Vital A, Lamarche J, Bernier J P, Boulan-Predseil P, Loiseau H, Rougier A, Henry P, Vital C
Service d'Anatomie-Pathologique et de Neuropathologie, Hôpital Pellegrin, Bordeaux.
Ann Pathol. 1995;15(4):269-75.
Seven observations, having in common an accumulation of polyglucosan bodies in the nervous system, are reported. In 2 cases of adult polyglucosan body disease, characteristic polyglucosan bodies were found on the peripheral nerve biopsies, and also on the cerebral biopsy in one of these patients who was demented. The 5 other cases presented medically intractable temporal lobe epilepsy. Recovery was obtained by a temporal lobectomy in which a massive accumulation of polyglucosan bodies was discovered. These personal observations are discussed with regard to other conditions characterized by accumulation of polyglucosan bodies in the nervous system. The non specificity of these formations is emphasized.
报告了7例在神经系统中共同存在多聚葡萄糖体蓄积的病例。在2例成人多聚葡萄糖体病患者中,外周神经活检发现了特征性的多聚葡萄糖体,其中1例患有痴呆的患者在脑活检中也发现了多聚葡萄糖体。其他5例表现为药物难治性颞叶癫痫。通过颞叶切除术获得了康复,术中发现大量多聚葡萄糖体蓄积。结合其他以神经系统中多聚葡萄糖体蓄积为特征的疾病对这些个人观察结果进行了讨论。强调了这些病变的非特异性。