Restrepo Carlos E, Amrami Kimberly K, Howe Benjamin M, Dyck P James B, Mauermann Michelle L, Spinner Robert J
Departments of 1 Neurologic Surgery.
Radiology, Mayo Clinic, Rochester, Minnesota.
Neurosurg Focus. 2015 Sep;39(3):E13. doi: 10.3171/2015.6.FOCUS15225.
Intraneural perineurioma is a rare, benign slow-growing lesion arising from the perineurial cells that surrounds the peripheral nerve fibers. Typically it presents during childhood and young adulthood as a motor mononeuropathy. MRI plays an essential role in the diagnosis and localization of the lesion, which appears as a fusiform enlargement of the nerve fascicles that enhances intensely with gadolinium. Despite the typical clinical and radiological features, intraneural perineurioma remains largely underdiagnosed because of the lack of familiarity with this entity, but also as a result of technical limitations with conventional MRI that is typically performed as a screening test over a large field of view and without contrast sequences. The purpose of this article is to present the pitfalls and pearls learned from years of experience in the diagnosis and management of this relatively rare condition. Clinical suspicion and detailed neurological examination followed by high-quality electrophysiological studies (EPS) must lead to an adequate preimaging localization of the lesion and narrowing of the imaging area. The use of high-resolution (3-T) MRI combined with gadolinium administration will allow adequate visualization of the internal anatomy of the nerve and help in differentiating other causes of neuropathy. In cases where the lesion is not recognized but clinical suspicion is high, possible errors must be assessed, including the EPS localization, area of imaging, MRI resolution, and slice thickness.
神经内神经束膜瘤是一种罕见的、生长缓慢的良性病变,起源于围绕周围神经纤维的神经束膜细胞。通常在儿童期和青年期表现为运动性单神经病。MRI在病变的诊断和定位中起着至关重要的作用,病变表现为神经束梭形增粗,钆增强扫描时有明显强化。尽管有典型的临床和影像学特征,但由于对该实体缺乏了解,以及传统MRI技术的局限性(传统MRI通常作为大视野筛查检查且无对比序列),神经内神经束膜瘤在很大程度上仍未得到充分诊断。本文的目的是介绍从多年来诊断和治疗这种相对罕见疾病的经验中获得的陷阱与要点。临床怀疑和详细的神经系统检查,随后进行高质量的电生理研究(EPS),必须能对病变进行充分的成像前定位并缩小成像区域。使用高分辨率(3-T)MRI结合钆剂注射将能充分显示神经的内部解剖结构,并有助于鉴别其他神经病变的原因。在病变未被识别但临床怀疑度高的情况下,必须评估可能出现的错误,包括EPS定位、成像区域、MRI分辨率和切片厚度。