Optic and auditory pathway dysfunction in demyelinating neuropathies
Optic and auditory pathway dysfunction in demyelinating neuropathies
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DOI：
10.1111/ane.12226
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发表时间：
2014-07-01
影响因子：
3.5
通讯作者：
Rajabally, Y. A.
中科院分区：
文献类型：
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作者：
Knopp, M.;Leese, R. J.;Rajabally, Y. A.
目的脱髓鞘性多发性神经病是否累及视、听通路的研究很少。我们在此的目的是在一组获得性和遗传性脱髓鞘神经病患者中进一步研究这一点。方法应用视觉诱发电位和脑干听觉诱发电位对8例遗传性压力易感性神经病（HNPP）、6例腓骨肌萎缩症1A型（CMT1A）、10例慢性炎性脱髓鞘性多发性神经病（CIDP）和7例抗髓鞘相关糖蛋白（MAG）神经病进行研究。结果7例抗MAG神经病患者中有6例出现视路功能障碍，约占CIDP和HNPP患者的一半，而CMT1A患者中仅1例出现视路功能障碍。除HNPP组外，其余各组均出现周围听神经功能障碍。脑干受累在所有组中都是例外。结论：除CMT1A外，所有脱髓鞘性多发性神经病，尤其是抗MAG神经病，都可能发生视神经受累。HNPP可能由于没有局部压迫而保留了周围听神经。在所有四种研究的神经病变中，中枢脑干病理学的证据很少出现。这项研究表明，获得性和遗传性脱髓鞘性多发性神经病可能与视和听神经受累有关，这可能导致神经功能障碍，需要更多的认识。
Objective The involvement of optic and auditory pathways has rarely been studied in demyelinating polyneuropathies. We here aimed to study this further in a cohort of patients with acquired and gentic demyelinating neuropathy. Methods We studied eight patients with hereditary neuropathy with liability to pressure palsies (HNPP), six with Charcot-Marie-Tooth disease type 1A (CMT1A), ten with chronic inflammatory demyelinating polyneuropathy (CIDP) and seven with antimyelin-associated glycoprotein (MAG) neuropathy using visual evoked potentials and brainstem auditory evoked potentials. Results Optic pathway dysfunction was detected in 6/7 anti-MAG neuropathy patients, about half of those with CIDP and HNPP, but only in 1/6 patients with CMT1A. Peripheral auditory nerve dysfunction appeared common in all groups except HNPP. Brainstem involvement was exceptional in all groups. Conclusions We conclude optic nerve involvement may be frequent in all demyelinating polyneuropathies, particularly anti-MAG neuropathy, except in CMT1A. Peripheral auditory nerves may be spared in HNPP possibly due to absence of local compression. Evidence for central brainstem pathology appeared infrequent in all four studied neuropathies. This study suggests that acquired and genetic demyelinating polyneuropathies may be associated with optic and auditory nerve involvement, which may contribute to neurological disability, and require greater awareness.