Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria
Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria
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DOI：
10.1093/brain/awt285
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发表时间：
2014-01-01
期刊：
影响因子：
14.5
通讯作者：
Jacobs, Bart Casper
中科院分区：
文献类型：
--
作者：
Fokke, Christiaan;van den Berg, Bianca;Jacobs, Bart Casper
格林-巴利综合征是一种急性多发性神经根神经病，临床表现多样。准确的诊断标准对于患者护理和研究（包括临床试验和疫苗安全性研究）至关重要。已经提出了几种吉兰-巴利综合征的诊断标准，包括布莱顿合作组织最近制定的标准。在本研究中，我们详细描述了在 494 名格林-巴利综合征成年患者的研究人群中满足这些布莱顿标准所需的关键诊断特征，这些患者先前已纳入治疗和观察性研究。患者的中位年龄为 53 岁（四分位数范围 36-66 岁），男性略占多数（56%）。所有患者均出现双侧肢体无力，通常累及上肢和下肢。 6% 的患者的无力仍然局限于腿部，1% 的患者则局限于手臂。最初，91% 的患者以及随访期间的所有患者均发现瘫痪手臂或腿部的反射减弱。然而，十名（2%）患者的麻痹手臂仍表现出持续的正常反射。 80% 的患者在 2 周内达到疾病最低点，97% 的患者在 4 周内达到疾病最低点，所有患者在 6 周内达到疾病最低点。 95%的患者出现单相病程，其中10%出现与治疗相关的波动。 23 名 (5%) 患者在出现无力 8 周后出现临床恶化。对 474 名 (96%) 患者进行了脑脊液检查。 15% 的细胞出现轻度细胞增多（5 至 50 个细胞/μl），且没有超过 50 个细胞/μl。仅 64% 的患者发现脑脊液蛋白浓度升高，高度依赖于无力发作后腰椎穿刺的时间（第一天为 49%，两周后为 88%）。神经电生理学结果与 99% 的患者存在神经病变相一致，但只有 59% 的患者符合格林-巴利综合征独特亚型的当前标准。拥有完整数据集的患者 (335) 根据 Brighton 标准进行分类，诊断确定性从高到低不等，其中 61% 为 1 级，33% 为 2 级，无 3 级患者，6% 为 4 级患者。分类在这些级别的患者在发生过既往事件、初始临床表现或结果的患者比例方面没有差异。在当前队列研究中观察到的吉兰-巴利综合征关键诊断特征的变异性可用于提高诊断标准的敏感性。
Guillain-Barre syndrome is an acute polyradiculoneuropathy with a variable clinical presentation. Accurate diagnostic criteria are essential for patient care and research, including clinical trials and vaccine safety studies. Several diagnostic criteria for Guillain-Barre syndrome have been proposed, including the recent set by the Brighton Collaboration. In the present study we describe in detail the key diagnostic features required to meet these Brighton criteria in a study population of 494 adult patients with Guillain-Barre syndrome, previously included in therapeutic and observational studies. The patients had a median age of 53 years (interquartile range 36-66 years) and males slightly predominated (56%). All patients developed bilateral limb weakness which generally involved both upper and lower extremities. The weakness remained restricted to the legs in 6% and to the arms in 1% of the patients. Decreased reflexes in paretic arms or legs were found initially in 91% of patients and in all patients during follow-up. Ten (2%) patients however showed persistence of normal reflexes in paretic arms. Disease nadir was reached within 2 weeks in 80%, within 4 weeks in 97% and within 6 weeks in all patients. A monophasic disease course occurred in 95% of patients, of whom 10% had a treatment-related fluctuation. A clinical deterioration after 8 weeks of onset of weakness occurred in 23 (5%) patients. Cerebrospinal fluid was examined in 474 (96%) patients. A mild pleocytosis (5 to 50 cells/mu l) was found in 15%, and none had more than 50 cells/mu l. An increased cerebrospinal fluid protein concentration was found only in 64% of patients, highly dependent on the timing of the lumbar puncture after onset of weakness (49% at the first day to 88% after 2 weeks). Nerve electrophysiology was compatible with the presence of a neuropathy in 99% of patients, but only 59% fulfilled the current criteria for a distinct subtype of Guillain-Barre syndrome. Patients with a complete data set (335) were classified according to the Brighton criteria, ranging from a high to a low level of diagnostic certainty, as level 1 in 61%, level 2 in 33%, level 3 in none, and level 4 in 6% of patients. Patients categorized in these levels did not differ with respect to proportion of patients with preceding events, initial clinical manifestations or outcome. The observed variability in the key diagnostic features of Guillain-Barre syndrome in the current cohort study, can be used to improve the sensitivity of the diagnostic criteria.