Prevalence and immunological spectrum of temporal lobe epilepsy with glutamic acid decarboxylase antibodies

Prevalence and immunological spectrum of temporal lobe epilepsy with glutamic acid decarboxylase antibodies
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DOI:
10.1111/j.1468-1331.2011.03609.x
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发表时间:
2012-06-01
影响因子:
5.1
通讯作者:
Graus, F.
Graus, F.
中科院分区:
医学3区
文献类型:
--
作者:
Falip, M.;Carreno, M.;Graus, F.

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背景和目的:谷氨酸脱羧酶(GAD)-ab的高水平最初被描述为僵人综合征患者,此后也在其他神经系统疾病的患者中观察到。颞叶癫痫(TLE)似乎是一种特殊的疾病。我们的目的是描述GAD-ab在TLE患者中的患病率,并描述高GAD-ab水平的TLE患者的临床免疫学特征。方法:对一系列TLE患者进行GAD-ab、抗核抗体、抗DNA抗体、抗心磷脂抗体、抗转谷氨酰胺酶抗体和抗甲状腺抗体检测。由于成年期是TLE+海马区硬化症和热性惊厥中最不常见的发病,我们选择了发病年龄在30岁以后的患者,以扩大病因的范围。患者分为两组:已知病因组19例(45%),病因不明组23例(55%)。临床免疫学研究包括GAD-ab水平高的TLE患者(>1000IU)。结果:42例患者中,5例血清GAD-ab阳性(15211~963IU/ml),均来自不明原因组。因此,在不明病因组中,GAD-ab水平阳性者占21.7%,高达8.7%。免疫档案研究包括9名患者(7名耐药患者),其中6名为女性(66%),平均年龄41岁。报告了3名患者的急性首发症状,4名(44%)胰岛素依赖型糖尿病,5名(55%)其他伴随的自身免疫性疾病,4名(44%)记忆障碍和4名中到重度情绪障碍。鞘内合成GAD-ab的患者有7例(77%)。结论:伴有GAD-ab的颞叶癫痫并不少见。在高滴度的患者亚组中,这种癫痫通常是耐药的,并与记忆障碍、抑郁和其他自身免疫性疾病有关。
Background and purpose: High levels of glutamic acid decarboxylase (GAD)-ab were initially described in patients with stiff person syndrome, and have since also been observed in patients with other neurological diseases. Temporal lobe epilepsy (TLE) seems to be specially associated. Our purpose is to describe the prevalence of GAD-ab in patients with TLE, and to characterize the clinical-immunological profile of TLE patients with high levels of GAD-ab. Methods: An immunological profile including GAD-ab and antinuclear, anti-DNA, anti-cardiolipin, anti-transglutaminase and antithyroid antibodies was determined in a consecutive series of patients with TLE. As adulthood onset is the least common onset in TLE + hipocampal sclerosis and febrile seizures, we selected patients whose onset was after 30 years of age, to expand the spectrum of aetiologies. Patients were divided into two groups: known aetiology, 19 patients (45%) and unknown aetiology, 23 (55%). The clinicalimmunological study included TLE patients with high GAD-ab levels (>1000 IU). Results: Amongst 42 patients, serum GAD-ab levels were positive in 5 (15211 963 IU/ml), all from the unknown aetiology group. Thus, GAD-ab levels were positive in 21.7% and high in 8.7% of the unknown aetiology group. The immunological profile study included nine patients (seven pharmacoresistant), of whom six were women (66%) with mean age 41 years. Three patients reported acute debut, four (44%) insulin-dependent diabetes mellitus, five (55%) other concomitant autoimmune diseases, four (44%) memory impairment and four moderate-to-severe mood disturbance. Intrathecal synthesis of GAD-ab was observed in seven patients (77%). Conclusions: Temporal lobe epilepsy with GAD-ab is not a rare condition. In the subgroup of patients with high titres, this epilepsy is often pharmacoresistant and associated with memory impairment, depression and other autoimmune diseases.