Aimard G, Boisson D, Kopp N, Devic M
Rev Neurol (Paris). 1984;140(8-9):510-2.
A further case of apparently primary sensorimotor neuropathy is reported. The patient, a 31-year-old man, developed the syndrome after a 4-year history of a solitary vertebral plasmocytoma revealed by post mortem examination. Circulating monoclonal immunoglobulin and specific anomalies on nerve biopsy were absent. The condition became progressively worse, with initial and predominant autonomic nervous system anomalies. After 3 years, contractures of the limbs and paravertebral muscles, with painful paroxysmal attacks suggestive of the stiff-man syndrome appeared. Pathology confirmed the severity of the root lesions with diffusion to the cranial nerves. Reflex tests could not be conducted but the semiologic characteristics and the efficacity of baclofen were distinctive of continuous muscle fiber activities reported in some peripheral neuropathies. This case was comparable to some reported ones of interneuron rigidity.
本文报告了另一例明显的原发性感觉运动性神经病变病例。患者为一名31岁男性，在尸检发现孤立性椎体浆细胞瘤4年后出现该综合征。神经活检未发现循环单克隆免疫球蛋白和特异性异常。病情逐渐恶化，最初以自主神经系统异常为主。3年后，出现肢体和椎旁肌挛缩，并伴有提示僵人综合征的疼痛性阵发性发作。病理学证实了神经根病变的严重性，并已扩散至颅神经。无法进行反射测试，但巴氯芬的症状学特征和疗效与一些周围神经病变中报道的持续性肌纤维活动一致。该病例与一些报道的中间神经元僵硬病例相似。
