van der Sluis R W, Wolfe G I, Nations S P, Bryan W W, Krampitz D E, Kissel J T, Barohn R J
From the *Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, and the daggerDepartment of Neurology, Ohio State University School of Medicine, Columbus, Ohio.
J Clin Neuromuscul Dis. 2000 Sep;2(1):10-7. doi: 10.1097/00131402-200009000-00004.
Objective To describe the clinical features of four patients we encountered with post-radiation lower motor neuron syndromes and to review the related literature BACKGROUND.: Radiation therapy for malignant neoplasms has been associated with a post-radiation lower motor neuron syndrome (PRLMNS). The earliest descriptions date back to World War II.
We evaluated four patients who developed a lower motor neuron syndrome several years after the completion of radiation therapy to treat malignancies. The clinical and electrophysiological features of these patients are described.
Our patients with PRLMNS developed weakness, muscle atrophy, loss of reflexes, and fasciculations in myotomal distributions that corresponded to the regions that had been exposed to radiation The mean time between radiation exposure and onset of motor symptoms was 14 years. Sensory symptoms were either absent or minor. Motor and sensory nerve conduction studies were normal or only mildly affected, Needle electromyography showed varying degrees of active and chronic denervation changes, primarily in the distributions that had received radiation. Magnetic resonance imaging of the spine and myelography were unremarkable. Serum creatine kinase levels were elevated in two patients. The patients followed a stable to slowly progressive course at a mean follow up of 6.5 years.
. Patients presenting with lower motor neuron syndromes should be questioned about prior radiation exposure. A diagnosis of PRLMNS carries a relatively favorable prognosis when compared with amyotrophic lateral sclerosis, another acquired motor neuron disorder.
目的:描述我们遇到的4例放疗后下运动神经元综合征患者的临床特征,并复习相关文献。背景:恶性肿瘤的放射治疗与放疗后下运动神经元综合征(PRLMNS)有关。最早的描述可追溯到第二次世界大战。
我们评估了4例在完成恶性肿瘤放射治疗数年后出现下运动神经元综合征的患者。描述了这些患者的临床和电生理特征。
我们的PRLMNS患者出现肌无力、肌肉萎缩、反射消失以及与接受放疗区域相对应的肌节分布区的肌束震颤。放疗暴露与运动症状发作之间的平均时间为14年。感觉症状要么不存在,要么很轻微。运动和感觉神经传导研究正常或仅轻度受影响,针极肌电图显示不同程度的主动和慢性失神经改变,主要出现在接受放疗的分布区。脊柱磁共振成像和脊髓造影无明显异常。2例患者血清肌酸激酶水平升高。患者在平均6.5年的随访中病情稳定或缓慢进展。
出现下运动神经元综合征的患者应询问其既往放疗史。与另一种获得性运动神经元疾病肌萎缩侧索硬化症相比,PRLMNS的诊断预后相对较好。