Zangemeister W H, Schwendemann G, Colmant H J
J Neurol. 1978 Apr 14;218(1):63-71. doi: 10.1007/BF00314720.
A man, aged 63, had an illness which lasted 11 months from onset with pain under the left costal margin which radiated to the epigastrium, until his death from cardiac failure. His symptoms consisted principally of parasthesias and proximal weakness of both upper and lower extremities with atrophy of the shoulder and pelvic girdles. He developed pyramidal tract signs, became euphoric, emotionally unstable and mentally retarded. There was no clinical evidence of cerebellar dysfunction. Bronchogenic carcinoma was suspected from a tomograph of the thorax, but, in spite of extensive clinical and laboratory studies, the diagnosis was verified only postmortem. The CSF cell count was high at first but diminished as the disease progressed. Muscle biopsies revealed chronic generalized denervation without signs of myopathy. Neuropathologically, encephalomyeloradiculoneuritis concentrated on the spinal cord was combined with severe rarefaction of the ganglion cells of the anterior horns and with bilateral degeneration of the lateral pyramidal spinocerebellar and posterior tracts. A more diffuse process was obvious in the anterolateral tracts of the lumbar region. Polyneuropathy concentrated in the distal region was accompanied by slight inflammatory reaction in the sciatic nerve. Cerebellocortical degeneration which exceeded physiological age-related rarefaction was also present. The findings are discussed in relation to the literature.
一名63岁男性,患病11个月,自发病起左肋缘下疼痛并放射至上腹部,最终死于心力衰竭。其症状主要包括感觉异常以及双上肢和双下肢近端无力,伴有肩胛带和骨盆带萎缩。他出现了锥体束征,变得欣快、情绪不稳定且智力发育迟缓。没有小脑功能障碍的临床证据。胸部断层扫描怀疑有支气管源性癌,但尽管进行了广泛的临床和实验室检查,诊断仅在死后得到证实。脑脊液细胞计数起初较高,但随着疾病进展而减少。肌肉活检显示慢性全身性去神经支配,无肌病迹象。神经病理学检查发现,集中在脊髓的脑脊髓神经根神经炎合并前角神经节细胞严重稀疏以及双侧锥体、脊髓小脑和后束变性。在腰椎区域的前外侧束中,更弥漫的病变明显。集中在远端区域的多神经病伴有坐骨神经轻微炎症反应。还存在超过生理年龄相关稀疏程度的小脑皮质变性。结合文献对这些发现进行了讨论。