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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Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Long-term treatment of Hashimoto’s encephalopathy. Migraine Familial hemiplegic Cluster Tension.

Evaluation of pathological anatomy revealed discrete venous encefaalopatia perivascular changes but not a true vasculitis 4,8.

Encefalopatia de Hashimoto

In this case, the Hashimoto’s encephalopathy encefalopqtia was made based on the exclusion of other toxic-metabolic encephalopathic and neurological diseases of infectious or vascular origin, and on the detection of high concentrations of anti-TPO antibodies. 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.

How to cite this article. The differential diagnosis includes incurable, degenerative diseases, like Creutzfeldt-Jacob disease, thus justifying a trial of steroids in selected patients 3.

Oligoclonal bands were absent. We related the case of a patient with Hashimoto’s encephalopathy and clinical features similar to those of Creutzfeldt-Jakob disease. Journal of the Neurological Sciences.

It is sometimes referred to as a neuroendocrine disorder, although the condition’s relationship to the endocrine system is widely disputed.

Hashimoto’s encephalopathy.

Hashimoto’s encephalopathy HE is a controversial neurological disorder that ahshimoto 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.

Encephalomyelitis Acute disseminated Myalgic Meningoencephalitis. Up to there were almost published case reports of this disease. It is probably under diagnosed as it is not well known 5. Normotensive, pulse bpm, she had universal alopecia, cyanosis and pale alternating in the extremities, growing nails of the right hand and scars on her back and face. Received 18 Junereceived in final form 1 September Further controlled 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.


The symptoms were recurrent and reversible and were not associated with thyroid dysfunction 1. The clinical manifestations associated with the presence of triphasic complexes in the EEG or the detection of the protein in the CSF make the diagnosis possible. Brain herniation Reye’s Hepatic encephalopathy Toxic encephalopathy Hashimoto’s encephalopathy. The hypocalcemia and hyperphosphatemia continued, despite calcium supplements with a reduction of PTH, consistent with hypoparathyoidism, compounding a picture of auto-immune polyglandular disease.

This improvement in the clinical presentation can be correlated to the complete resolution of the electroencephalographic changes Fig 2B. For many other patients, the condition may result in ongoing problems with a variety of manifestations, often confusing clinicians due to the diffuse nature of symptoms.

Its two sub-types can co-exist: 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.

Alopecia is associated with polyglandular autoimmunity. 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, J Neurol Neurosurg Psychiatry ; InBrain et al. Her mother, 2 sisters and a daughter have Hashitmoto’s thyroiditis, one sister also has vitiligo. The clinical presentation may suggest a diagnosis of Creutzfeldt-Jakob disease.

Brain Encephalitis Viral encephalitis Herpesviral encephalitis Limbic encephalitis Encephalitis lethargica Cavernous sinus thrombosis Brain abscess Amoebic.

There have been no controlled trials so the optimal treatment is not known. Archived from the original on Received 6 Novemberreceived in final form 30 March However, the role of those antibodies and their pathophysiology are unknown. 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.


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. A good response to corticoteroids is typical 2,4. The thyroid-stimulating hormone TSH was slightly elevated at 7. The levels of triiodothyronine T3 and free thyroxine T4 L were within normal limits. Views Read Edit View history. Duration of treatment is usually between 2 and 25 years.

At admission, she was sleepy, disoriented, showing myoclonus, paratonia on the back of the head and arms, her deep reflexes were symmetrical with plantar flexion response and suction and grasping reflexes were present. It is associated with Hashimoto’s thyroiditis. From Wikipedia, the free encyclopedia.

Diagnostic criteria are encephalopathy with elevated anti-thyroid antibodies in the absence of infection, tumour or alteration in the cerebral vascular system.

High titres of anti-TPO are common markers of an auto-immune process encrfalopatia it is unlikely that they have a pathogenic role 9. The recognition of the condition was essential for the treatment and regression of the clinical presentation.

She had a history of hypothyroidism and did not use any medication. Prion diseases are under compulsory notification in Brazil: Some patients need to stay emcefalopatia low doses or a new course of high dose of prednisolone is necessary 2.