Auer R N, Rowlands C G, Perry S F, Remmers J E
Department of Pathology, University of Calgary, Canada.
Clin Neuropathol. 1996 Mar-Apr;15(2):101-5.
We report 2 patients with multiple sclerosis (MS) who were symptomatic but ambulatory, and died in their sleep. Sleep studies in 1 patient showed hypercarbia and hypoxia accompanying sleep. In both cases autopsy showed plaques of multiple sclerosis in the medulla oblongata, incompletely involving the neuroanatomic areas of the medullary reticular formation controlling automatic breathing. A systematic analysis of the location of the plaques in relation to areas known to be important in breathing control revealed that the regions corresponding to the ventral nuclear complex of respiratory control in animals were incompletely and unilaterally involved in both cases. Close correlation with nuclei that have been demonstrated in animal experiments to be important in descending respiratory control of phrenic and intercostal musculature was not possible due to possible differences in anatomy between animals and humans, and the fact that plaques of MS affect axons of passage and spare neuronal cell bodies. Nevertheless, the cases clearly illustrate that patchy, unilateral lesions of the medullary reticular formation in humans can give rise to sleep disordered breathing. The cases also illustrate the risk of death during sleep in MS patients with demyelination in the medulla oblongata, and demonstrate the need to carefully examine the medulla in MS patients if they die unexpectedly during sleep.
我们报告了2例患有多发性硬化症（MS）的患者，他们有症状但仍可走动，并在睡眠中死亡。对1例患者的睡眠研究显示，睡眠期间伴有高碳酸血症和低氧血症。在这两个病例中，尸检均显示延髓有多发性硬化斑块，未完全累及控制自主呼吸的延髓网状结构的神经解剖区域。对斑块位置与已知在呼吸控制中重要的区域进行系统分析发现，在这两个病例中，与动物呼吸控制腹侧核复合体相对应的区域均未完全且单侧受累。由于动物和人类之间可能存在解剖学差异，以及MS斑块影响传导轴突并使神经元细胞体免受影响，因此无法将其与动物实验中已证明对膈神经和肋间肌下行呼吸控制很重要的核进行紧密关联。尽管如此，这些病例清楚地表明，人类延髓网状结构的斑片状、单侧性病变可导致睡眠呼吸障碍。这些病例还说明了延髓脱髓鞘的MS患者在睡眠期间的死亡风险，并表明如果MS患者在睡眠中意外死亡，需要仔细检查其延髓。
