Hashimoto Encephalopathy

Chong, Ji Y.; Rowland, Lewis P.; Utiger, Robert D.

doi:10.1001/archneur.60.2.164

Cited by 522 publications

(584 citation statements)

References 56 publications

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“…In the CSF the most frequent finding is elevated protein, as found in our patient, though anti-thyroid antibodies have also been noted (these were not tested in our patient) [1,3] . EEG abnormalities are seen in a majority of patients, most commonly diffuse slowing but also triphasic waves, periodic sharp waves, and frontal rhythmic slowing [1,4,5] .…”

Section: Discussionsupporting

confidence: 59%

“…Supportive laboratory tests include thyroid-related antibodies such as anti-thyrotropin, anti-TPO, anti-thyroglobulin, and anti-alpha-enolase. An association with thyroid disorders is sometimes found but neurologic symptoms do not appear to depend on thyroid status [3,4] . Though anti-alpha-enolase was not tested in our patient, her anti-thyroid antibodies were noted to be significantly elevated.…”

Section: Discussionmentioning

confidence: 99%

“…EEG abnormalities are seen in a majority of patients, most commonly diffuse slowing but also triphasic waves, periodic sharp waves, and frontal rhythmic slowing [1,4,5] . There are no neuropathology findings specific to HE though an encephalitic or vasculitic process has been debated in the literature [1,3,4,6,7] . A relationship to CNS demyelination has also been shown [4,8,9] .…”

Section: Discussionmentioning

confidence: 99%

“…MRI findings can also change over time within the same patient [1,4] . A review of 85 HE patients showed that 42 of 82 patients (52%) who had undergone neuroimaging had normal findings [3] . In a review of 121 patients, 71 patients were noted to have normal neuroimaging (it is unclear how many of these 121 patients underwent imaging, however) [4] .…”

Section: Discussionmentioning

confidence: 99%

“…It is unclear if the anti-thyroid antibodies that characterize this disorder are an autoimmune epiphenomenon or are pathogenic [1] . Many prefer the term "steroid responsive encephalopathy associated with autoimmune thyroiditis" because of this condition's responsiveness to corticosteroids -greater than 90% of patients in some reviews [3,4] . HE can present with several neurological manifestations and disease courses that can cause initial diagnostic confusion.…”

Section: Introductionmentioning

confidence: 99%

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A case of refractory hashimoto’s encephalopathy demonstrating improvement with plasmapheresis

Nelson

Jassam

Taylor

2014

CRIM

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Hashimoto's encephalopathy (HE) can present with multiple neurologic manifestations and various neuroimaging findings. While its progressive form is often well-recognized, patients with a waxing and waning course may provide diagnostic difficulty. Treatment with corticosteroids has been well-documented, but the benefit of immune-related therapies such as plasmapheresis has not been solidified. Here we describe a patient with waxing and waning signs and symptoms as well as significant fluctuations in neuroimaging findings that initially caused uncertainty regarding her diagnosis. After several largely negative tests including two brain biopsies, she was found to have high levels of anti-thyroid antibodies. Though sometimes found in disorders such as Hashimoto's thyroiditis and Graves' disease, these laboratory values in the setting of unremarkable thyroid function tests and her symptoms and their initial responsiveness to corticosteroids helped solidify her diagnosis as HE. However, due to refractory symptoms and signs, this patient required plasmapheresis. We demonstrate clinical, serological, and radiological improvement with this yet unconfirmed therapy, thus raising questions about the appropriateness of the term "steroid-responsive encephalopathy".

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Section: Discussionsupporting

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A case of refractory hashimoto’s encephalopathy demonstrating improvement with plasmapheresis

Nelson

Jassam

Taylor

2014

CRIM

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The Uncertain Nosology of Hashimoto Encephalopathy

Schott¹,

Warren²,

Rossor³

2003

Arch Neurol

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We welcome the review of Hashimoto encephalopathy (HE) by Chong et al 1 and share their concern regarding the indiscriminate use of this poorly defined term. There is sparse evidence for the pathogenicity of antithyroid antibodies in this condition: HE has been associated with variable titers of either thyroglobulin or microsomal antibodies, and these antibodies have different antigenic targets. We agree that HE is likely to represent a heterogeneous group of disorders, probably due to a variety of autoimmune causes, with thyroid antibody elevation being a bystander effect. It is interesting to speculate whether some of the patients might have had the recently described encephalopathy associated with voltage-gated potassium channel antibodies. 2,3Furthermore, not all cases that would meet the criteria of Chong et al 1 for HE have an autoimmune etiology. We cared for a previously healthy 62-year-old woman who had a 4-month history of progressive cognitive and behavioral decline accompanied by extrapyramidal signs and multifocal myoclonus. The level of thyroid-stimulating hormone was mildly raised (10.8 mIU/L; range, 0.3-5.0 mIU/L), and antithyroid microsomal antibodies were present in high titers (1:102400). The cerebrospinal fluid was acellular with no evidence of bacterial or viral infection. There was no response to an empirical trial of steroid therapy, and the patientsubsequentlydied.Postmortemexaminationofthebrain confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease with no evidence of inflammation. This case illustrates the importance of considering histopathologic examination in suspected HE cases, both to exclude alternative diagnoses and to further our understanding of this complex entity. The significance of lymphocytic perivascular infiltration, a nonspecific finding in cerebral vasculitis, remains uncertain in thiscondition. 4 Whereasempiricaluseofcorticosteroidsmay produce clinical improvement in some cases labeled as HE, such treatment is not without its risks and may obscure alternative diagnoses. We suggest that the term Hashimoto encephalopathy be abandoned because implications of causality, which have not been conclusively proved, will remain while this term is in use.

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What's in a NAIM?

Chong¹,

Rowland²

2006

Arch Neurol

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Hashimoto Encephalopathy

Cited by 522 publications

References 56 publications

A case of refractory hashimoto’s encephalopathy demonstrating improvement with plasmapheresis

A case of refractory hashimoto’s encephalopathy demonstrating improvement with plasmapheresis

The Uncertain Nosology of Hashimoto Encephalopathy

What's in a NAIM?

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