Terao Shin-ichi, Miura Naofumi, Noda Aiji, Yoshida Mari, Hashizume Yoshio, Ikeda Hiroshi, Sobue Gen
Division of General Medicine, Department of Internal Medicine, Aichi Medical University School of Medicine, Aichi 480-1195, Japan.
Clin Neurol Neurosurg. 2006 Oct;108(7):670-4. doi: 10.1016/j.clineuro.2005.03.015. Epub 2005 Sep 13.
We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis. He showed mild weakness of the facial muscle and tongue, dysarthria, and severe muscle atrophy from the neck to proximal upper extremities and trunk, but no obvious pyramidal signs. Electromyogram revealed neurogenic changes in the right leg, and in the paraspinal, sternocleidomastoid, and lingual muscles. There was a slight increase in central motor conduction time from the motor cortex to the lumbar anterior horn. Pulmonary function showed restrictive ventilation dysfunction, which was the eventual cause of death. Some neuropathological features were suggestive of amyotrophic lateral sclerosis (ALS), namely Bunina bodies. In patients with a history of paralytic poliomyelitis who present after a long stable period with advanced fatal respiratory failure, one may consider not only respiratory impairment from post-polio syndrome but also the onset of ALS.
我们报告了一名69岁男性,他童年时患麻痹性脊髓灰质炎,数十年后死于呼吸衰竭。在这一事件发生前6个月,他出现进行性体重减轻和呼吸急促。作为麻痹性脊髓灰质炎的后遗症,他整个右腿有严重的肌肉萎缩。他表现出面部肌肉和舌头轻度无力、构音障碍,以及从颈部到近端上肢和躯干的严重肌肉萎缩,但无明显锥体束征。肌电图显示右腿以及椎旁肌、胸锁乳突肌和舌肌有神经源性改变。从运动皮层到腰前角的中枢运动传导时间略有增加。肺功能显示限制性通气功能障碍,这是最终的死亡原因。一些神经病理学特征提示肌萎缩侧索硬化(ALS),即布宁小体。对于曾患麻痹性脊髓灰质炎且在长期稳定期后出现晚期致命性呼吸衰竭的患者,不仅要考虑脊髓灰质炎后综合征导致的呼吸功能损害,还要考虑ALS的发病。