Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures.
Basal ganglia T1 hyperintensity in LGI1-autoantibody faciobrachial dystonic seizures.
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DOI：
10.1212/nxi.0000000000000161
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发表时间：
2015-12
期刊：
影响因子：
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通讯作者：
Pittock SJ
中科院分区：
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作者：
Flanagan EP;Kotsenas AL;Britton JW;McKeon A;Watson RE;Klein CJ;Boeve BF;Lowe V;Ahlskog JE;Shin C;Boes CJ;Crum BA;Laughlin RS;Pittock SJ
探讨富含亮氨酸胶质瘤失活1（LGI 1）自身抗体（Ab）引起的面臂肌张力障碍性癫痫发作（FBDS）的临床特征和MRI异常。通过检索2002年1月1日至2015年6月1日的临床和血清学数据库，回顾性确定了48例LGI 1-Ab脑病患者。其中，26例符合该病例系列的入选标准：LGI 1-Ab血清阳性和FBDS。在对最初确定的所有48例患者进行的单独分析中，由2名对临床细节不知情的神经放射科医生比较了有（n = 26）和无（n = 22）FBDS患者的MRI。纳入的26例患者的中位年龄为62.5岁（范围37-78岁）; 65%为男性。FBDS涉及手臂（26）、面部（22）和腿部（12）。其中10人曾被诊断为心因性。接受评估的23名患者中，有20名的发作期脑电图正常。在11例患者（42%）中检测到基底节T1和T2信号异常，神经放射学家之间的一致性非常好（κ评分分别为0.86和0.93），包括仅T1高信号（2），仅T2高信号（1）或两者兼而有之（8）。T1高信号持续时间长于T2高信号（中位数11周vs 1周，p = 0.02）。免疫治疗（18/18）的改善比抗癫痫药物（10/24）更频繁。对所有48例最初被确定为LGI 1-Ab脑病的患者进行的单独分析显示，26例FBDS患者中有11例存在基底节MRI异常，但在无FBDS的患者中不存在（0/22）（p < 0.001）。相反，近中颞部MRI异常在FBDS患者中（42%）比无FBDS患者（91%）更少见（p < 0.001）。基底节T1高信号是LGI 1-Ab FBDS的临床有用MRI生物标志物，提示基底节定位。
To characterize the clinical features and MRI abnormalities of leucine-rich glioma-inactivated 1 (LGI1)-autoantibody (Ab) faciobrachial dystonic seizures (FBDS). Forty-eight patients with LGI1-Ab encephalopathy were retrospectively identified by searching our clinical and serologic database from January 1, 2002, to June 1, 2015. Of these, 26 met inclusion criteria for this case series: LGI1-Ab seropositivity and FBDS. In a separate analysis of all 48 patients initially identified, the MRIs of patients with (n = 26) and without (n = 22) FBDS were compared by 2 neuroradiologists blinded to the clinical details. The median age of the 26 included patients was 62.5 years (range 37–78); 65% were men. FBDS involved arm (26), face (22), and leg (12). Ten were previously diagnosed as psychogenic. Ictal EEGs were normal in 20 of 23 assessed. Basal ganglia T1 and T2 signal abnormalities were detected in 11 patients (42%), with excellent agreement between neuroradiologists (κ scores of 0.86 and 0.93, respectively), and included T1 hyperintensity alone (2), T2 hyperintensity alone (1), or both (8). The T1 hyperintensities persisted longer than the T2 hyperintensities (median 11 weeks vs 1 week, p = 0.02). Improvement with immunotherapy (18/18) was more frequent than with antiepileptic medications (10/24). A separate analysis of all 48 patients initially identified with LGI1-Ab encephalopathy showed that basal ganglia MRI abnormalities were present in 11 of 26 with FBDS but not present in those without FBDS (0/22) (p < 0.001). In contrast, mesial temporal MRI abnormalities were less common among those with FBDS (42%) than those without (91%) (p < 0.001). Basal ganglia T1 hyperintensity is a clinically useful MRI biomarker of LGI1-Ab FBDS and suggests a basal ganglia localization.