A Patient with Probable Dementia with Lewy Bodies and Positive Autoantibodies against the Anti-NH2-terminal of α-Enolase Takahiro Ikura 1 , Hiroshige Fujishiro 1,2 , Yukitoshi Takahashi 3 , Makoto Yoneda 4 , Tomoyuki Saito 1 , Yuhei Chiba 1 , Ayuko Kamada 1 , Omi Katsuse 1 , Yoshio Hirayasu 1 1Department of Psychiatry, Yokohama City University School of Medicine 2Yokohama Maioka Hospital 3Shizuoka Institute of Epilepsy and Neurological Disorders 4School of Nursing, Fukui Prefectural University Keyword: 橋本脳症 , レヴィ小体型認知症 , 抗αエノラーゼN末端抗体 , 抗グルタミン酸受容体抗体ε2 , 抗グルタミン酸受容体抗体δ2 , Hashimoto's encephalopathy , dementia with Lewy bodies , anti-NH2-terminal of α-enolase autoantibodies , anti-glutamate receptor ε2 subunit antibodies , anti-glutamate receptor δ2 subunit antibodies pp.967-972
Published Date 2015/7/1
DOI https://doi.org/10.11477/mf.1416200239
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Dementia with Lewy bodies (DLB) is clinically characterized by progressive dementia that is frequently accompanied by neurological and psychiatric manifestations. Hashimoto's encephalopathy (HE) is a rare autoimmune disease with neurological and psychiatric manifestations that is not well understood. However, this disease has attracted growing attention as a treatable dementia. Although autoimmune mechanisms are thought to play a pathogenic role in HE, the etiology of the disease remains unclear. Recently, it was reported that the serum in patients with HE is frequentry positive for autoantibodies against the anti-NH2-terminal of α-enolase (anti-NAE), indicating a useful serological diagnostic marker for HE.

We report the case of an 81-year-old Japanese woman with probable DLB and hypothyroidism. In her serum, elevated anti-thyroid antibodies and positive autoantibodies against anti-NAE were observed. Elevated levels of anti-glutamate receptor ε2 subunit (GluRε2) antibodies were also detected in her cerebrospinal fluid. Because her clinical condition became stable after treatment with cholinesterase inhibitor, levodopa, and levothyroxine, immunotherapy was not performed. Although the relationship between autoimmunity and cognitive decline in this patient was unclear, the present observations suggest the coexistence of neurodegeneration and autoimmunity as the underlying pathogenic mechanism.

(Received March 3, 2014; Accepted February 17, 2015; Published July 1, 2015)

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