Anti-CASPR2 antibody associated encephalitis with anosmia and demyelinating pseudotumor: A case report

Xing, Xiang; Chen, Siliang; Zheng, Linmao; Feng, Wentao; Liu, Qing

doi:10.1016/j.jneuroim.2020.577393

Cited by 2 publications

(2 citation statements)

References 22 publications

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“…In contrast, these antibodies were detectable in both sera and CSF of the patients with pure CNS involvement ( 2 , 4 , 20 ), with the former exhibiting much higher antibody titers and detectable rate than the latter ( 2 , 17 , 20 ). In this study, both patients had positive anti-CASPR2 antibodies in serum but not in CSF, which is consistent with previous case reports ( 21 – 23 ). A similar pattern of antibody distribution was observed in other 11 patients with anti-CASPR2 antibody-associated autoimmune encephalitis from three centers from Northwest China, where all cases had positive serum antibodies but only five cases had positive CSF results (data not shown).…”

Section: Discussionsupporting

confidence: 93%

Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

Liu

Bai

et al. 2021

Front. Immunol.

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Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy. The classical lesions reported are located at the medial temporal lobe or hippocampus, whereas prominent brainstem lesions have not been addressed to date. Herein, we reported two patients mimicking progressive brainstem infarction with severe neurological manifestations. On brain magnetic resonance imaging (MRI), prominent brainstem lesions were noted, although multifocal lesions were also shown in the juxtacortical and subcortical white matters, basal ganglia, hippocampus, and cerebellar hemisphere. Unexpectedly and interestingly, both cases had detectable CASPR2 antibodies in sera, and an exclusive IgG1 subclass was documented in the further analysis. They were treated effectively with aggressive immunosuppressive therapies including corticosteroids, intravenous immunoglobulin G, and rituximab, with the first case achieving a rapid remission and the other undergoing a slow but gradual improvement. To the best of our knowledge, this is the first report on prominent brainstem involvement with definite MRI lesions in anti-CASPR2 antibody-associated autoimmune encephalitis, which helps to expand the clinical spectrum of this rare autoimmune disease and update the lesion patterns in the CNS.

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Section: Discussionsupporting

confidence: 93%

Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

Liu

Bai

et al. 2021

Front. Immunol.

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“…Studies based on MRI and positron emission tomography have identified the medial temporal lobe as the part of the central nervous system most vulnerable to anti-CASPR2 antibodies ( 6 , 8 , 9 , 14 , 15 ). However, some cases of abnormalities have been reported in the frontal, parietal, and occipital lobes, brainstem, cerebellar regions, basal ganglia, and the insula ( 6 , 9 , 16 , 17 ). Frontal lobe lesions have been detected by positron emission tomography in a 22-year-old woman with systemic lupus erythematosus ( 18 ) and a 61-year-old man without systemic disease ( 19 ), neither of whom showed abnormal brain MRI.…”

Section: Discussionmentioning

confidence: 99%

Case report: Autoimmune encephalitis associated with anti-CASPR2 antibody mimicking cerebral infarction

Chen

Tang²

2023

Front. Immunol.

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Autoimmune encephalitis associated with antibody against contactin-associated protein-like 2 (CASPR2) varies in its clinical presentation. The disease is difficult to distinguish from some other conditions without testing for anti-CASPR2 antibody in blood serum or cerebrospinal fluid. Cerebral lesions are typically detected by magnetic resonance imaging (MRI) in the medial temporal lobe or hippocampus. Here, we describe a patient with anti-CASPR2 antibody autoimmune encephalitis whose imaging manifestations mimicked infarction in the left frontal lobe. The 48-year-old man reported memory loss, convulsions, and disturbed consciousness one day after drinking wine. The right upper arm showed reduced autonomous movement after painful stimuli, and MRI showed abnormal hyperintensities in the left frontal lobe on T2 and fluid-attenuated inversion recovery sequences, restricted diffusion, and decreased cerebral blood flow, mimicking acute cerebral infarction. Contrast-enhanced T1-weighted MRI showed gyral enhancement involving the cortex and subcortical white matter. Computed tomography angiography did not identify culprit blood vessels. Symptoms did not improve with anti-platelet or lipid-lowering therapy. Screening for serum antibodies associated with autoimmune encephalitis detected antibody against CASPR2, and intravenous immunoglobulin therapy substantially improved symptoms. This case provides the first indication that anti-CASPR2 antibody-associated autoimmune encephalitis can manifest as involvement of the cortex and subcortical white matter in the frontal lobe based on MRI. It emphasizes the need for thorough investigation, including analysis of potential autoimmunity, of patients whose imaging findings mimic ischemic infarction.

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Anti-CASPR2 antibody associated encephalitis with anosmia and demyelinating pseudotumor: A case report

Cited by 2 publications

References 22 publications

Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

Case report: Autoimmune encephalitis associated with anti-CASPR2 antibody mimicking cerebral infarction

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