Hashimoto encephalopathy (HE) is an uncommon syndrome associated with Hashimoto thyroiditis Cantón A, de Fàbregas O, Tintoré M, et al. Request PDF on ResearchGate | On Jun 1, , Paloma Bacarizo and others published Encefalopatía de Hashimoto en un paciente de 20 años. CLINICAL/SCIENTIFIC NOTE. Hashimoto’s encephalopathy. Encefalite de Hashimoto. Renata Telles Rudge de AquinoI; Eduardo Genaro MutarelliII.

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Post-mortem studies of some individuals have shown lymphocytic vasculitis of venules and veins in the brain-stem and a diffuse gliosis encefalopaatia gray matter more than white matter. The dose of steroid was gradually reduced. Hashimoto’s disease and encephalopathy. Hashimoto’s encephalopathy with clinical features similar to those of Creutzfeldt-Jakob disease.

Anti-thyroperoxidase antibodies anti-TPO was 1. Encephalopathy associated with Hashimoto thyroiditis: All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. In this period there was a regression of all signs and symptoms.

The presence rapidly progressive dementia, ataxia, myoclonus, and especially the presence of triphasic complexes in the EEG, make dee diagnosis a probable one for Encegalopatia disease. She had a history of hypothyroidism and did not use any medication. Her mother, 2 sisters and a daughter have Hashitmoto’s thyroiditis, one sister also has vitiligo. The thyroid-stimulating hormone TSH was slightly elevated at 7. How to cite this article.

Encefalolatia the proceeding 2 months she had lost the nails of her right hand and had lesions on her back and face compatible with excoriation. Failure of some patients encefalopatis respond to this first line treatment has produced a variety of alternative treatments including azathioprinecyclophosphamidechloroquinemethotrexateperiodic intravenous immunoglobulin and plasma exchange.

Evaluation of pathological anatomy revealed discrete venous or perivascular changes but not a true vasculitis 4,8. It presents with acute or sub-acute encephalopathy, tremor, myoclonus, ataxia, fits, psychosis or stroke like events, progressive or relapsing, high titres of anti-thyroid antibodies but independent of thyroid function 2,4, Serology, cultures and diagnostic imaging did not indicate an infectious process.


Hashimoto’s encephalopathy with clinical features similar to those of Creutzfeldt-Jakob disease

Archived from the original on The differential diagnosis includes incurable, degenerative diseases, like Creutzfeldt-Jacob disease, thus justifying a trial of steroids in selected patients 3.

The electroencephalogram EEG at admission was hashi,oto, with slow, monomorphic, occasionally sharp, triphasic, with theta-delta frequency ranges from 2 to 3 Hz, intermittent, bilateral waves, predominantly in the temporoparietal regions Fig 2A.

The hypocalcemia and hyperphosphatemia continued, despite calcium supplements with a reduction of PTH, consistent with hypoparathyoidism, compounding a picture of auto-immune polyglandular disease.

Hashimoto’s encephalopathy is a rare condition associated with Hashimoto thyroiditis, the presence of high concentrations of anti-thyroid antibodies, without any evidence of thyroid dysfunction, and an excellent response to treatment with corticosteroids 2.

Accepted 26 May Hashimlto ICD – Little is known about the pathogenesis. The most common findings are diffuse or generalized slowing or frontal intermittent rhythmic delta activity. Oligoclonal bands were absent. Hypothyroidism, hormonal factors and cerebral hypoperfusion could contribute for pathogenesis 2.

Department of Medical Genetics. In this case, the Hashimoto’s encephalopathy diagnosis was made based on the exclusion of other toxic-metabolic hashimoti and neurological diseases of infectious or vascular origin, and on the detection of high concentrations of anti-TPO antibodies. High titres of anti-TPO are common markers of an auto-immune process but it is unlikely that they have a pathogenic role 9.

Encefalopatia de Hashimoto

The symptoms were recurrent and reversible and were not associated with thyroid dysfunction 1. Prion diseases are under compulsory notification in Brazil: The mechanism of pathogenesis is not known but it has been hypothesized to be an autoimmune disorder, similar to Hashimoto’s thyroiditis as its name suggests.

The dose of thyroxine was reduced to mcg. Symptoms sometimes resolve themselves within emcefalopatia to weeks, leaving a patient undiagnosed. Since then, Hashimoto’s encephalopathy has been recognized as a rare condition associated to Hashimoto’s thyroiditis and to the presence of high concentrations of antithyroid antibodies, hashimotp those described in myxedema and in thyrotoxicosis 2.


The magnetic resonance imaging MRI of the brain showed small changes necefalopatia were not significant, such as the presence of hypersignal focus in the corona radiata and bilaterally semioval center Fig 1.

HE occur predominantly in women 4: The responsiveness of HE to steroids or other therapies such as plasmapheresis supports the hypothesis that this is a disorder that involves immune pathogenic mechanisms. The prevalence has been estimated to be 2.

Hashimoto’s encephalopathy

Hashimoto’s disease and encephalopathy. Hashimoto’s encephalopathyalso known as steroid responsive encephalopathy associated with autoimmune thyroiditis SREATis a neurological condition characterized by encephalopathythyroid autoimmunity, and good clinical response to steroids. Accepted 23 September Since that time, research has expanded and numerous cases are being reported by scientists around the world, suggesting that this rare condition is likely to have been significantly undiagnosed in the past.

J Neurol Sci ; A recent study by Martins et al. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or encefalppatia are really associated with pathogenic mechanisms of the disorder.

Since then, about cases have been described To minimize the side effects of steroid therapy, in the second month the dose was reduced and azathioprine added, but due to hepato-toxicity this was suspended. The first case of HE was described by Brain et al.

She was stable, suggesting a hhashimoto dysfunction.

This report emphasizes the importance of a differential diagnosis between the two pathologies.