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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From Wikipedia, the free encyclopedia. Although it has been linked to cases of Hashimoto’s thyroiditis or thyroid dysfunction, the most common immunological feature of HE is the presence of high titers of antithyroglobulin or anti-TPO antimicrosomal antibodies.
Hashimoto’s encephalitis HEencephalitis which responds to steroids and hashimoho associated with auto-immune thyroiditis, was described by Brain et encefalopatka. Recent studies have shown the presence of anti-thyroid antibodies in the CSF 5 and alpha-enolase antibodies in the serum of patients with Hashimoto’s encephalopathy, suggesting the involvement of an autoimmune mechanism in this condition 6.
Consistent with this hypothesis, autoantibodies to alpha-enolase have been found to be associated with Hashimoto’s encephalopathy. She had a 6 month history of behavioral changes and agitation, resulting in psychiatric treatment of estazolan 2 mg, bupropion mg, carbamazepine mg and duoxetine 30 mg daily. Prion diseases are under compulsory notification in Brazil: Antithyroid antibodies in the CSF: We believe that this pathology is misdiagnosed, therefore, tests detecting the presence of anti-thyroid antibodies are recommended in a clinical presentation of encephalopathy of subacute onset or rapidly progressive dementia associated with myoclonus and triphasic complexes in the EEG, when the ethiology is not identified.
As mentioned above, autoantibodies to alpha- enolase associated with Hashimoto’s encephalopathy have thus far been the most hypothesized mechanism of injury.
InBrain et al. At present, it is unclear whether antithyroid antibodies represent an immune epiphenomenon in a subset of patients with encephalopathic processes or they are really associated with pathogenic mechanisms of the disorder.
All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Further hasimoto studies of the use of steroids, plasmapheresis, or immunosuppressant medications are needed in the future to prove the concept of the pathogenic role of antithyroid antibodies in HE. Hashimoto’s disease and encephalopathy.
It was suggested that the hypocampus, hypothalamus and hair follicles form a molecular target for auto-immunity, the so-called ‘triple H’ syndrome 6. The first case of HE was described by Brain et al. This case is consistent with the literature with the presence of behavioral changes, tremors, myoclonus and ataxia.
Hashimoto’s encephalopathy HE is a controversial neurological disorder that comprises a heterogenous group of neurological symptoms that manifest in patients with high titers of antithyroid antibodies.
Patients can have normal TSH and hypo or hyperthyroidism 2,4,8. The magnetic resonance imaging MRI of the brain showed small changes that were not significant, such as the presence of hypersignal focus in the corona radiata and bilaterally semioval center Fig 1.
The dose of thyroxine was reduced to mcg. Prominent triphasic waves, focal slowing, epileptiform abnormalities, photoparoxysmal and photomyogenic responses may be seen.
Hashimoto’s encephalopathy – Wikipedia
It is associated with Hashimoto’s thyroiditis. The main change in the CSF is the increase in total protein, that was mild in this case, and rarely some lymphocytic pleocytosis 2,4. Japan, and Depannient of Anatomy and Biology.
Rarity of encephalopathy associated with autoimmune thyroiditis: The levels of triiodothyronine T3 and free thyroxine T4 L were within normal limits.
The symptoms were recurrent and reversible and were not associated with thyroid dysfunction 1. Accepted 26 May Ataxia, low amplitude high frequency action and postural tremors and myoclonus were observed in the four limbs. Over scientific articles on Hashimoto’s encephalopathy were published between and Now, two years later, she is well and is on prednisolone 2.
A recent study by Martins et al. However, the role of those antibodies and their pathophysiology are unknown. In this period there was a regression of all signs and symptoms.
J Neurol Neurosurg Psychiatry ; Hashimoto’s encephalopathy with clinical features similar to those of Creutzfeldt-Jakob disease. Long-term treatment of Hashimoto’s encephalopathy. Encephalopathy associated with Hashimoto thyroiditis: A good response to corticoteroids is typical 2,4. Between and 43 cases were published. This improvement in the clinical presentation can be correlated to the complete resolution of the electroencephalographic changes Fig 2B.
The patients met the clinical criteria for Creutzfeldt-Jakob disease and were notified 8. Since that time, research has expanded and numerous hasgimoto are being reported by scientists around the world, suggesting that this rare condition is likely to have been significantly undiagnosed in the past.
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, unlike those described in myxedema and in thyrotoxicosis 2.
D ICD – The criteria established by World Health Organization WHO for diagnosing to sporadic Creutzfeldt-Jakob disease sCJD d progressive dementia with a duration of less than two years and two of the clinical findings – myoclonus, cerebellar or visual symptoms, pyramidal or extrapyramidal signs and akinetic mutism.
This report emphasizes the importance of a differential diagnosis between the two pathologies. The electroencephalogram EEG at admission was abnormal, 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. High titres of anti-TPO are common markers of an auto-immune process but it is unlikely that they have a pathogenic role 9. Accepted 23 September In the first two weeks, a considerable improvement in level of consciousness and myoclonus was encefalkpatia.
Failure of some patients to respond to this first line treatment has produced a variety of alternative treatments including azathioprinecyclophosphamidechloroquinemethotrexateperiodic intravenous immunoglobulin and plasma exchange.
Proteomic analysis of human brain identifies alpha-enolase as a novel autoantigen in Hashimoto’s encephalopathy. Received 18 Junereceived in final form 1 September She had a 2 years history of feeling progressively tired, insomnia, cramps, tremors, arthralgia, paraethesia in her hands and back pain.