Hernández A, Céspedes G, Pinto J, Santana M
Sección de Neuropatología, Instituto Anatomopatológico, Venezuela.
Neurologia. 2001 Mar;16(3):138-42.
Adult polyglucosan body disease is a recently established clinicopathological entity, with few cases reported in the literature. In this paper we describe a case of a 46-year-old man who had died to a pancreatic cancer whose neuropathological study revealed a massive accumulation of polyglucosan bodies in the cerebral white matter, brainstem, cerebellum and spinal cord consistent with the adult polyglucosan body disease. We also discuss the pathological criteria used to differentiate this disease from others in which polyglucosan bodies may accumulate in the nervous system.
成人多聚葡萄糖体病是一种最近才确定的临床病理实体,文献中报道的病例很少。在本文中,我们描述了一例46岁死于胰腺癌的男性病例,其神经病理学研究显示,在大脑白质、脑干、小脑和脊髓中大量积聚多聚葡萄糖体,符合成人多聚葡萄糖体病。我们还讨论了用于将这种疾病与其他可能在神经系统中积聚多聚葡萄糖体的疾病相鉴别的病理标准。