Royal (Dick) School of Veterinary Studies and The Roslin Institute, The University of Edinburgh, Midlothian, United Kingdom.
J Vet Intern Med. 2019 Sep;33(5):2302-2311. doi: 10.1111/jvim.15567. Epub 2019 Jul 22.
Equine dysautonomia (ED) causes degeneration and loss of autonomic neurons. Approximately 50% of chronic cases recover, but it is unclear how they survive neuronal loss.
To assess lesions, autonomic neuron numbers, interstitial cells of Cajal (ICC), and neurodegeneration in recovered cases.
Thirteen cases (group ED), euthanized 10.3 ± 5.2 (1-16) years from diagnosis and 6 age-matched controls (group C).
Prospective, case control; routine post mortem examination, neuron counts in peripheral and enteric ganglia and immunohistochemical assessment of neural networks (Protein gene product [PGP] 9.5), ICC (c-kit), and neurodegeneration (beta-amyloid precursor protein and ubiquitin) in intestine.
Postmortem findings in group ED were small intestinal dilation (4/12, 33%) and muscular hypertrophy (4/12, 33%), and gastric mucosal hypertrophy (3/11, 27%) and ulceration (4/11, 36%). Neuron density was lower in group ED (mean 39% lower for cranial cervical ganglion [P < .001], median 44% lower in celiacomesenteric ganglion [P = .01]). In intestine, neuronal depletion was worst in ileum (median 100% lower in submucosal plexus [P < .001], 91% lower in myenteric plexus [P = .004]). Group ED had less PGP 9.5 staining in ileal myenteric plexus (mean 66% lower [P = .04]) and circular muscle (median 75% lower [P = .006]). In ileum, there was less c-kit staining in myenteric plexus (median 57% lower [P = .02]) but not muscularis externa. Beta-amyloid precursor protein and ubiquitin results were not indicitive of neurodegeneration.
Intact ICC in muscularis externa might help maintain motility after neuronal loss. Treatment supporting ICC function warrants investigation.
马的自主神经病变（ED）导致自主神经元变性和丧失。大约 50%的慢性病例会康复，但尚不清楚它们是如何在神经元丧失的情况下存活的。
评估恢复病例中的病变、自主神经元数量、Cajal 间质细胞（ICC）和神经退行性变。
13 例（ED 组），诊断后 10.3±5.2（1-16）年安乐死，6 例年龄匹配的对照组（C 组）。
前瞻性病例对照；常规尸检，检测外周和肠神经节中的神经元计数，以及肠道中神经网络（蛋白基因产物[PGP]9.5）、ICC（c-kit）和神经退行性变（β-淀粉样前体蛋白和泛素）的免疫组织化学评估。
ED 组的尸检结果为小肠扩张（4/12，33%）和肌肉肥大（4/12，33%），以及胃黏膜肥大（3/11，27%）和溃疡（4/11，36%）。ED 组的神经元密度较低（颅颈神经节平均低 39%[P<0.001]，腹腔肠系膜神经节中位数低 44%[P=0.01]）。在肠道中，回肠的神经元耗竭最为严重（黏膜下神经丛中位数低 100%[P<0.001]，肌间神经丛中位数低 91%[P=0.004]）。ED 组在回肠肌间神经丛中的 PGP 9.5 染色较少（平均低 66%[P=0.04]）和环形肌（中位数低 75%[P=0.006]）。在回肠中，肌间神经丛中的 c-kit 染色较少（中位数低 57%[P=0.02]），但外肌层没有。β-淀粉样前体蛋白和泛素的结果并不表明存在神经退行性变。
外肌层中完整的 ICC 可能有助于在神经元丧失后维持运动。支持 ICC 功能的治疗值得进一步研究。
