Hashimoto encephalopathy: Etiologic considerations

doi:10.1016/0887-8994(94)90011-6

Pediatric Neurology

Volume 11, Issue 4, November 1994, Pages 328-331

https://doi.org/10.1016/0887-8994(94)90011-6 Get rights and content

Abstract

A 15-year-old girl is described with encephalopathy associated with Hashimoto thyroiditis which subsequently developed into autoimmune thyrotoxicosis (hashitoxicosis) and distal renal tubular acidosis at age 5 years, pernicious anemia at age 9, and encephalopathy at age 12. Thyroid hormone levels were within the normal ranges at the time of neurologic presentation. Serum IgG concentration and oligoclonal IgG bands in cerebrospinal fluid closely correlated with the severity of neurologic symptoms. Treatment with glucocorticoids improved the level of consciousness but her mental state continued to deteriorate. Repeated cranial computed tomographic scans revealed progressive symmetric atrophy in the gray matter without infarction. These findings suggest that encephalopathy associated with Hashimoto thyroiditis is caused by an antineuronal antibody-mediated reaction.

References (9)

M Shein et al.
Encephalopathy in comprensated Hashimoto's thyroiditis: A clinical expression of autoimmune cerebral vasculitis
Brain Dev
(1986)
EH Jellinek et al.
Hashimoto's disease, encephalopathy, and splenic atrophy
Lancet
(1976)
RL Becker et al.
Significance of morphologic thyroiditis
Ann Intern Med
(1965)
N Suzuki et al.
Hashimoto thyroiditis, distal renal tubular acidosis, pernicious anaemia and encephalopathy: A rare combination of auto-immune disorders in a 12-year-old girl
Eur J Pediatr
(1994)

There are more references available in the full text version of this article.

Cited by (80)

