Department of Neurology and Clinical Neurophysiology, Bay of Plenty District Health Board, Tauranga Hospital, Tauranga, New Zealand.
Department of Neurosciences, Reproductive Sciences and Odontostomatology, University Federico II, Naples, Italy.
Muscle Nerve. 2019 Sep;60(3):271-278. doi: 10.1002/mus.26613. Epub 2019 Jul 5.
Use of peripheral nerve ultrasound alongside standard electrodiagnostic tests may help to gain insight into the pathophysiology of peripheral nerve involvement in type 2 spinocerebellar ataxia (SCA2).
Twenty-seven patients with SCA2 underwent ultrasound cross-sectional area (CSA) measurement of median, ulnar, sural and tibial nerves, and motor (median, ulnar, tibial) and sensory (median, ulnar, radial, sural) nerve conduction studies.
Twenty patients had pathologically small-nerve CSAs, suggestive of sensory neuronopathy. In these patients, electrophysiology showed non-length-dependent sensory neuropathy (14 of 20), "possible sensory neuropathy" (1 of 20), or normal findings (5 of 20). Four different patients had length-dependent sensory neuropathy on electrophysiology, and 1 had enlarged nerve CSAs. Regression analysis showed an inverse relationship between ataxia scores and upper limb nerve CSA (P < 0.03).
Our findings suggest that a majority of patients with SCA2 (74%) have a sensory neuronopathy and this correlates with disability. A minority of patients have findings consistent with axonal neuropathy (18%). Muscle Nerve, 2019.
在标准电诊断测试的基础上联合应用周围神经超声检查，可能有助于深入了解 2 型脊髓小脑共济失调（SCA2）患者周围神经受累的病理生理学机制。
27 例 SCA2 患者接受正中神经、尺神经、腓肠神经和胫神经的超声横截面积（CSA）测量，以及正中神经、尺神经、胫神经的运动神经传导研究和正中神经、尺神经、桡神经、腓肠神经的感觉神经传导研究。
20 例患者的小神经 CSA 存在病理改变，提示存在感觉神经元病。这些患者的电生理检查显示非长度依赖性感觉神经病（20 例中的 14 例）、“可能的感觉神经病”（20 例中的 1 例）或正常发现（20 例中的 5 例）。4 例不同的患者电生理检查显示长度依赖性感觉神经病，1 例患者的神经 CSA 增大。回归分析显示，小脑共济失调评分与上肢神经 CSA 呈负相关（P<0.03）。
我们的研究结果表明，大多数 SCA2 患者（74%）存在感觉神经元病，且这与残疾程度相关。少数患者的检查结果符合轴索性神经病（18%）。《肌肉神经》，2019 年。
