Sakurai Takeo, Tanaka Yuji, Koumura Akihiro, Hayashi Yuichi, Kimura Akio, Hozumi Isao, Yoneda Makoto, Inuzuka Takashi
Department of Neurology and Geriatrics, Division of Neuroscience, Research Field of Medical Sciences, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan.
Brain Nerve. 2008 May;60(5):559-65.
A 79-year-old female was admitted to our hospital because of unconsciousness and convulsion following mental deterioration. On admission, she exhibited myoclonic movement of the right side of the face and right fingers in addition to rigospasticity and tremors in the right arm and leg. Laboratory tests revealed hyperthyroidism with an increased anti-TSH-R antibody titer. In addition, an echogram indicated excessive blood flow at the thyroid; hence, the patient was diagnosed with Basedow's disease. Interestingly, the tests also revealed increased titer of anti-TPO antibody, anti-Tg antibody, and anti-NH2 terminal of alpha-enolase (NAE) antibody; in addition, an EEG showed abnormal findings potentially indicating periodic synchronous discharge. Brain MRI showed cerebral atrophy, and brain 99mTc-ECD-SPECT images demonstrated an overall decrease in the accumulation of 99mTc in the cerebrum. The abovementioned findings are common to patients with Creutzfeldt-Jakob disease (CJD). We initiated treatment for hyperthyroidism with thiamazole and lugol, but this did not regain consciousness. Because she had anti-thyroid antibody was observed, we considered a differential diagnosis of Hashimoto's encephalopathy and, in fact, methylprednisolone pulse therapy alleviated her symptoms and normalized the EEG findings. The condition in this case clinically mimicked CJD; therefore, the differentiated diagnosis is important because Hashimoto's encephalopathy is treatable disease.
一名79岁女性因精神衰退后出现意识丧失和抽搐而入住我院。入院时,她除了右臂和右腿有强直痉挛和震颤外,还表现出右侧面部和右手手指的肌阵挛运动。实验室检查显示甲状腺功能亢进,抗促甲状腺激素受体(TSH-R)抗体滴度升高。此外,超声心动图显示甲状腺血流过多;因此,该患者被诊断为突眼性甲状腺肿。有趣的是,检查还显示抗甲状腺过氧化物酶(TPO)抗体、抗甲状腺球蛋白(Tg)抗体和抗α-烯醇化酶(NAE)抗体氨基末端的滴度升高;此外,脑电图显示有异常发现,可能提示周期性同步放电。脑部磁共振成像(MRI)显示脑萎缩,脑部99m锝-双半胱乙酯(99mTc-ECD)单光子发射计算机断层扫描(SPECT)图像显示大脑中99mTc的积聚总体减少。上述发现是克雅氏病(CJD)患者的常见表现。我们开始用甲巯咪唑和卢戈氏碘液治疗甲状腺功能亢进,但这并未使患者恢复意识。由于观察到她有抗甲状腺抗体,我们考虑鉴别诊断为桥本脑病,事实上,甲泼尼龙冲击疗法缓解了她的症状并使脑电图结果恢复正常。该病例的病情在临床上类似于CJD;因此,鉴别诊断很重要,因为桥本脑病是一种可治疗的疾病。