Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Hilvarenbeekseweg 60, 5022 GC, Tilburg, The Netherlands.
Muscle Nerve. 2018 Feb;57(2):312-316. doi: 10.1002/mus.25734. Epub 2017 Jul 18.
Neurofibromatosis type 2 (NF2) is mainly associated with central nervous system (CNS) tumors. Peripheral nerve involvement is described in symptomatic patients, but evidence of subclinical peripheral nerve involvement is scarce.
We conducted a cross-sectional pilot study in 2 asymptomatic and 3 minimally symptomatic patients with NF2 to detect subclinical peripheral nerve involvement. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS).
A total of 30 schwannomas were found, divided over 20 nerve segments (33.9% of all investigated nerve segments). All patients had at least 1 schwannoma. Schwannomas were identified with HRUS in 37% of clinically unaffected nerve segments and 50% of nerve segments with normal NCS findings.
HRUS shows frequent subclinical peripheral nerve involvement in NF2. Clinicians should consider peripheral nerve involvement as a cause of weakness and sensory loss in the extremities in patients with this disease. Muscle Nerve 57: 312-316, 2018.
神经纤维瘤病 2 型(NF2)主要与中枢神经系统(CNS)肿瘤相关。有症状的患者会出现周围神经受累,但亚临床周围神经受累的证据很少。
我们对 2 名无症状和 3 名轻度症状的 NF2 患者进行了横断面试点研究,以检测亚临床周围神经受累。患者接受了临床检查、神经传导研究(NCS)和高分辨率超声(HRUS)检查。
共发现 30 个神经鞘瘤,分布在 20 个神经节段(所有研究神经节段的 33.9%)。所有患者均至少有 1 个神经鞘瘤。HRUS 在 37%的临床无影响神经节段和 50%的 NCS 正常神经节段中识别出了神经鞘瘤。
HRUS 显示 NF2 中存在频繁的亚临床周围神经受累。对于患有这种疾病的患者,临床医生应将周围神经受累视为四肢无力和感觉丧失的原因。《肌肉与神经》57:312-316,2018 年。