Benarroch E E, Schmeichel A M, Parisi J E
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Neurology. 2000 Feb 22;54(4):963-8. doi: 10.1212/wnl.54.4.963.
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.
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.
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.
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.
确定主要表现为体位性低血压(OH)的帕金森病(PD)合并自主神经功能衰竭(AF)患者,其延髓头端腹外侧区(RVLM)的酪氨酸羟化酶(TH)神经元是否像多系统萎缩(MSA)合并AF患者那样出现一致性缺失,并确定两组患者的RVLM中烟酰胺腺嘌呤二核苷酸磷酸(NADPH)黄递酶(NADPH-d)神经元是否缺失。
对5例疑似PD合并OH患者、6例MSA患者、2例无AF的皮质基底节变性患者以及10例无神经疾病病史的对照者尸检获得的脑切片中RVLM的TH和NADPH-d神经元数量进行评估。比较各组间的细胞数量,并将其与最终神经病理学诊断结果相关联。
PD合并OH患者RVLM中TH神经元数量与对照者无显著差异,且存在明显个体差异。尽管OH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合并OH患者RVLM中的NADPH-d细胞数量与对照者相比减少,但同样存在明显个体差异。MSA患者的NADPH-d细胞数量持续且更明显地减少。
与MSA患者不同,PD患者RVLM中TH神经元数量高度可变,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中的NADPH-d神经元数量减少,但部分患者的细胞计数与对照者相似。另一方面,RVLM中NADPH-d细胞缺失是MSA中的一个一致发现,可能导致该疾病的心肺功能障碍。