Malandrini A, Villanova M, Tripodi S, Palmeri S, Sicurelli F, Parrotta E, Berti G, Salvadori C, Cintorino M, Guazzi G C
Institute of Neurological Sciences, University of Siena, Italy.
Brain Dev. 1998 Aug;20(5):290-4. doi: 10.1016/s0387-7604(98)00032-1.
We report neuropathological findings in a 22-year-old man affected with neuronal intranuclear inclusion disease. The inclusions affected to different extents the various structures of the central nervous system, being more numerous in cerebral cortex, inferior olives, hypoglossal and oculomotor nuclei. They ultrastructurally differed from Marinesco bodies. In the neurons of the substantia nigra, we occasionally observed intranuclear inclusions resembling the so-called rodlets of Roncoroni. We did not observe inclusions in the extraneuronal tissues. There was no apparent correlation between frequency of the inclusions and neuronal loss. Intranuclear inclusions were found in many morphologically normal neurons. We suggest that the intranuclear inclusions are the marker of a distinctive disorder, even though their role in neuronal degeneration remains to be clarified.
我们报告了一名患有神经元核内包涵体病的22岁男性的神经病理学发现。这些包涵体对中枢神经系统的各种结构造成了不同程度的影响，在大脑皮层、下橄榄核、舌下神经核和动眼神经核中更为常见。它们在超微结构上与马里内斯科小体不同。在黑质神经元中，我们偶尔观察到核内包涵体类似于所谓的龙科罗尼小杆。我们在神经外组织中未观察到包涵体。包涵体的频率与神经元丢失之间没有明显的相关性。在许多形态正常的神经元中发现了核内包涵体。我们认为，核内包涵体是一种独特疾病的标志物，尽管它们在神经元变性中的作用仍有待阐明。
