Attarian Shahram, Franques Jérôme, Elisabeth Jouve, Trébuchon Agnes, Duclos Yann, Wybrecht Délphine, Verschueren Annie, Salort-Campana Emmanuelle, Pouget Jean
Reference Centre for Neuromuscular Diseases and ALS, Department of Neurology and Neuromuscular Diseases, Centre Hospitalier Universitaire La Timone, 264 rue Saint-Pierre, 13385, Marseille, France; Inserm UMR_S 910 Medical Genetics and Functional Genomics, Aix-Marseille University, Marseilles, France.
Muscle Nerve. 2015 Apr;51(4):541-8. doi: 10.1002/mus.24352. Epub 2015 Jan 9.
A difficult clinical situation occurs when a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patient does not fulfill any of the diagnostic criteria. Moreover, nerve conduction studies (NCS) can be consistent with axonal neuropathy and lead to misdiagnosis.
We aimed to assess the usefulness of the triple-stimulation technique (TST) for detection of proximal conduction blocks (CBs) in patients with axonal-like CIDP. Four patients with axonal-like CIDP were studied and compared with 10 typical CIDP patients. In the axonal-like group, NCS showed a decrease in compound muscle action potential amplitude without features of demyelination, but nerve biopsy showed features of demyelination in all 4.
Twelve nerves were tested with TST, and 8 CBs were detected between the root emergence and the Erb point in the 4 patients, all of whom improved after treatment with intravenous immunoglobulin.
TST can identify very proximal CBs in CIDP. The sensitivity of nerve conduction studies may be improved by TST in CIDP.
当慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)患者不符合任何诊断标准时,就会出现困难的临床情况。此外,神经传导研究(NCS)可能与轴索性神经病一致并导致误诊。
我们旨在评估三重刺激技术(TST)对检测轴索性CIDP患者近端传导阻滞(CBs)的有用性。研究了4例轴索性CIDP患者,并与10例典型CIDP患者进行比较。在轴索性组中,NCS显示复合肌肉动作电位幅度降低,无脱髓鞘特征,但神经活检显示所有4例均有脱髓鞘特征。
用TST检测了12条神经,在4例患者的神经根穿出点与Erb点之间检测到8处CBs,所有患者经静脉注射免疫球蛋白治疗后均有改善。
TST可识别CIDP中非常近端的CBs。TST可提高CIDP中神经传导研究的敏感性。