Galaburda A M, Waxman S G, Kemper T L, Jones H R
J Neuropathol Exp Neurol. 1976 Sep-Oct;35(5):481-94. doi: 10.1097/00005072-197609000-00001.
A sixty-six year-old man developed visual and gait difficulties, a deficit in recent memory and hearing, and acute urinary retention. He subsequently became blind, deaf, and quadriparetic. Terminally, weakness deepended and he lapsed into semi-coma. The cerebrospinal fluid contained increased amounts of protein with a normal electrophoretic pattern, and a moderate mononuclear pleocytosis. Gross examination of the brain revealed normal vessels and meninges. Coronal sections showed irregular areas of subpial discoloration in forebrain and brainstem. Stains for myelin revealed scattered foci of pallor in the intracortical myelinated fibers; demyelination throughout the brainstem, most severe in subpial areas; and a focus of central pontine myelin loss. The subependymal myelin was intact throughout the neuraxis. Histological examination showed axonal preservation in areas of extreme myelin loss. Demyelination was accompanied by astrocytic gliosis showing different degrees of activity, and occasional perivascular cuffing with mononuclear cells in cerebral and meningeal vessels. Electron microscopy revealed filamentous structures in cell nuclei. These were 180-220 A in diameter, had a hollow core, and, in some tangential sections, showed cross striations. The unusual clinical picture and correspondingly striking pattern of demyelination suggest that this case might represent a distinct clinical entity.
一名66岁男性出现视觉和步态障碍、近期记忆力和听力减退以及急性尿潴留。随后他失明、失聪并出现四肢瘫痪。临终时，虚弱加重，陷入半昏迷状态。脑脊液中蛋白质含量增加，电泳图谱正常，单核细胞轻度增多。大脑大体检查显示血管和脑膜正常。冠状切面显示前脑和脑干软膜下有不规则的变色区域。髓磷脂染色显示皮质内有髓纤维有散在的苍白灶；整个脑干脱髓鞘，软膜下区域最严重；还有一个脑桥中央髓鞘脱失灶。整个神经轴的室管膜下髓磷脂完整。组织学检查显示在髓鞘严重脱失区域轴突保存。脱髓鞘伴有不同程度活跃的星形胶质细胞增生，以及大脑和脑膜血管周围偶尔有单核细胞套袖状浸润。电子显微镜检查在细胞核中发现丝状结构。这些结构直径为180 - 220埃，有一个中空的核心，在一些切线切片中显示有横纹。这种不寻常的临床表现和相应显著的脱髓鞘模式表明该病例可能代表一种独特的临床实体。
