Anti-neuronal autoantibody in Hashimoto's encephalopathy: neuropathological, immunohistochemical, and biochemical analysis of two patients

• Published in: Journal of the neurological sciences Vol. 217; no. 1; pp. 7 - 12
• Main Authors: Oide, Takashi, Tokuda, Takahiko, Yazaki, Masahide, Watarai, Megumi, Mitsuhashi, Shigeaki, Kaneko, Kazuma, Hashimoto, Takao, Ohara, Shinji, Ikeda, Shu-ichi
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 15.01.2004; Elsevier Science
• Subjects: Animals; Autoantibodies - blood; Autoantibodies - immunology; Autoantibody; Autoimmune; Biological and medical sciences; Blotting, Western - methods; Brain Diseases - blood; Brain Diseases - etiology; Brain Diseases - immunology; Hashimoto's encephalopathy; Humans; Immunoglobulin G - blood; Immunoglobulin G - immunology; Immunohistochemistry - methods; Male; Medical sciences; Mice; Mice, Inbred BALB C; Middle Aged; Neurology; Neurons - immunology; Thyroiditis; Thyroiditis, Autoimmune - blood; Thyroiditis, Autoimmune - complications; Thyroiditis, Autoimmune - immunology; Thyroiditis; Autoantibody; Autoimmune; Hashimoto's encephalopathy; Human; Immunohistochemistry; Anatomic pathology; Nervous system diseases; Biochemical analysis
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2003.08.005

Hashimoto's encephalopathy (HE) is thought to be caused by disorders of immune mechanisms. Although immunologically mediated central nervous system vasculitis or unidentified anti-neuronal autoantibodies have been suspected of causing HE, its pathogenesis is still unclear. For the study presented here, two patients with typical clinical and laboratory/electrophysiological findings of HE were analyzed to clarify the role of anti-neuronal autoantibodies in the pathogenesis of HE. The autopsied brain of one of the patients was histopathologically examined. For Western blotting analysis and immunohistochemistry, serum and purified immunoglobulin G obtained from the other patient were used. Autopsy revealed no evidence of central nervous system vasculitis or other abnormal findings in the brain. The patient's serum contained an anti-neuronal autoantibody that immunohistochemically labeled neurons of mouse and human cerebral cortices and reacted with the 36-kDa antigenic protein present in a soluble fraction obtained from human cerebral cortex. Our results indicate that anti-neuronal autoantibodies may be associated with the pathogenesis of HE.