Modignani R L, Venegoni M, Beretta F, Fassina S
Divisione Medicina I, Ospedale Fatebenefratelli e Oftalmico, Milano.
Ann Ital Med Int. 1992 Oct-Dec;7(4):250-4.
A 41-year-old woman is described, first hospitalized in the neurosurgical department for a transient ischemic attack with left hemiparesis followed after 6 hours by tonic-clonic seizures starting from the left hemiface and quickly generalized. Brain computed tomography (CT) scan and magnetic resonance imaging were normal. Clinically the patient presented tremor, tachycardia, generalized muscle weakness, and profuse diaphoresis. T4 and T3 were elevated. The patient was transferred from the neurosurgical to the medical department where a thyroid storm due to autoimmune Graves' disease with normal thyrotropin (TSH) values responsive to thyrotropin-releasing hormone (TRH) stimulation was diagnosed. A syndrome of inappropriate secretion of TSH was suspected in an unusual presentation as autoimmune Graves' hyperthyroidism. The TSH alpha-subunit and alpha-subunit/TSH molar ratio were normal, which supported the diagnosis of non-neoplastic inappropriate secretion of TSH. However, severe autoimmune Graves' hyperthyroidism is very rare indeed because autoantibodies to thyroid antigens are generally non-detectable in such patients. Our patient was treated initially with barbiturates, then with dexamethasone, Lugol's solution, methimazole and propranolol. Treatment of this patient proved difficult, and definitive improvement was obtained only after triiodothyroacetic acid administration, but methimazole and propranolol administration could not be discontinued. Fine needle aspiration biopsies of the thyroid in 2 occasions showed follicular or follicular-papillary proliferation with lymphocytic infiltration as in chronic thyroiditis. The patient is now in good clinical conditions and is followed up regularly. Autoimmune Graves' hyperthyroidism may be associated in extremely rare instances with non neoplastic inappropriate secretion of TSH.
本文描述了一名41岁女性，她最初因短暂性脑缺血发作伴左侧偏瘫入住神经外科，6小时后出现从左侧面部开始并迅速泛化的强直阵挛性癫痫发作。脑部计算机断层扫描（CT）和磁共振成像均正常。临床上，患者表现为震颤、心动过速、全身肌肉无力和大量出汗。T4和T3升高。患者从神经外科转至内科，被诊断为自身免疫性格雷夫斯病导致的甲状腺风暴，促甲状腺激素（TSH）值正常，对促甲状腺激素释放激素（TRH）刺激有反应。怀疑存在一种不常见的促甲状腺激素分泌异常综合征，表现为自身免疫性格雷夫斯甲亢。TSHα亚基和α亚基/TSH摩尔比正常，这支持了非肿瘤性促甲状腺激素分泌异常的诊断。然而，严重的自身免疫性格雷夫斯甲亢确实非常罕见，因为此类患者通常检测不到甲状腺抗原自身抗体。我们的患者最初接受巴比妥类药物治疗，然后使用地塞米松、卢戈氏液、甲巯咪唑和普萘洛尔。该患者的治疗证明很困难，仅在给予三碘甲状腺乙酸后才获得明确改善，但甲巯咪唑和普萘洛尔的给药不能停止。两次甲状腺细针穿刺活检显示为滤泡或滤泡乳头样增生伴淋巴细胞浸润，如同慢性甲状腺炎。患者目前临床状况良好，正在定期随访。自身免疫性格雷夫斯甲亢在极其罕见的情况下可能与非肿瘤性促甲状腺激素分泌异常有关。
