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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. 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.

journal_name

J Neurol Sci

journal_title

Journal of the neurological sciences

authors

Lee CH,Lin JJ,Lin KJ,Chang BL,Hsieh HY,Chen WH,Lin KL,Fung HC,Wu T

doi

10.1016/j.jns.2014.03.043

subject

Has Abstract

pub_date

2014-06-15 00:00:00

pages

36-40

issue

1-2

eissn

0022-510X

issn

1878-5883

pii

S0022-510X(14)00202-0

journal_volume

341

pub_type

杂志文章

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