Béquet D, Labauge P, Larroque P, Renard J L, Goasguen J
Clinique de Neurologie, Hôpital du Val-de-Grâce, Paris.
Rev Neurol (Paris). 1990;146(12):757-61.
We report a case of giant plexiform neurofibromatosis revealed by a sensori-motor, axo-myelinic polyneuropathy. Only the lumbar and sacral spinal nerves were involved in this peripheral form of Von Recklinghausen's disease. The polyneuropathy was due to compression of the nervous fibers by the neurofibromatous tumors. Ultrasonography DTPA 99 m Technetium scintigraphy, CT and MRI were the diagnostic procedures. MRI proved superior to CT in demonstrating the extent of the lesions. Biopsy confirmed the diagnosis. This form of neurofibromatosis did not involve the central nervous system, cranial nerves of cervicothoracic spinal nerves and roots; nor was there any visceral involvement, particularly endocrine neoplasia.
我们报告一例由感觉运动性轴索-髓鞘型多发性神经病所揭示的巨大丛状神经纤维瘤病。在这种周围型冯·雷克林霍增氏病中，仅腰骶部脊神经受累。多发性神经病是由神经纤维瘤性肿瘤对神经纤维的压迫所致。超声、二巯基丁二酸（DTPA）锝-99m闪烁扫描、CT和MRI为诊断方法。MRI在显示病变范围方面被证明优于CT。活检确诊了诊断。这种神经纤维瘤病形式未累及中枢神经系统、颈胸段脊神经和神经根的颅神经；也没有任何内脏受累，特别是内分泌肿瘤形成。
