• 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Coexistence of amyotrophic lateral sclerosis with neuro-Beh?et's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient.
【24h】

Coexistence of amyotrophic lateral sclerosis with neuro-Beh?et's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient.

机译：肌萎缩性侧索硬化症与神经-贝叶氏病共存，表现为纵向广泛的脊髓病变：尸检患者的临床病理特征。

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

We report the clinical and autopsy features of a 65-year-old Japanese man who clinically exhibited overlap of both neuro-Beh?et's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA-B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1-weighted images, high intensity on T2-weighted and fluid-attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein-like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.
机译：我们报告了一名65岁的日本男子的临床和尸检特征，该男子在临床上表现出神经贝叶氏病（NBD）和肌萎缩性侧索硬化症（ALS）重叠。该患者患有HLA-B51血清型，最近有葡萄膜炎病史，并患有截瘫，感觉和自主神经紊乱，额叶体征和震颤。一项大脑和脊柱MRI研究显示了一个纵向广泛的胸索（Th）病变，但没有明显的颅内异常。病变从Th4到Th9腹侧扩展，在T1加权图像上显示低强度，在T2加权和液体衰减的反转恢复图像上显示高强度，and增强。患者的上下运动神经元体征和感觉障碍加重，入院后16个月死亡。尸检时，脊髓和大脑均表现出NBD和ALS的特征性组织病理学特征，包括慢性破坏腹侧胸白质和灰质，血管周淋巴细胞浸润，双核神经元，上下运动神经元变性，布尼纳体和类绞线夹杂物。尽管这些罕见疾病的偶然共存可能会在个体中发生，但这种情况增加了NBD和ALS之间发病机制关联的可能性。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号