Hashimoto's Encephalopathy Presenting with Smoldering Limbic Encephalitis

Nagano, Masahiro; Kobayashi, Katsuya; Yamada-Otani, Mayumi; Kuzuya, Akira; Matsumoto, Riki; Oita, Jiro; Yoneda, Makoto; Ito, Akio; Takahashi, Ryōsuke

doi:10.2169/internalmedicine.1289-18

Cited by 10 publications

(6 citation statements)

References 35 publications

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“…Conversely, Nagano et al . reported hypermetabolism of limbic lesions in 18 F‐fluorodeoxyglucose positron emission tomography, which reflected the disease activity degree in HE patients who showed limbic encephalitis …”

Section: Neuroimagingmentioning

confidence: 93%

Hashimoto encephalopathy

Matsunaga

Ikawa

Yoneda

2019

Clinical & Exp Neuroim

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Hashimoto encephalopathy (HE) is an autoimmune encephalopathy associated with autoimmune chronic thyroiditis. The clinical entity and nosology of HE have long been debated. Recently, new autoantibodies associated with autoimmune encephalitis have been discovered. With accumulated reported cases, and our discovery of serum autoantibody against NH2‐terminal of alpha‐enolase as a specific diagnostic marker, HE has been recognized as a distinct clinical entity. The condition appears dominant in females. The clinical features of HE are characterized by various neuropsychiatric symptoms, such as altered consciousness, psychosis and seizures, the presence of serum anti‐thyroid antibodies, normal or non‐specific changes in brain magnetic resonance imaging, diffuse slow wave activities in electroencephalogram and responsiveness to immunotherapy. A few pathological studies showed a lymphocytic infiltration of the small and medium‐sized vessels of the brain, which suggested vasculitis. Non‐specific neurological symptoms, laboratory findings and neuroimaging of HE make its diagnosis an oversight and difficult, whereas antibodies against NH2‐terminal of alpha‐enolase can be useful as a diagnostic biomarker for HE. In this review, we describe the current state of knowledge about HE and discuss progress in the diagnosis of HE.

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Section: Neuroimagingmentioning

confidence: 93%

Hashimoto encephalopathy

Matsunaga

Ikawa

Yoneda

2019

Clinical & Exp Neuroim

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“…Cognitive decline and psychosis/hallucinations would suggest a form of limbic encephalitis [ 14 ]. Stroke-like symptoms, myoclonus and tremors are also frequently seen, either alone or as part of a syndrome [ 15 ]. Sometimes, HE can present with an acute encephalopathy [].…”

Section: Discussion and Reviewmentioning

confidence: 99%

“…New onset status epilepticus (SE), including epilepsia partialis continua (EPC), and non-convulsive SE (NCSE) have been reported [ 25 ••, 26 – 28 ]. A limbic encephalitis-like presentation with subacute onset memory deficits, decreased level of consciousness, seizures, and behavioral change can also occur [ 15 ]. Autoimmune encephalitis including Anti-IgLON5 disease [ 29 ••] needs to be excluded in such cases with appropriate tests for neuronal surface antibodies.…”

Section: Discussion and Reviewmentioning

confidence: 99%

Hashimoto’s Encephalopathy: Case Series and Literature Review

Chaudhuri

Mukherjee

Chakravarty

2023

Curr Neurol Neurosci Rep

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Purpose of ReviewTo describe the clinical manifestations of Hashimoto's encephalopathy (HE) and discuss its pathogenesis in light of recent research. Recent FindingsThe pathogenesis of HE is uncertain. Available evidences point towards an autoimmune etiology due to vasculitis or other inflammatory process. Detection of thyroid antibodies -antithyroid peroxidase and anti-thyroglobulin are essential for diagnosis. Autoimmune encephalitis including Anti-IgLON5 disease needs to be excluded in suspected cases with appropriate tests for neuronal surface antibodies. Detection of thyroid autoantibodies is nonspecific, as these can be detected in some normal individuals and in other autoimmune diseases. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels in younger males with very high levels of thyroid antibodies. The role of the thyroid autoantibodies in the central nervous system (CNS) tissue damage remains unclear and these can act only as markers for diagnosis. Conversely, they have a role to play in determining the thyroid pathology -more glandular fibrosis associated with thyro-peroxidase antibody than with the thyroglobulin antibody. Summary HE is a syndrome characterized by altered mental status, confusion, hallucinations, delusions, and sometimes seizures, in association with high serum anti-thyroid antibody concentration that is usually responsive to glucocorticoid therapy. Diagnosis requires the exclusion of other causes of encephalopathies and encephalitis including autoimmune encephalitis associated with neuronal surface antibodies and paraneoplastic ones. Diagnosis also is dependent on the demonstration of thyroid autoantibodies in serum. Since there is no direct pathophysiologic link between antithyroid antibodies, Hashimoto thyroiditis and the cerebral syndrome, the nomenclature HE could be misleading. The response to steroids led to a renaming of the syndrome to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), though some cases do not respond to steroids. In recent years, attention has turned to an aggressive form of Hashimoto's thyroiditis accompanied by elevated serum IgG4 levels (IgG4-related disease). This is characterized by a higher incidence in men (5:1) than in women, onset at a younger age, more intense thyroid inflammation and higher antithyroid antibody titters. Such patients have excessive production of IgG4 + plasmacytes, which infiltrate various organs leading to their fibrosis and sclerosis, sometimes resulting in inflammatory tumors. HE is treated with corticosteroids along with treatment of the dysthyroid condition, if any.There are yet no guidelines regarding steroid dose and/or duration.

