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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Hashimoto’s encephalitis HEencephalitis which responds to steroids and is associated with auto-immune thyroiditis, was described by Brain et al. Since then, about cases have been described It is probably under diagnosed as it is not well known 5. It presents with acute or sub-acute encephalopathy, tremor, myoclonus, ataxia, fits, psychosis or stroke like events, encefalolatia or relapsing, high titres of anti-thyroid antibodies but independent of thyroid function 2,4, Its two sub-types can co-exist: Diagnostic criteria are encephalopathy with elevated anti-thyroid antibodies in the absence of infection, tumour or alteration in the cerebral vascular encefalopztia.
A good response to corticoteroids is typical 2,4. She has universal alopecia which started when she was There followed a period of three years with difficulties at school.
She had three pregnancies with normal gestation, last 9 years ago. Eight years ago she developed hypothyroidism due to Hashimoto’s thyroiditis, and has been taking mcg levothyroxine daily. Her mother, 2 sisters and a daughter have Hashitmoto’s thyroiditis, one sister also has vitiligo. She had a 2 years history of feeling progressively tired, insomnia, cramps, tremors, arthralgia, paraethesia se her hands and back pain.
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. Over the proceeding 2 months she had lost the nails of her right hand and had lesions on her back and face compatible with excoriation.
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. She was jashimoto with disturbed thought. Ataxia, low amplitude high frequency action and postural tremors and myoclonus were observed in the four limbs.
She encefapopatia stable, suggesting a hypothalamic dysfunction. Serology, cultures and diagnostic imaging did not indicate an infectious process.
Anti-thyroperoxidase antibodies anti-TPO was 1. encefalopatiq
Oligoclonal bands were absent. She was maintained on oral prednisolone. 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 dose of thyroxine was reduced to mcg.
The dose of steroid was gradually reduced. Episodes of sinus tachycardia of bpm ed, with normal cardiac investigations.
The hypocalcemia and hyperphosphatemia continued, encefakopatia calcium supplements with a reduction of PTH, consistent with hypoparathyoidism, compounding a picture of auto-immune polyglandular disease. There was a reduction in anti-TPO which co-related with the clinical picture. Now, two years later, she is well and is on prednisolone 2.
There must be suspicion of HE patients with diverse encefalopatix manifestations and a history of Hashimoto’s thyroiditis. It is a diagnosis of exclusion in a clinical picture of encephalitis with negative investigations 2,4,7,9. The differential diagnosis includes incurable, degenerative diseases, like Creutzfeldt-Jacob disease, thus justifying a trial of steroids in selected patients 3. Little is known about the pathogenesis.
High titres of anti-TPO are common markers of an auto-immune process but it is unlikely that they have a pathogenic role 9. It is thought that there is an immune mediated cerebral vasculitis, with or without deposits of immune complexes, or an anti-neuronal mechanism mediated by anti-bodies 8.
Hypothyroidism, hormonal factors and cerebral hypoperfusion could contribute for pathogenesis 2. Evaluation of pathological anatomy revealed discrete venous or perivascular changes but dw a true vasculitis 4,8.
HE occur predominantly in women 4: Besides behavioral problems and cognitive decline, there may be fluctuating symptoms, tremor, transitory aphasia, myoclonus, ataxia, convulsions, sleep abnormalities, motor or sensory deficits and psychosis. Patients can have normal TSH and hypo or hyperthyroidism 2,4,8. 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.
There are EEG changes in most cases, mainly general slowing 2,8. MRI and cerebral angiography are normal in the majority of cases 2. The most common abnormality is diffuse increased signal in the white substance 2.
SPECT can be normal but usually shows focal hypoperfusion 8. The clinical improvement can occur on the first day, like in this case, but generally occurs within a week and up to 6 weeks 8. The majority of patients return to their normal neurological state.
Encefalopatia de Hashimoto
Some symptoms may persist, such as tremors and loss of memory. It is recommended to reduce the dose of steroids over several months.
Some patients need to stay on low doses or a new course of high dose of prednisolone is necessary 2. Like other autoimmune disease improvement may occur spontaneously 4. Therapeutic options for relapsing cases or as an alternative to steroids include azathioprine, cyclophosfamide, mycophenolate mofetil, methotrexate, IV immunoglobulin and plasmapheresis, alone or in combination 8.
Hepato-toxicity led to the interruption of azathioprine in this case. Alopecia is associated with polyglandular autoimmunity.
ENCEFALOPATIA DE HASHIMOTO by Raul Gonçalves on Prezi
Hasyimoto involvement is rare in this context, HE being the most common example. 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 correlation between anti-TPO and initial clinical progress observed was seen in some cases 8. This case is consistent with the literature with the presence of behavioral changes, tremors, myoclonus and enccefalopatia.
Some aspects warrant particular attention: Hashimoto’s disease and encephalopathy. Steroid-responsive encephalopathy associated with autoimmune thyroiditis. Hashimoto’s encephalopathy with clinical features similar to those of Creutzfeldt-Jacob disease.
J Neurol Sci ; J Clin Endocrinol Metab ; Long-term treatment of Hashimoto’s encephalopathy. J Neuropsychiatry Clin Neurosci ; Rarity of encephalopathy associated with autoimmune thyroiditis: Received 6 Novemberreceived in final form 30 March Accepted 26 May All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License.
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