Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of Hashimoto's encephalopathy successfully treated with oral steroid therapy, resistant to high-dose methylprednisolone, plasma exchange and intravenous immunoglobulin

Naoki Tokuda, M.D.1)2), Keisuke Imai, M.D.2), Takashi Kasai, M.D., Ph.D.1), Ayaka Kimura, M.D.1), Yoshinari Abe, M.D.3), Toshiyuki Tominaga, M.D., Ph.D.4), Kenji Fukui, M.D., Ph.D.3), Makoto Yoneda, M.D., Ph.D.5), Masanori Nakagawa, M.D., Ph.D.6) and Toshiki Mizuno, M.D., Ph.D.1)

1)Department of Neurology, Kyoto Prefectural University of Medicine, Graduate School of Medicine
2)Department of Neurology and Stroke Treatment, Kyoto First Red Cross Hospital
3)Department of Psychiatry, Kyoto Prefectural University of Medicine, Graduate School of Medicine
4)Health Management Doctor's Office (Mental Health), Salary, Personnel Health and Welfare Division, Kyoto Prefecture
5)Faculty of Nursing and Social Welfare Science/Department of Nursing Science, Graduate School of Nursing and Social Welfare Sciences, Fukui Prefectural University
6)North Medical Center, Kyoto Prefectural University of Medicine, Graduate School of Medicine

A 30-year-old woman was admitted to the first institution with subacutely progressive aphasia and depression. Despite of lacking conclusive evidence on magnetic resonance imaging, cerebrospinal fluid examination, or electroencephalogram, we tentatively diagnosed her disease as limbic encephalopathy due to its acute progression. Highdose methylprednisolone was started on admission. However, symptoms did not improve. To make matters worse, psychiatric symptoms, such as hallucinations and emotional incontinence, appeared on the same day. Additional treatment with plasma exchange and intravenous immunoglobulin administration was also ineffective. Therefore, we could not manage the patient in a general ward due to severe psychiatric symptoms. The patient was transferred to a psychiatric ward in the second institution. She received both psychiatric treatment and steroid therapy, including a second course of intravenous high-dose methylprednisolone, followed by long-term oral prednisolone. Her symptoms gradually improved. A final diagnosis of Hashimoto's encephalopathy was made based on the patient's clinical course and positive results for both serum anti-thyroid antibody and anti-NAE antibody. In our case, long-term oral steroid therapy under psychiatric treatment was effective for good outcome.
Full Text of this Article in Japanese PDF (557K)

(CLINICA NEUROL, 55: 737|742, 2015)
key words: anti-NAE antibody, Hashimoto's encephalopathy, intravenous immunoglobulin therapy, long-term oral steroid therapy, plasma exchange

(Received: 31-Mar-15)