Neurodegeneration involving putative respiratory neurons in Perry syndrome
Neurodegeneration involving putative respiratory neurons in Perry syndrome
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DOI：
10.1007/s00401-007-0246-1
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发表时间：
2008-02-01
影响因子：
12.7
通讯作者：
Benarroch, Eduardo E.
中科院分区：
文献类型：
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作者：
Tsuboi, Yoshio;Dickson, Dennis W.;Benarroch, Eduardo E.
本研究的目的是评估腹侧延髓神经元参与佩里综合征（常染色体显性帕金森综合征，伴抑郁、体重减轻和中枢通气不足）患者呼吸节律发生和化疗敏感性的可能性。以前的佩里综合征的神经病理学报告显示，黑质神经元丢失，没有或很少有路易体，没有tau蛋白夹杂物。前Botzinger复合体（preBotC）的腹外侧延髓，确定其神经激肽-1受体（NK-1 R）的免疫反应，在呼吸节律和呼吸化学敏感性的延髓中缝中的肾上腺素能神经元中发挥重要作用，但其潜在的参与佩里综合征尚未得到解决。我们对一例临床诊断为佩里综合征的新尸检病例进行了临床和神经病理学研究，包括免疫组化检查。我们的病人在41岁时出现帕金森综合征。随后，佩里综合征的所有主要特征都出现了。他在46岁时死于呼吸衰竭和败血症。苏木精-伊红染色显示髓质无明显病理变化。然而，NK-1 R，酪氨酸羟化酶（TH）和色氨酸羟化酶（TrOH）的免疫反应神经元显着减少，在延髓腹外侧区相比，控制。延髓中缝和腹侧表面的多巴胺能神经元也有丢失。黑质严重神经元丢失，无α-突触核蛋白或tau蛋白病理学，但腹外侧髓质NK-1 R和TH免疫反应性神经元丢失，以及延髓中缝和腹外侧髓质中多巴胺能神经元丢失可能是佩里综合征的病理学标志。
The objective of this study was to assess the potential involvement of ventral medullary neurons implicated in respiratory rhythmogenesis and chemosensitivity in a patient with Perry syndrome (autosomal dominant parkinsonism associated with depression, weight loss and central hypoventilation). Previous neuropathologic reports in Perry syndrome demonstrated neuronal loss in the substantia nigra with no or few Lewy bodies and no tau inclusions. Neurons in the pre-Botzinger complex (preBotC) of the ventrolateral medulla, identified by their immunoreactivity for neurokinin-1 receptors (NK-1R), play an essential role in respiratory rhythmogenesis and serotonergic neurons in the medullary raphe in respiratory chemosensitivity, but their potential involvement in Perry syndrome has not yet been addressed. We conducted clinical and neuropathologic studies including immunohistochemistry examination in a new autopsied case clinically diagnosed as Perry syndrome. Our patient presented with parkinsonism at age 41. Subsequently, all cardinal features of Perry syndrome developed. He died of respiratory failure and sepsis at age 46. Hematoxylin-eosin staining revealed no significant pathology in the medulla. However, NK-1R, tyrosine hydroxylase (TH) and tryptophan hydroxylase (TrOH) immunoreactive neurons were significantly reduced in the ventrolateral medulla compared to controls. There was also loss of serotonergic neurons in the medullary raphe and ventral medullary surface. Severe neuronal loss in the substantia nigra, without alpha-synuclein or tau pathology but with loss of NK-1R and TH immunoreactive neurons in the ventrolateral medulla, and loss of serotonergic neurons in the medullary raphe and ventrolateral medulla may be a pathologic hallmark of Perry syndrome.