Serial brain MRI changes related to autoimmune pathophysiology in Hashimoto encephalopathy with anti-NAE antibodies: A case-series study

Matsunaga, Akiko; Ikawa, Masamichi; Kawamura, Yasutaka; Kishitani, Toru; Yamamura, Osamu; Hamano, Tadanori; Kimura, Hirohiko; Nakamoto, Yasunari; Yoneda, Makoto

doi:10.1016/j.jns.2019.116453

Cited by 15 publications

(20 citation statements)

References 28 publications

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“…Neuroimaging findings similar to those in the present case, i.e. nonspecific scattered increased signals in the subcortical white matter on brain MRI and focal hypoperfusion in the area corresponding to the patient's clinical symptoms on brain perfusion SPECT, may also be observed in patients with Hashimoto encephalopathy (16,17).…”

Section: Discussionsupporting

confidence: 84%

Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH<sub>2</sub>-terminal of α-enolase

et al. 2021

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We herein report a case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis concurrent with NH 2-terminal of α-enolase (NAE) antibodies. A 36-year-old Japanese woman presented with Gerstmann's syndrome followed by jerky involuntary movements, seizure, autonomic instability, and consciousness disturbance. NAE antibodies were detected in the serum; however, NMDAR antibodies were identified in the cerebrospinal fluid with a cell-based assay, confirming the diagnosis of anti-NMDAR encephalitis. This case highlights the fact that Gerstmann's syndrome can be a manifestation of anti-NMDAR encephalitis and that NAE may be identified concurrently with NMDAR antibodies, suggesting that the diagnosis of Hashimoto encephalopathy requires the reasonable exclusion of alternative diagnoses, including anti-NMDAR encephalitis.

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Section: Discussionsupporting

confidence: 84%

Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH<sub>2</sub>-terminal of α-enolase

et al. 2021

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“…However, several case reports have also shown reversible white matter lesions or DWI high‐intensity spots in subcortical regions in HE patients, suggesting that cerebral vasculitis is associated with white matter lesions that are apparently non‐specific. Furthermore, we recently demonstrated that serial MRI studies showed expanded and diminished white matter lesions and emerging subcortical high‐intensity spots on DWI . Interestingly, these varying MRI lesions worsened at relapse and improved after recovery after immunotherapies.…”

Section: Neuroimagingmentioning

confidence: 95%

“…Furthermore, we recently demonstrated that serial MRI studies showed expanded and diminished white matter lesions and emerging subcortical high-intensity spots on DWI. 38 Interestingly, these varying MRI lesions worsened at relapse and improved after recovery after immunotherapies.…”

Section: Neuroimagingmentioning

confidence: 99%

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Hashimoto encephalopathy

Matsunaga

Ikawa

Yoneda

2019

Clinical & Exp Neuroim

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Hashimoto encephalopathy (HE) is an autoimmune encephalopathy associated with autoimmune chronic thyroiditis. The clinical entity and nosology of HE have long been debated. Recently, new autoantibodies associated with autoimmune encephalitis have been discovered. With accumulated reported cases, and our discovery of serum autoantibody against NH2‐terminal of alpha‐enolase as a specific diagnostic marker, HE has been recognized as a distinct clinical entity. The condition appears dominant in females. The clinical features of HE are characterized by various neuropsychiatric symptoms, such as altered consciousness, psychosis and seizures, the presence of serum anti‐thyroid antibodies, normal or non‐specific changes in brain magnetic resonance imaging, diffuse slow wave activities in electroencephalogram and responsiveness to immunotherapy. A few pathological studies showed a lymphocytic infiltration of the small and medium‐sized vessels of the brain, which suggested vasculitis. Non‐specific neurological symptoms, laboratory findings and neuroimaging of HE make its diagnosis an oversight and difficult, whereas antibodies against NH2‐terminal of alpha‐enolase can be useful as a diagnostic biomarker for HE. In this review, we describe the current state of knowledge about HE and discuss progress in the diagnosis of HE.

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“…Hashimoto encephalopathy (HE), typically presents with a range of symptoms such as cognitive impairment, convulsions, confusion, speech disorder, and delusions [1,2]. HE is associated with the presence of antithyroid peroxidase (TPO) and/or anti-thyroglobulin antibodies, and clinical improvement with steroid and immunotherapy is often seen [2][3][4][5]. Existing literature on MRI findings of HE reports predominantly normal findings or non-specific white matter changes and infrequently mesial temporal involvement [1,3,4,6,7].…”

Section: Introductionmentioning

confidence: 99%