评估肾上腺脑白质营养不良患者的传入性疼痛通路。
Evaluation of afferent pain pathways in adrenomyeloneuropathic patients.
机构信息
Neurophysiology Unit, Neurology Department, Hospital Universitari de Bellvitge, Feixa Llarga, 08907 L'Hospitalet de Llobregat, Barcelona, Spain; Department of Clinical Neurophysiology, Quiron-Dexeus Hospital, Sabino Arana 5-19, 08028 Barcelona, Spain.
Neurophysiology Unit, Neurology Department, Hospital Universitari de Bellvitge, Feixa Llarga, 08907 L'Hospitalet de Llobregat, Barcelona, Spain.
出版信息
Clin Neurophysiol. 2018 Mar;129(3):507-515. doi: 10.1016/j.clinph.2017.12.011. Epub 2017 Dec 24.
OBJECTIVE
Patients with adrenomyeloneuropathy may have dysfunctions of visual, auditory, motor and somatosensory pathways. We thought on examining the nociceptive pathways by means of laser evoked potentials (LEPs), to obtain additional information on the pathophysiology of this condition.
METHODS
In 13 adrenomyeloneuropathic patients we examined LEPs to leg, arm and face stimulation. Normative data were obtained from 10 healthy subjects examined in the same experimental conditions. We also examined brainstem auditory evoked potentials (BAEPs), pattern reversal full-field visual evoked potentials (VEPs), motor evoked potentials (MEPs) and somatosensory evoked potentials (SEPs).
RESULTS
Upper and lower limb MEPs and SEPs, as well as BAEPs, were abnormal in all patients, while VEPs were abnormal in 3 of them (23.1%). LEPs revealed abnormalities to stimulation of the face in 4 patients (30.7%), the forearm in 4 patients (30.7%) and the leg in 10 patients (76.9%).
CONCLUSIONS
The pathologic process of adrenomyeloneuropathy is characterized by a preferential involvement of auditory, motor and somatosensory tracts and less severely of the visual and nociceptive pathways. This non-inflammatory distal axonopathy preferably damages large myelinated spinal tracts but there is also partial involvement of small myelinated fibres.
SIGNIFICANCE
LEPs studies can provide relevant information about afferent pain pathways involvement in adrenomyeloneuropathic patients.
目的
肾上腺脑白质营养不良患者可能存在视觉、听觉、运动和躯体感觉通路的功能障碍。我们考虑通过激光诱发电位（LEP）来检查伤害感受通路，以获得有关这种疾病病理生理学的更多信息。
方法
我们检查了 13 名肾上腺脑白质营养不良患者的腿部、手臂和面部刺激的 LEP。在相同的实验条件下，我们还检查了 10 名健康受试者的正常数据。我们还检查了脑干听觉诱发电位（BAEPs）、图形反转全视野视觉诱发电位（VEPs）、运动诱发电位（MEPs）和躯体感觉诱发电位（SEPs）。
结果
所有患者的上肢和下肢 MEPs 和 SEPs 以及 BAEPs 均异常，而 3 名患者（23.1%）的 VEP 异常。4 名患者（30.7%）面部刺激、4 名患者（30.7%）前臂刺激和 10 名患者（76.9%）腿部刺激的 LEP 显示异常。
结论
肾上腺脑白质营养不良的病理过程表现为听觉、运动和躯体感觉通路的优先受累，而视觉和伤害感受通路的受累程度较轻。这种非炎症性远端轴索性神经病优选地损害大的有髓鞘脊髓束，但也有小的有髓纤维的部分参与。
意义
LEP 研究可以提供有关肾上腺脑白质营养不良患者传入疼痛通路受累的相关信息。
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