Fustes Otto Hernandez, Kay Cláudia Suemi Kamoi, Lorenzoni Paulo José, Ducci Renata Dal-Prá, Werneck Lineu Cesar, Scola Rosana Herminia
Department of Neurology, Complexo Hospital de Clínicas da UFPR, Curitiba, Paraná, Brazil.
Surg Neurol Int. 2020 Dec 22;11:464. doi: 10.25259/SNI_861_2020. eCollection 2020.
Hirayama's disease (HD) is characterized by an insidious onset asymmetric weakness and atrophy of the forearm and hand. Taking as a premise, the etiopathogenesis of the disease is attributed to forward displacement of posterior wall of lower cervical dural canal in neck flexion causing marked compression and flattening of lower spinal cord. This may result in compression of the posterior column of the spinal cord and seems likely to result in somatosensory evoked potentials (SSEPs) abnormalities. In the present study, we studied the possible involvement of the lemniscal dorsal pathway in patients with HD.
SSEPs in upper and lower extremities were prospectively performed in eight patients with HD. All the patients were recruited from the outpatient clinic of a neuromuscular disorder center from South Brazil. SSEPs were obtained by transcutaneous electrical stimulation of the median and posterior tibial nerves, on both sides. We collected the amplitude and the latency of the different components obtained in each channel. The interpretation was based on Brazilian study standards.
We evaluated seven men and one woman (mean age 27). The data obtained were compared to a control group consisting of eight patients with spondylotic cervical myelopathy, 6 men and 2 women with mean age of 59 years. The measurements of obtained by the SSEP were also compared between the groups and no significant difference was found for any of them.
SSEP did not turn out to be an electrophysiological marker in our HD patients.
平山病（HD）的特征是隐匿起病，表现为前臂和手部不对称性肌无力及萎缩。基于此，该病的发病机制归因于颈部屈曲时下颈段硬脊膜管后壁向前移位，导致下脊髓明显受压并变扁。这可能导致脊髓后柱受压，似乎也会导致体感诱发电位（SSEP）异常。在本研究中，我们研究了HD患者中薄束背侧通路可能受到的影响。
对8例HD患者前瞻性地进行了上下肢SSEP检查。所有患者均来自巴西南部一家神经肌肉疾病中心的门诊。通过经皮电刺激双侧正中神经和胫后神经获得SSEP。我们收集了每个通道中不同成分的波幅和潜伏期。解读基于巴西的研究标准。
我们评估了7名男性和1名女性（平均年龄27岁）。将获得的数据与一个由8例脊髓型颈椎病患者组成的对照组进行比较，该对照组包括6名男性和2名女性，平均年龄59岁。还比较了两组之间通过SSEP获得的测量结果，未发现任何一项有显著差异。
在我们的HD患者中，SSEP并非一种电生理标志物。
