Ruiz Marta, Puthenparampil Marco, Campagnolo Marta, Castellani Francesca, Salvalaggio Alessandro, Ruggero Susanna, Toffanin Elisabetta, Cacciavillani Mario, Gallo Paolo, Franciotta Diego, Briani Chiara
Department of Neurosciences (DNS), University of Padova, Padova, Italy.
Padova Neuroscience Center (PNC), Padova, Italy.
J Neurol Neurosurg Psychiatry. 2021 Sep;92(9):969-974. doi: 10.1136/jnnp-2020-325868. Epub 2021 Apr 13.
Cerebrospinal fluid (CSF) albumincytologic dissociation represents a supportive diagnostic criterion of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Few studies have investigated possible systemic or intrathecal humoral immune response activation in CIDP.Aim of our study was to investigate whether the search of oligoclonal IgG bands (OCBs) might provide additional data helpful in CIDP diagnostic work-up.
Forty-eight consecutive patients with CIDP (34 men, mean age 59.4, range 16-83) were recruited. CSF analysis included nephelometric measurement of albumin and IgG concentrations, calculation of Q, QAlb and intrathecal IgG synthesis, and OCBs detection with isoelectric focusing. Data were compared with those from CSF and serum of 32 patients with Guillain-Barré syndrome (GBS), 18 patients with anti-myelin associated glycoprotein (MAG) antibody neuropathy, 4 patients with multifocal motor neuropathy and 32 patients with non-inflammatory neuropathies (NINPs).
Patients with CIDP and anti-MAG antibody neuropathy had significantly higher CSF albumin concentrations and Q values than NINPs (p=0.0003 and p=0.0095, respectively). A total of 9 (19%) patients with CIDP presented identical serum and CSF OCBs ('mirror pattern') versus 3 patients (16.6%) with anti-MAG antibody neuropathy, 13 patients (40.6%) with GBS and 12.5% patients with NINPs. Only one patient with CIDP showed unique-to-CSF OCBs. First-line therapy was effective in 80.4% of patients with CIDP, irrespective of CSF findings.
Compared with NINP, CIDP, GBS and anti-MAG antibody neuropathies had a significantly increased CSF protein and blood-spinal nerve root barrier damage. Intrathecal humoral immune response is rare in our patients with CIDP. Systemic oligoclonal activation is more frequent, but not significantly different from what was detected in the control groups.
脑脊液(CSF)白蛋白细胞分离是慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)的一项支持性诊断标准。很少有研究调查CIDP中可能的全身或鞘内体液免疫反应激活情况。我们研究的目的是调查检测寡克隆IgG带(OCB)是否能为CIDP的诊断检查提供额外有用数据。
连续招募了48例CIDP患者(34例男性,平均年龄59.4岁,范围16 - 83岁)。CSF分析包括用比浊法测量白蛋白和IgG浓度、计算Q值、QAlb值和鞘内IgG合成,以及用等电聚焦法检测OCB。将数据与32例吉兰 - 巴雷综合征(GBS)患者、18例抗髓鞘相关糖蛋白(MAG)抗体神经病患者、4例多灶性运动神经病患者和32例非炎性神经病(NINP)患者的CSF和血清数据进行比较。
CIDP患者和抗MAG抗体神经病患者的CSF白蛋白浓度和Q值显著高于NINP患者(分别为p = 0.0003和p = 0.0095)。共有9例(19%)CIDP患者出现血清和CSF OCB相同的情况(“镜像模式”),而抗MAG抗体神经病患者中有3例(16.6%)、GBS患者中有13例(40.6%)、NINP患者中有12.5%出现这种情况。只有1例CIDP患者显示出仅存在于CSF中的OCB。一线治疗对80.4%的CIDP患者有效,与CSF检查结果无关。
与NINP相比,CIDP、GBS和抗MAG抗体神经病患者的CSF蛋白和血 - 脊神经根屏障损伤显著增加。在我们的CIDP患者中,鞘内体液免疫反应很少见。全身寡克隆激活更常见,但与对照组检测到的情况无显著差异。