Espinal J B, Indakoetxea B, López de Munain A, Martí-Massó J F, Riu I
Sección de Neurología, Hospital Nuestra Señora de Aránzazu, San Sebastián.
Neurologia. 1991 Apr;6(4):142-7.
Three patients (two females and one male) with radiculospinal neurologic involvement secondary to different forms of neurofibromatosis are reported. The first two met the diagnostic criteria for NF-1, although case 2 had a posterior fossa meningioma, which is an uncommon finding in this group. The male patient had an apparently sporadic NF-2, with bilateral acoustic nerve neurinoma, multiple meningioma, multiple radicular neurinoma and an intraspinal tumor apparent in magnetic resonance imaging. In the three cases the whole central nervous system was evaluated with gadolinium-enhanced magnetic resonance imaging. A great number of radicular tumors, many of which were asymptomatic, were detected. In spite of the severity of the clinical features, the three patients showed a dramatic improvement after the surgical removal of the symptomatic tumors. The use of magnetic resonance is encouraged, owing to its high resolution and safety, for the assessment of incidence, character and localization of tumors in neurofibromatosis and to establish a good clinico-lesional correlation before surgery. This technique may help to a better understanding of the spectrum of abnormalities in each type of neurofibromatosis, thus facilitating the evaluation of this complex condition.
本文报告了3例因不同类型神经纤维瘤病继发神经根脊髓神经受累的患者（2例女性，1例男性）。前两例符合NF-1的诊断标准，尽管病例2患有后颅窝脑膜瘤，这在该组中并不常见。男性患者表现为明显散发的NF-2，患有双侧听神经神经瘤、多发脑膜瘤、多发神经根神经瘤以及磁共振成像中显示的椎管内肿瘤。在这3例病例中，均采用钆增强磁共振成像对整个中枢神经系统进行了评估。检测到大量神经根肿瘤，其中许多无症状。尽管临床症状严重，但3例患者在手术切除有症状的肿瘤后均有显著改善。由于磁共振具有高分辨率和安全性，因此鼓励使用其评估神经纤维瘤病中肿瘤的发生率、特征和定位，并在手术前建立良好的临床病变相关性。该技术有助于更好地了解每种类型神经纤维瘤病的异常谱，从而便于对这种复杂疾病进行评估。