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Citation Excerpt :
Also, the brain of HE patients was quite rare available for post mortem pathomorphological studies – just in few cases: like in suicides, in very old deceased patients for differentiation with neurodegenerative diseases, or after lethal epileptic attacks [92,93,96,97]. Nevertheless, in such cases several authors documented presence of vasculitis (phlebitis or polyangiitis) through all the brain or at least in the brainstem of HE victims [93,96–99]. Small and medium-sized blood vessels or perivascular spaces were infiltrated with lymphocytes, predominantly of T-subsets [98,100].
The versatile clinical manifestations of the Hashimoto's chronic autoimmune thyroiditis often include psycho-neurological disorders. Although hypothyroidism disturbs significantly the ontogenesis and functions of central nervous system, causing in severe cases of myxedema profound impairment of cognitive abilities and even psychosis, the behavioral, motor and other psychoneurological disorders accompany euthyroid and slightly hypothyroid cases and periods of Hashimoto's disease as well, thus constituting the picture of so called “Hashimoto's encephalopathy”. The entity, although discussed and explored for more than 50 years since its initial descriptions, remains an enigma of thyroidology and psychiatry, because its etiology and pathogenesis are obscure. The paper describes the development of current views on the role of thyroid in ontogeny and functions of brain, as well as classical and newest ideas on the etiology and pathogenesis of Hashimot's encephalopathy. The synopsis of the world case reports and research literature on this disorder is added with authors' own results obtained by study of 17 cases of Hashimoto's thyroiditis with schizophrenia-like clinical manifestations. The relation of the disease to adjuvant-like etiological factors is discussed. Three major mechanistic concepts of Hashimoto's encephalopathy are detailed, namely cerebral vasculitis theory, hormone dysregulation theory and concept, explaining the disease via direct action of the autoantibodies against various thyroid (thyroperoxidase, thyroglobulin, and TSH-receptor) and several extrathyroid antigens (alpha-enolase and other enzymes, gangliosides and MOG-protein, onconeuronal antigens) – all of them expressed in the brain. The article demonstrates that all above mentioned concepts intermingle and prone to unification, suggesting the unified scheme of pathogenesis for the Hashimoto's encephalopathy. The clinical manifestations, criteria, forms, course, treatment and prognosis of Hashimoto's encephalopathy and its comorbidity to other diseases – are also discussed in brief. The relation between Hashimoto's encephalopathy and non-vasculitis autoimmune encephalomyelitides of paraneoplastic and non-paraneoplastic origin is emphasized [1 figure, bibliography – 200 references].
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Citation Excerpt :
In children focal hyperintensity or volume depletion of the nucleus accumbens, frontal white matter changes, cerebellar atrophy and hippocampal and periventricular lesions have been documented [82]. Among HE cases presenting with seizures, bitemporal symmetrical hippocampal lesions with focal edema have been reported [83,84], as well as dural enhancement [21], and diffuse/asymmetrical atrophy [83,85]. Others have reported diffuse white matter involvement simulating leukodystrophy that has normalized following treatment [85].
Hashimoto's encephalopathy (HE) is a rare not well understood, progressive and relapsing multiform disease, characterized by seizures, movement disorders, subacute cognitive dysfunction, psychiatric symptoms and responsiveness to steroid therapy. The disorder is generally associated with thyroid diseases and the most common feature is the presence of anti-thyroperoxidase antibodies (TPOAb). Patients are usually euthyroid or mildly hypothyroid at presentation. All age groups can be affected. The pathophysiology is still unclear, especially the link between elevated serum TPOAb and the encephalopathy. Most reported cases occurred in women and girls. Unspecific symptoms, non-pathognomonic laboratory neurophysiology and neuroimaging features make its diagnosis a real challenge for clinicians.
The case of a 16 year old boy, with a clinical picture of HE associated with hypothyroidism, demonstrating an excellent response to high dose steroids is presented together with a systematic review of the literature.
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2012, Revue de Medecine Interne
L’encéphalopathie d’Hashimoto a été décrite pour la première fois en 1966 par Lord Brain. Depuis, une controverse est née quant à l’existence même de cette entité dont les appellations sont nombreuses. Pour autant, le nombre de cas rapportés dans la littérature est en constante augmentation et le clinicien est confronté à la difficile situation de patients ayant des symptômes neuropsychiatriques et des anticorps antithyroïdiens positifs. Nous proposons ici une mise au point, basée sur une recherche Medline ayant répertorié 316 références publiées entre 1961 et 2011. Syndrome ou maladie à part entière, l’encéphalopathie d’Hashimoto est rare, parfois mortelle. Son diagnostic et sa prise en charge souffrent de l’absence de critères consensuels.
Hashimoto's encephalopathy was first described by Lord Brain in 1966. Since, other designations have been proposed and the existence of the disease itself has been debated. However, the number of reported cases in the literature is still increasing and physicians are sometimes confronted with patients with neuropsychiatric manifestations and positive thyroid antibodies. This article is an update based upon a search through Medline database that identified 316 references published between 1961 and 2011. Hashimoto's encephalopathy is a rare condition for which there is a need for both diagnostic criteria and therapeutic consensus.
Recurrent partial seizures with ictal yawning as atypical presentation of Hashimoto's encephalopathy (steroid-responsive encephalopathy associated with autoimmune thyroiditis)
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Citation Excerpt :
Hashimoto's encephalopathy, also referred to as SREAT, is a rare condition whose etiology is unknown, though autoimmune-mediated mechanisms are believed to be involved in its pathogenesis [1, 2]. The pathogenetic hypotheses include autoimmune vasculitis, autoimmune reaction to antigens shared by the thyroid gland and the central nervous system, cerebral hypoperfusion, and the toxic effect of thyrotropin-releasing hormone [4, 5]. To date, HE is described as a disease affecting both the neuroendocrine and nervous systems.
Hashimoto's encephalopathy (HE), also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare condition whose pathogenesis is unknown, though autoimmune-mediated mechanisms are thought to be involved. The prevalent neurological manifestations of this disorder are epileptic seizures and psychocognitive disorders associated with EEG alterations. High anti-thyroid antibody titers (particularly in cerebrospinal fluid) and the effectiveness of steroid therapy are usually considered to be crucial elements in the diagnostic process. We describe a 19-year-old female patient who had been referred to the psychiatric unit because of behavioral disorders characterized predominantly by delirium with sexual content. She developed recurrent focal seizures characterized by atypical ictal semiology (repetitive forceful yawning) and a rare EEG pattern (recurrent seizures arising from the left temporal region without evident “encephalopathic” activity). The presence of anti-thyroperoxidase antibodies in her cerebrospinal fluid and a good response to steroids confirmed the diagnosis of HE. The atypical presentation in the case we describe appears to widen the electroclinical spectrum of HE and highlights its importance for differential diagnosis purposes in the neuropsychiatric setting.
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Case reportHashimoto encephalopathy: Etiologic considerations

Abstract

Brain Dev

Lancet

Significance of morphologic thyroiditis

Ann Intern Med

Hashimoto thyroiditis, distal renal tubular acidosis, pernicious anaemia and encephalopathy: A rare combination of auto-immune disorders in a 12-year-old girl

Eur J Pediatr

Case report
Hashimoto encephalopathy: Etiologic considerations