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“…Тремор, миоклонии, экстрапирамидные нарушения, судорожные и/или эпилептические припадки могут присутствовать при обоих типах развития заболевания, но более характерны для диффузного прогрессирующего типа. Клинические проявления ЭХ во взрослом возрасте представлены генерализованными тонико-клоническими припадками (60-66% пациентов) [11]. Эпилептический статус редко встречается у взрослых пациентов с ЭХ, но описан у детей.…”

Section: резюме _____________________________________________________...unclassified

Hashimoto’s Encephalopathy: A Literature Review and Own Clinical Observation

Костюкевич¹,

Рушкевич²,

Лихачев³

et al. 2023

Неврология И Нейрохирургия. Восточная Европа

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Энцефалопатия Хашимото представляет собой редкий нейроэндокринный синдром и является вариантом энцефалопатии аутоиммунной этиологии, ассоциированной с выработкой антитиреоидных антител. Заболевание может встречаться в любом возрастном периоде, средний возраст дебюта составляет 44 года, 20% случаев приходится на лиц моложе 18 лет. Женщины болеют в 4 раза чаще. Заболевание встречается редко, недостаточно изучено, отличается трудностями диагностики, динамичностью клинических симптомов и их обратимостью в ответ на иммуносупрессивную терапию глюкокортикостероидами (ГКС). Сложность диагностики энцефалопатии Хашимото связана с особенностями течения и полиморфизмом клинических симптомов, которые могут имитировать картину различных неврологических и психиатрических расстройств. Тем не менее своевременно установленный диагноз позволяет эффективно провести иммуносупрессивную терапию и быстро купировать симптомы заболевания, в том числе угрожающие жизни пациента. Обратимость симптоматики заболевания в ответ на терапию ГКС свидетельствует о развитии особых, скорее функциональных, чем грубых, органических нарушений в нервной системе. Назначение в адекватных дозировках ГКС позволяет быстро купировать симптомы энцефалопатии Хашимомто, достичь полного регресса психоневрологических расстройств, а также обусловливает хороший долгосрочный прогноз. Авторы представили обзор литературы и собственное клиническое наблюдение энцефалопатии Хашимото у пациентки 50 лет. Hashimoto’s encephalopathy is a rare neuroendocrine syndrome being a variant of autoimmune encephalopathy associated with the production of antithyroid antibodies. The disease can occur in any age period, the average age of onset is 44 years, in 20% of cases it occurs in persons under 18 years of age. Women get sick 4 times more often. The disease is rare, insufficiently studied, and characterized by diagnostic difficulties, clinical symptoms dynamics, and their reveфrsibility in response to immunosuppressive therapy with glucocorticosteroids (GCS). The difficulty in diagnosing Hashimoto’s encephalopathy is associated with peculiarities of its course and polymorphism of clinical symptoms, which can imitate pictures of various neurological and psychiatric disorders. Nevertheless, a timely diagnosis allows effective immunosuppressive therapy and rapid relief of symptoms, including life-threatening ones. The reversibility of disease symptoms in response to GCS therapy indicates special, rather functional, than rough, organic disorders in the nervous system. Prescribing GCS in adequate dosages allows quickly relieving Hashimoto’s encephalopathy symptoms, achieving a complete regression of neuropsychiatric disorders, and also determines a good long-term prognosis. The authors presented a literature review and their own clinical observation of Hashimoto’s encephalopathy in a 50-year-old female patient.

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Hashimoto's Encephalopathy Presenting with Smoldering Limbic Encephalitis

Cited by 10 publications

References 35 publications

Hashimoto encephalopathy

Hashimoto encephalopathy

Hashimoto’s Encephalopathy: Case Series and Literature Review

Hashimoto’s Encephalopathy: A Literature Review and Own Clinical Observation

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