.

Clinical and electroencephalographic manifestations of Hashimoto's encephalopathy / 中华全科医师杂志

Clinically rare with an acute or subacute onset,Hashimoto's encephalopathy (HE) is characterized as autoimmune encephalopathy with elevated anti-thyroid autoantibodies.If timely diagnosed and treated,its prognosis is often fair.Four HE cases admitted from January 2012 to June 2014 were analyzed with a literature review.HE 4 cases had a significantly higher level of thyroperoxidase (TPO) while the lowest increase over four folds.There were cognitive decline,memory loss and even coma.And 50％ had abnormal electroencephalogram (EEG) consistent with radiographic lesions.In short,EEG may aid an early diagnosis of HE.

Hashimoto's Encephalopathy Presented with Nonspecific Vasogenic Edema in Brain Magnetic Resonance Imaging

Hashimoto's encephalopathy (HE) is a rare autoimmune disorder characterized by a nonspecific encephalopathy with high titers of serum anti-thyroid antibody in the absence of other defined causes. A 54-year-old woman was admitted due to recurrent seizures and confusion. Her serum anti-thyroid antibody level was elevated, and brain MRI showed multiple instances of vasogenic edema. Her symptoms disappeared after treatment with high-dose steroids and antiepileptic drugs. We propose that HE should be considered in the differential diagnosis of multiple vasogenic edema on brain imaging.

Hashimoto's Encephalopathy Presented with Nonspecific Vasogenic Edema in Brain Magnetic Resonance Imaging

Hashimoto's encephalopathy (HE) is a rare autoimmune disorder characterized by a nonspecific encephalopathy with high titers of serum anti-thyroid antibody in the absence of other defined causes. A 54-year-old woman was admitted due to recurrent seizures and confusion. Her serum anti-thyroid antibody level was elevated, and brain MRI showed multiple instances of vasogenic edema. Her symptoms disappeared after treatment with high-dose steroids and antiepileptic drugs. We propose that HE should be considered in the differential diagnosis of multiple vasogenic edema on brain imaging.

Hashimoto's Encephalopathy with Positive 14-3-3 Protein in the Cerebrospinal Fluid and Atypical Course Mimicking Creutzfeldt-Jakob Disease

BACKGROUND: Creutzfeldt-Jakob disease (CJD) shares common clinical features with Hashimoto's encephalopathy (HE). The 14-3-3 protein is a relatively sensitive and specific marker of CJD but is not commonly detected in HE. We report the case of a patient with HE with unusual features including positive 14-3-3 protein in the cerebrospinal fluid (CSF) and an atypical course mimicking that of CJD. CASE REPORT: A 64-year-old male was admitted due to acute-onset cognitive dysfunction. HE was suspected based on increased titers of anti-thyroid microsomal antibody and an excellent response to steroid. However, 14-3-3 protein was detected in the CSF and a recurrent attack with progressive cognitive decline, pyramidal symptoms and myoclonus mimicking CJD occurred. Cognitive dysfunction showed progressive worsening and the response to steroid treatment was decreased. CONCLUSIONS: 14-3-3 protein could be considered a general marker of neuronal destruction and not specific to CJD. The clinical manifestations of HE are variable and its diagnosis is difficult due to the lack of a specific phenotype and reliable diagnostic criteria. We recommend that patients with clinical features of CJD and antithyroid antibodies should be considered for empirical steroid treatment for HE, despite a positive result for 14-3-3 protein.

Hashimoto's Encephalopathy Presenting with Ocular Flutter

Hashimoto's encephalopathy is a neurological disorder associated with high titers of antithyroid antibodies. The common neurologic symptoms are seizure, stroke-like episode, or mental change. However other atypical presentations, such as aphasia, myoclonus, ataxia, and cognitive dysfunction have also been described. We report hear a 44-year-old woman with Hashimoto's encephalopathy. The patient presented with ocular flutter as a principal manifestation, which is rare in Hashimoto's encephalopathy.

Nonconvulsive Status Epilepticus Associated with Hashimoto's Encephalopathy / 대한임상신경생리학회지

Nonconvulsive status epilepticus usually presents with altered mentation without distinct manifestations of seizures. It may be related with various medical disorders. Hashimoto's encephalopathy is characterized by various neurological manifestations accompanied by high titers of anti-thyroid antibodies. Here, we report a patient with nonconvulsive status epilepticus caused by Hashimoto's encephalopathy who showed a dramatic response to steroids.

