Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Tokyo, Japan.
Department of Neurology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan.
J Parkinsons Dis. 2016 Apr 2;6(2):325-34. doi: 10.3233/JPD-150773.
Loss of intermediolateral nucleus (IML) neurons is considered to play a major role in orthostatic hypotension (OH) of multiple system atrophy (MSA). In Parkinson disease (PD) and dementia with Lewy bodies (DLB), autonomic phenomena such as OH are common and attributed to dysfunction of sympathetic, parasympathetic, and visceral autonomic neurons. However, apart from MSA, few reports have focused on the neuropathologic aspects in PD and DLB. Here we assessed IML degeneration as well as the fine myelinated fibers (FFs; maximum diameter less than 3 μm) considered to be preganglionic sympathetic nerve fibers derived from IML neurons in PD, DLB, MSA, and age-matched normal controls (NC).
We counted IML neurons and measured the diameter and number of myelinated fibers of the ventral root at the level of the 12th thoracic segment.
Compared to NC, number of IML neurons and density of FF were significantly reduced in PD (53% and 67%), DLB (47% and 71%) and MSA (27% and 42%). Compared to combined group of PD and DLB without OH (OH-), IML neurons in combined group of PD and DLB with OH (OH+) were significantly reduced (77%). Compared to NC, FF densities in OH-, OH+ were significantly reduced (74% and 59%). The mean ratio of small to large myelinated fibers in OH+ (1.18), but not that in OH-(1.58), was significantly lower than that in NC (3.17).
We present neuropathological evidence that IML neurons and FFs in the ventral root are reduced in PD and DLB and that the reduction was more severe in the combined group of OH+ than in OH-.
中侧核(IML)神经元的丧失被认为在多系统萎缩(MSA)的直立性低血压(OH)中起主要作用。在帕金森病(PD)和路易体痴呆(DLB)中,OH 等自主现象很常见,归因于交感神经、副交感神经和内脏自主神经元的功能障碍。然而,除了 MSA 之外,很少有报道关注 PD 和 DLB 中的神经病理学方面。在这里,我们评估了 IML 退化以及被认为源自 IML 神经元的节前交感神经纤维的精细有髓纤维(FF;最大直径小于 3μm),在 PD、DLB、MSA 和年龄匹配的正常对照(NC)中。
我们计数了 IML 神经元,并测量了第 12 胸段水平的脊神经根的髓鞘纤维直径和数量。
与 NC 相比,PD(53%和 67%)、DLB(47%和 71%)和 MSA(27%和 42%)中 IML 神经元的数量和 FF 的密度显著降低。与无 OH 的 PD 和 DLB 联合组(OH-)相比,OH+的 PD 和 DLB 联合组的 IML 神经元显著减少(77%)。与 NC 相比,OH-和 OH+中的 FF 密度显著降低(74%和 59%)。OH+(1.18)的小到大有髓纤维的平均比值,而不是 OH-(1.58)的比值,显著低于 NC(3.17)。
我们提供了神经病理学证据,表明 PD 和 DLB 中脊神经根的 IML 神经元和 FF 减少,且在 OH+的联合组中比在 OH-中更严重。