Hashimoto's encephalopathy

Abstract

Hashimoto's encephalitis (HE), encephalitis which responds to steroids and is associated with auto-immune thyroiditis, was described by Brain et al. in 1966 1 . Since then, about 100 cases have been described [2][3][4][5][6][7][8][9][10] . 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, progressive or relapsing, high titres of anti-thyroid antibodies but independent of thyroid function 2,4,10 . Its two sub-types can co-exist: multiple stroke like events, and diffuse progressive, with dementia and psychiatric symptoms 3 . Diagnostic criteria are encephalopathy with elevated anti-thyroid antibodies in the absence of infection, tumour or alteration in the cerebral vascular system. A good response to corticoteroids is typical 2,4 . CaSEA 42 years old woman, white, secondary school education, married, from São Paulo, was admitted in Hospital in January 2007 with an acute febrile condition. She has universal alopecia which started when she was 13. 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 125 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 in 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 150 mg, carbamazepine 600 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.Five days prior to admission she had a high fever (41 o C), body pain, tremors, cold extremities and pins and needles in her legs.On admission, axillary temperature was 41 o C. Normotensive, pulse 120 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 histrionic with disturbed thought. Ataxia, low amplitude high frequency action and postural tremors and myoclonus were observed in the four limbs. On the second day her temperature fell to 34 o C. She was stable, suggesting a hypothalamic dysfunction.Serology, cultures and diagnostic imaging did not indicate an infectious process. ESR was normal (7 mm) and CRP slightly elevated (2.23 mg/dL). ANCA, anti-DNA and ANA were negative. Biochemical results showed raised aminotransferases (AST >2×; ALT >3×), hypocalcemia and hyperphosphatemia. Anti-thyroperoxidase antibodies (anti-TPO) was 1.100 U/mL with normal TSH and free T4.Cerebral MRI, eletroencephalogram (EEG) and single photon emission computed tomography (SPECT) were all normal. Lumbar puncture was clear, 1 cell/mm 3 , mild hyperproteinorrachia (51 mg/dL...

Psikotik bulgularla seyreden Hashimoto ensefalopatisi: Olgu sunumu

“…EEG signs in HE may be a nonspecific slowing of the background rhythm, focal sharp and steep waves, triphasic rhythmic activity, frontal intermittent rhythmic activity, and temporary epileptic activities (14). While the EEG findings in our case support HE, they are not specific to this disease.…”

“…Mean age at onset is 41-44 years. In 20% of the cases, onset is seen below the age of 18 (14). In our case, age at disease onset was 81 years.…”

“…In addition to elevated CSF proteins, OCB (oligoclonal band) and lymphocytic pleocytosis can be seen, while the glucose level is normal. No source of infection is to be found in the CSF that could explain the clinical picture (14,15). While elevated protein in the CSF and diffuse anomalies in the EEG are described as typical (17,18), it is reported that these are not specific (6,15,17,19).…”

Recurrent Hashimoto's Encephalopathy, Showing Spontaneous Remission: A Case Report

“…TPO-Ab is present in 95-100% and TGAb in 73% of patients with HE (8). Elevated titers of antithyroid antibodies are the most relevant paraclinical finding, which could be considered to be a mark of HE.…”

Hashimoto Encephalopathy: A Rare Intricate Syndrome

“…She had alopecia since 13 years of age (51) and Hashimoto thyroiditis since 8 years prior to the case report. Since 2 years prior to the case report, she also was progressively tired with insomnia, cramps, tremors, athralgia, back pain, and paraesthesia of the hands as well as behavioral changes and agitation that resulted in psychiatric treatment.…”