医学
自身抗体
多神经根神经病
抗体
共济失调
神经学
血浆置换术
慢性炎症性脱髓鞘性多发性神经病
内科学
人口
神经节苷脂
免疫学
胃肠病学
数据库
病理
格林-巴利综合征
精神科
化学
环境卫生
生物化学
计算机科学
作者
Giuseppe Liberatore,Alberto De Lorenzo,Claudia Giannotta,Fiore Manganelli,Massimiliano Filosto,Giuseppe Cosentino,Dario Cocito,Chiara Briani,Andrea Cortese,Raffaella Fazio,Giuseppe Lauria,Angelo Maurizio Clerici,Tiziana Rosso,Girolama Alessandra Marfia,Giovanni Antonini,Guido Cavaletti,Marinella Carpo,Pietro Emiliano Doneddu,Emanuele Spina,Stefano Cotti Piccinelli,Erdita Peci,Luis Querol,Eduardo Nobile-Orazio
标识
DOI:10.1007/s10072-021-05811-0
摘要
To investigate the frequency and clinical correlates of anti-nerve autoantibodies in an unselected series of Italian patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) METHODS: Sera from 276 CIDP patients fulfilling the EFNS/PNS criteria and included in the Italian CIDP database were examined for the presence of anti-nerve autoantibodies. Results were correlated with the clinical data collected in the database.Anti-neurofascin155 (NF155) antibodies were found in 9/258 (3.5%) patients, anti-contactin1 (CNTN1) antibodies in 4/258 (1.6%) patients, and anti-contactin-associated protein1 (Caspr1) in 1/197 (0.5%) patients, while none had reactivity to gliomedin or neurofascin 186. Predominance of IgG4 isotype was present in 7of the 9 examined patients. Anti-NF155 patients more frequently had ataxia, tremor, and higher CSF protein levels than antibody-negative patients. Anti-CNTN1 patients more frequently had a GBS-like onset, pain, and ataxia and had more severe motor impairment at enrollment than antibody-negative patients. They more frequently received plasmapheresis, possibly reflecting a less satisfactory response to IVIg or steroids. IgM antibodies against one or more gangliosides were found in 6.5% of the patients (17/260) and were more frequently directed against GM1 (3.9%). They were frequently associated with a progressive course, with a multifocal sensorimotor phenotype and less frequent cranial nerve involvement and ataxia.Anti-paranodal and anti-ganglioside antibodies are infrequent in patients with CIDP but are associated with some typical clinical association supporting the hypothesis that CIDP might be a pathogenically heterogeneous syndrome possibly explaining the different clinical presentations.
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