Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma

Lee, Chih-Hong; Lin, Jainn-Jim; Lin, Kun‐Ju; Chang, Bao‐Luen; Hsieh, Hsiang‐Yao; Chen, Wei‐Hsun; Lin, Kuang-Lin; Fung, Hon-Chung; Wu, Tony

doi:10.1016/j.jns.2014.03.043

Cited by 17 publications

(8 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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“…Although LGI1-Ab was commonly agreed with no association with neoplasm, no agreement on the relation between CASPR2-Ab and neoplasm has been reached. Previous studies have shown CASPR2-Ab positive patients were more likely to be found with neoplasm especially thymomas, accompanied with acetylcholine receptor antibodies and myasthenia gravis (MG) [ 4 , 6 , 11 – 13 ]. And CASPR2 has been reported to function as a tumor suppressor gene in glioma by Bralten et al [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

A Chinese female Morvan patient with LGI1 and CASPR2 antibodies: a case report

et al. 2016

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BackgroundMorvan syndrome is a rare disorder characterized by the combination of peripheral nerve hyperexcitability, encephalopathy and dysautonomia with marked insomnia. It was reported to have association to antibodies to voltage-gated potassium channels including contactin associated protein-like 2 antibodies (CASPR2-Ab) and leucine-rich glioma inactivated protein 1 antibodies (LGI1-Ab). LGI1-Ab was reported to associate with seizures, amnesia, confusion, hyponatraemia and a good prognosis, while CASPR2-Ab with peripheral presentations, probable risk for tumor and a poor prognosis. The vast majority of Morvan syndrome patients were male, with normal magnetic resonance imaging of the brain.Case presentationWe report a female case presenting with a combination of bilateral leg pain, widespread myokymia, memory disturbance, seizure, hyperhidrosis and insomnia. She had antibodies targeting CASPR2 and LGI1, tested by the indirect immunofluorescence test, which demonstrated the diagnosis of typical Morvan syndrome as well as classical limbic encephalitis. Cranial MRI revealed bilateral hyper-intensity of the medial temporal lobe, insular lobe and basal ganglia on T2/FLAIR and DWI sequence. As the treatment carried on, her serum LGI1-Ab disappeared and her memory loss, seizure and confusion quickly relieved. But her peripheral presentations did not relieve until serum CASPR2-Ab turned negative. Intravenous immunoglobulin treatment showed limited efficacy while she achieved almost complete remission with corticosteroids therapy.ConclusionsThis case provides a rare female resource of Morvan syndrome, which is the first patient with both CASPR2-Ab and LGI1-Ab positive Morvan syndrome in China and one of the few female patients with Morvan syndrome reported so far. Through the detailed analysis of her clinical course, the diverse and overlapping clinical phenotype of CASPR2-Ab and LGI1-Ab in patients with Morvan syndrome was obvious and interesting.

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“…This is consistent with previous reports outlining the co-occurrence of these syndromes with thymoma. 27 Other autoimmune diseases including Lambert-Eaton myasthenic syndrome, encephalitis, autonomic neuropathy, StiffPerson syndrome have also been reported with thymoma, with or without MG. [27][28][29][30] It can be speculated that specific antigens related to these illnesses are expressed in thymoma, leading to aberrant T cell priming and subsequent specific autoimmunity.…”

Section: Discussionmentioning

confidence: 99%

A Case Series of Patients With Autoimmune Myasthenia Gravis in Association With Invasive Thymoma

McCombe

Raj

Henderson³

et al. 2016

Journal of Clinical Neuromuscular Disease

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Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma

Cited by 17 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

A Chinese female Morvan patient with LGI1 and CASPR2 antibodies: a case report

A Case Series of Patients With Autoimmune Myasthenia Gravis in Association With Invasive Thymoma

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