Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies

Kishitani, Toru; Matsunaga, Akiko; Ikawa, Masamichi; Hayashi, Kouji; Yamamura, Osamu; Hamano, Tadanori; Watanabe, Osamu; Tanaka, Keiko; Nakamoto, Yasunari; Yoneda, Makoto

doi:10.1097/md.0000000000006181

Cited by 32 publications

(22 citation statements)

References 27 publications

(47 reference statements)

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“…The other abnormalities reported were limbic lesions presenting with limbic symptoms (Fig. b) . These limbic lesions were improved by immunotherapies.…”

Section: Neuroimagingmentioning

confidence: 92%

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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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“…The other abnormalities reported were limbic lesions presenting with limbic symptoms (Fig. b) . These limbic lesions were improved by immunotherapies.…”

Section: Neuroimagingmentioning

confidence: 92%

“…3b). 40 These limbic lesions were improved by immunotherapies. Matsunaga et al noted that hyperperfusion was clearly detected in the right hippocampus on continuous arterial spin labeling imaging at the incipient stage.…”

Section: Neuroimagingmentioning

confidence: 99%

Hashimoto encephalopathy

Matsunaga

Ikawa

Yoneda

2019

Clinical & Exp Neuroim

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“…They developed a human brain proteome map using two-dimensional electrophoresis and applied it to the immunoscreening of brain proteins that reacted with serum antithyroid antibodies in Hashimoto's encephalopathy patients, identifying the novel antigen α-enolase, encoded on 1p36.23, as a candidate biomarker for Hashimoto's encephalopathy-related pathology and corticosteroid sensitivity. Kishitani and coworkers [3] extended the findings of Ochi and colleagues [116] noting anti-NH2-terminal of α-enolase antibodies in 24% of Hashimoto's encephalopathy patient sera, and limbic abnormalities on MRI demonstrating abnormal signal in unilateral or bilateral medial temporal lobes, and diffuse slow wave activity with epileptogenic discharges. These findings suggested that limbic encephalitis-associated with anti-NH 2 -terminal of α-enolase antibodies could be a possible manifestation of Hashimoto's encephalopathy in some cases.…”

Section: Immunopathogenic Mechanismsmentioning

confidence: 77%

Hashimoto’s Thyroiditis and Encephalopathy

Younger¹

2017

WJNS

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Although very rare, the literature describes cases of Hashimoto encephalopathy attesting to its occurrence. Affected patients present with seizures, stroke-like episodes, transient focal and global neurological deficits, and a variety of neuropsychiatric disturbance from dementia to hallucinations and psychosis. The encephalopathy evolves with concomitantly elevated anti-thyroid peroxidase antibodies, independent from hormonal thyroid function setting it apart from thyrotoxicosis and myxedema. A new patient reported with Hashimoto's encephalopathy, in whom neurocognitive impairment and stroke-like onset of hemiparesis and hemiparkinsonism correlated with brain magnetic resonance imaging fused with positron emission tomography.

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“…9,12 Additionally, studies have shown that some patients with encephalitis attributable to antineuronal antibodies also have antithyroid antibodies, although the clinical significance of the presence of both neuronal and thyroid autoimmunity remains uncertain. [12][13][14] A key finding in patients with Hashimoto encephalopathy is the remarkable response to steroid treatment. In one study of 251 patients with Hashimoto encephalopathy, more than 90% of patients had complete or near-complete recovery from symptoms following steroid treatment.…”

Section: Discussionmentioning

confidence: 99%

Anti–N-Methyl-D-Aspartate Receptor Encephalitis in a Patient With Antithyroid Peroxidase Antibodies and a Parotid Pleomorphic Adenoma

Sitterley¹,

Kleist²,

Bagert³

2019

TOJ

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Background: Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is an autoimmune disorder first described in 2007. It can cause severe neurologic and psychiatric symptoms, and patients often are misdiagnosed with a primary psychiatric disorder. Anti-NMDA-R encephalitis is an important differential diagnosis of psychosis, as the condition can be treated with the appropriate therapy. Case Report: A 42-year-old female with a history of hypothyroidism presented for evaluation of agitation and weakness. Six months prior to her presentation at our hospital, she had complained of headache, and neuroimaging showed a small right frontal hyperintensity on fluid-attenuated inversion recovery imaging. Workup was largely unremarkable, and she improved briefly with steroids at the outside institution. She then developed psychiatric symptoms and had 4 admissions to separate inpatient psychiatric facilities. She was tried on various antipsychotic medications and mood stabilizers with no improvement and lost 30 pounds in 3 months. When the patient was transferred to our facility, her neuroimaging was stable, and serum and cerebrospinal fluid were positive for antithyroid peroxidase and NMDA-R antibodies. She was treated with intravenous immunoglobulin (IVIG), highdose steroids, and rituximab. Positron emission tomography scan revealed a parotid mass that was resected and determined to be a pleomorphic adenoma. The patient improved remarkably to her baseline and was able to return to full duties at work within 6 months. In the 2 years since her initial admission, she has had minor relapses that have responded well to rituximab and IVIG. Conclusion: This case demonstrates a typical presentation of anti-NMDA-R encephalitis, as well as the challenges that can delay diagnosis. Symptoms vary and are often psychiatric, making these patients difficult to diagnose clinically, and they therefore require thorough investigation. This case illustrates the necessity to be inquisitive and to challenge the diagnosis when a clinical picture is not congruent because an accurate diagnosis is imperative in guiding appropriate treatment.

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Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies

Abstract: Supplemental Digital Content is available in the text

Cited by 32 publications

References 27 publications

Hashimoto encephalopathy

Hashimoto encephalopathy

Hashimoto’s Thyroiditis and Encephalopathy

Anti–N-Methyl-D-Aspartate Receptor Encephalitis in a Patient With Antithyroid Peroxidase Antibodies and a Parotid Pleomorphic Adenoma

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