Ohkura M, Mitsudome A, Yasumoto S, Ogawa A
Department of Pediatrics, School of Medicine, Fukuoka University.
No To Hattatsu. 1996 Jul;28(4):332-5.
Although pathological changes are observed in both the oculomotor nucleus and abducens nucleus in autopsied cases of infantile progressive spinal muscular atrophy, external and internal ocular palsy and ptosis have not been previously reported clinically. We presented here two long-surviving cases on respirators which gradually developed ophthalmoplegia and ptosis were presented. From our observation of these cases, it was suggested that there are certain periods of latencies between the occurrence of pathological changes and their clinical manifestation and that the lack of clinical signs of upper cranial nerve involvement in cases with Werdnig-Hoffmann type I is due to their short survival length.
尽管在婴儿进行性脊髓性肌萎缩症的尸检病例中，动眼神经核和展神经核均观察到病理变化，但此前临床上尚未报告过眼外肌和眼内肌麻痹及上睑下垂的情况。我们在此报告两例长期使用呼吸机存活的病例，其逐渐出现了眼肌麻痹和上睑下垂。通过对这些病例的观察，提示病理变化的发生与其临床表现之间存在一定的潜伏期，且Ⅰ型韦尼克 - 霍夫曼病患者缺乏上颅神经受累的临床体征是由于其存活时间较短。
