Involvement of the ventrolateral medulla in parkinsonism with autonomic failure
Involvement of the ventrolateral medulla in parkinsonism with autonomic failure
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DOI：
10.1212/wnl.54.4.963
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发表时间：
2000-02-22
期刊：
影响因子：
9.9
通讯作者：
Parisi, JE
中科院分区：
文献类型：
--
作者：
Benarroch, EE;Schmeichel, AM;Parisi, JE
目的：确定主要表现为直立性低血压（OH）的PD和自主神经功能衰竭（AF）患者的头端腹外侧延髓（RVLM）中酪氨酸羟化酶（TH）神经元是否持续丢失，与多系统萎缩（MSA）和AF患者中发生的情况相似，并确定是否存在烟酰胺丢失，腺嘌呤二核苷酸磷酸（NADPH）黄递酶（NADPH-d）RVLM神经元在两组患者。研究方法：在RVLM TH和NADPH-d神经元的数量进行了评估，从5例疑似PD和OH，6例MSA患者，2例皮质基底节变性和无AF患者，和10例对照组无神经系统疾病史的尸检脑切片。比较各组间的细胞数量，并与其最终的神经病理诊断相关。结果如下：PD和OH患者RVLM中TH神经元的数量与对照组相比无显著差异，但存在明显的个体差异。尽管OH的严重程度相似，但PD患者RVLM中的TH细胞数量显著高于MSA患者（p < 0.06）。作为一个群体，PD和OH患者与对照组相比，RVLM中NADPH-d细胞数量减少，但同样存在明显的个体差异。NADPH-d细胞数量在MSA患者中持续减少且更明显。结论：与MSA患者的情况不同，PD患者RVLM中TH神经元的数量变化很大，不太可能对OH的病理生理学有显著影响。作为一个群体，PD患者RVLM中NADPH-d神经元的数量减少，但一些患者的细胞计数与对照组相似。另一方面，RVLM中的NADPH-d细胞耗竭是MSA中的一致发现，并且可能导致这种疾病中的心肺功能障碍。
Objective: To determine whether patients with PD and autonomic failure (AF), manifested primarily with orthostatic hypotension (OH), have a consistent loss of tyrosine hydroxylase (TH) neurons in the rostral ventrolateral medulla (RVLM), similar to that occurring in patients with multiple system atrophy (MSA) and AF, and to determine whether there is loss of nicotinamide, adenine dinucleotide phosphate (NADPH) diaphorase (NADPH-d) RVLM neurons in both groups of patients. Methods: The numbers of TH and NADPH-d neurons in the RVLM was assessed in brain sections obtained at autopsy from five patients with suspected PD and OH, six patients with MSA, two patients with corticobasal ganglionic degeneration and no AF, and 10 control subjects with no history of neurologic disease. Cell numbers were compared among groups and correlated with their final neuropathologic diagnosis. Results: The number of TH neurons in the RVLM of patients with PD and OH were not significantly different from control subjects, and there were marked individual variations. The TH cell numbers in the RVLM were significantly higher (p < 0.06) in patients with PD than in patients with MSA, despite a similar degree of severity of OH. As a group, patients with PD and OH had reduced numbers of NADPH-d cells in the RVLM compared with control subjects, but again there were marked individual variations. NADPH-d cell numbers were reduced consistently and more markedly in patients with MSA. Conclusion: Unlike the case in patients with MSA, the number of TH neurons in the RVLM is highly variable in patients with PD and is unlikely to contribute significantly to the pathophysiology of OH. As a group, patients with PD have reduced numbers of NADPH-d neurons in the RVLM, but some patients had cell counts similar to control subjects. On the other hand, NADPH-d cell depletion in the RVLM is a consistent finding in MSA and may contribute to cardiorespiratory dysfunction in this disorder.