Rara associação entre linfoma não-Hodgkin e encefalopatia de Hashimoto: relato de caso / Rare association between non-Hodgkin's lymphoma and Hashimoto's encephalopathy: case report

JUSTIFICATIVA E OBJETIVOS: O objetivo deste estudo foi apresentar um caso de doença incomum do sistema nervoso central, a encefalopatia de Hashimoto (EH), presente em um paciente portador de linfoma não-Hodgkin, condição sabidamente associada com manifestações raras no sistema nervoso,fato que permitiu uma análise muito ampla de diversos quadros neurológicos.RELATO DO CASO: Paciente do sexo masculino, 77 anos,portador de linfoma não-Hodgkin sob tratamento oncológico,que desenvolveu doença neurológica com as características clássicas de EH.CONCLUSÃO: O paciente em questão desenvolveu encefalopatia florida com múltiplas manifestações neurológicas, e com investigação semiótica armada extensa, o que permitiu a exclusão de diversas entidades com sinais e sintomas semelhantes.O tratamento com corticosteroide e plasmaférese resultou em brilhante remissão do seu quadro, ratificando o diagnóstico de EH.

BACKGROUND AND OBJECTIVES: The main objective of this study was to present a case about an uncommon pathology of the central nervous system, Hashimoto's encephalopathy (HE), found in a non-Hodgkin lymphoma patient, a condition widely known as being associated with rare manifestations in the central nervous system, a fact which allowed a broad analysis of many neurological manifestations.CASE REPORT: Male, 77 year-old patient with non-Hodgkin lymphoma undergoing oncologic treatment, who developed a neurological disease with classic characteristics of HE.CONCLUSION: This patient, suffering from Hashimoto thyroditis and non-Hodgkin lymphoma, developed a very distinctive encephalopathy with multiple neurological manifestations, and a very broad investigation made it possible to rule out various medical conditions with similar symptoms and signs. The treatment using corticosteroids and plasmapheresis resulted in excellent remission of his clinical picture, confirming the diagnosis of HE.

Serial Changes in Diffusion-Weighted Magnetic Resonance Images with Hypoperfusion on Brain SPECT in a Case of Hashimoto's Encephalopathy: Understanding Pathophysiology of Hashimoto's Encephalopathy

Diffuse or focal white matter hyperintensity lesions on MRI have been reported in only a few patients with Hashimoto's encephalopathy (HE), and anti-TPO antibody level is high in most cases. We report a 59-year-old woman who presented with acute onset of disorientation with confusion. Anti-thyroglobulin antibody was detected in high titer, although anti-TPO antibody titer was not high. Thyroid sonography and biopsy revealed Hashimoto's thyroiditis. Initial fluid-attenuated inversion recovery (FLAIR) image and diffusion-weighted imaging (DWI) revealed ill-defined, diffuse, high-signal intensity lesions on the deep white matters and globus pallidus. Brain SPECT showed significant hypoperfusion in both basal ganglia (especially globus pallidus), frontal and temporal lobes. With the impression of HE, the patient was treated on a high-dose steroid. Over the next 15 weeks, her cognition improved to a nearly normal state and the MRI findings on DWI and FLAIR showed resolution paralleling her clinical improvement. Our case illustrates the peculiar changes in the MR findings, especially in DWI, with hypoperfusion on brain SPECT in patients with HE and allows for a greater understanding of the pathophysiology of HE.

Hashimoto's Encephalopathy Presenting With Memory Disturbance and Complex Partial Seizure

No abstract available.

Nonconvulsive Status Epilepticus as the First Manifestation of Hashimoto's Encephalopathy / 대한간질학회지

Hashimoto's encephalopathy (HE) is an uncommon syndrome accompanied by Hashimoto's thyroiditis. It is an immune-mediated disorder characterized by subacute onset of confusion, altered consciousness, seizures and myoclonus. However, the diagnosis is often difficult because clinical manifestations are heterogeneous and nonspecific. Several reports of Hashimoto's encephalopathy presenting with focal or generalized seizures are described, but only few have focused on status epilepticus as the first clinical manifestation. We report a 60-year-old female patient who developed decreased consciousness with nonconvulsive status epilepticus due to Hashimoto's encephalopathy and was successfully treated with high doses of intravenous steroid.

Nonconvulsive Status Epilepticus as the First Manifestation of Hashimoto's Encephalopathy

Hashimoto's encephalopathy (HE) is an uncommon syndrome accompanied by Hashimoto's thyroiditis. It is an immune-mediated disorder characterized by subacute onset of confusion, altered consciousness, seizures and myoclonus. However, the diagnosis is often difficult because clinical manifestations are heterogeneous and nonspecific. Several reports of Hashimoto's encephalopathy presenting with focal or generalized seizures are described, but only few have focused on status epilepticus as the first clinical manifestation. We report a 60-year-old female patient who developed decreased consciousness with nonconvulsive status epilepticus due to Hashimoto's encephalopathy and was successfully treated with high doses of intravenous steroid.

A case of Hashimoto's encephalopathy presenting with seizures and psychosis / 소아과

Hashimoto's encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.

Non-Convulsive Status Epilepticus as a Manifestation of Hashimoto's Encephalopathy

No abstract available.