Caspr2 antibody limbic encephalitis is associated with Hashimoto thyroiditis and thymoma

Abstract Background Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms....

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Bibliographic Details
Published in:Journal of the neurological sciences 2014-06, Vol.341 (1), p.36-40
Main Authors: 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
Format: Article
Language:English
Subjects:
Aged
Antigens, Nuclear - immunology
Autoantibodies - blood
Autoantibodies - cerebrospinal fluid
Autoimmune encephalitis
Brain - diagnostic imaging
Brain - pathology
Case-Control Studies
Caspr2
Cell Line, Transformed
Female
Fluorodeoxyglucose F18
Follow-Up Studies
Hashimoto Disease - complications
Hashimoto thyroiditis
HEK293 Cells
Humans
Limbic Encephalitis - complications
Limbic Encephalitis - diagnostic imaging
Limbic Encephalitis - drug therapy
Limbic Encephalitis - metabolism
Magnetic Resonance Imaging
Membrane Proteins - immunology
Nerve Tissue Proteins - immunology
Neurology
Neuronal surface antibodies
Positron-Emission Tomography
Thymoma
Thymoma - complications
Thyroxine
Time Factors
Transfection
VGKC
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Summary:Abstract Background Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be found in these patients, but Hashimoto thyroiditis (HT) has not been reported. Methods A 76-year-old woman presented with sub-acute-onset changes in mental status. Further investigations revealed thymoma and HT. The presence of NSAb was tested by immunofluorescence on human embryonic kidney-293 cells. Treatment included corticosteroids, azathioprine, thyroxine, plasmapheresis, and thymectomy. Results Caspr2 antibody was positive in serum but absent in CSF. Brain magnetic resonance imaging (MRI) showed diffuse cortical atrophy, but did not change significantly after treatments. Brain positron emission tomography (PET) revealed diffuse hypometabolism over the cerebral cortex. The patient's mental status only partially improved. Conclusions In Caspr2 antibody-associated syndromes, thymoma can occur in patients presenting only with LE, and HT can be an accompanying disease. Brain MRI and PET may not show specific lesions in limbic area. Patients with Caspr2 antibodies and thymoma may not have good prognosis.
ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2014.03.043