Farschtschi Said, Gelderblom Mathias, Buschbaum Sabriena, Bostock Hugh, Grafe Peter, Mautner Victor F
Department of Neurology, University Medical Centre Hamburg-Eppendorf Hospital, Hamburg, Germany.
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, London, UK.
Muscle Nerve. 2017 Mar;55(3):350-358. doi: 10.1002/mus.25256. Epub 2016 Dec 16.
The neuropathy in patients with neurofibromatosis type 2 (NF2) is difficult to quantify and follow up. In this study we compared 3 methods that may help assess motor axon pathology in NF2 patients.
Nerve conduction studies in median nerves were supplemented by deriving motor unit number estimates (MUNEs) from compound muscle action potential (CMAP) scans and by high-resolution ultrasound (US) peripheral nerve imaging.
CMAP amplitudes and nerve conduction velocity were normal in the vast majority of affected individuals, but CMAP scan MUNE revealed denervation and reinnervation in many peripheral nerves. In addition, nerve US imaging enabled monitoring of the size and number of schwannoma-like fascicular enlargements in median nerve trunks.
In contrast to conventional nerve conduction studies, CMAP scan MUNE in combination with US nerve imaging can quantify the NF2-associated neuropathy and may help to monitor disease progression and drug treatments. Muscle Nerve 55: 350-358, 2017.
2型神经纤维瘤病（NF2）患者的神经病变难以量化和随访。在本研究中，我们比较了3种可能有助于评估NF2患者运动轴突病变的方法。
通过从复合肌肉动作电位（CMAP）扫描得出运动单位数量估计值（MUNE）以及高分辨率超声（US）周围神经成像，对正中神经进行神经传导研究。
绝大多数受影响个体的CMAP波幅和神经传导速度正常，但CMAP扫描MUNE显示许多周围神经存在去神经支配和再支配现象。此外，神经超声成像能够监测正中神经干中神经鞘瘤样束状增粗的大小和数量。
与传统神经传导研究不同，CMAP扫描MUNE联合超声神经成像可量化NF2相关神经病变，并可能有助于监测疾病进展和药物治疗。《肌肉与神经》55: 350 - 358, 2017年。
