Song Misa, Kimura Hayato, Ogiyama Hideharu, Nishigami Takayuki
Department of Diagnostic Pathology, Itami City Hospital, Itami, Hyogo, Japan.
Gastrointestinal Medicine, Itami City Hospital, Itami, Hyogo, Japan.
Pathol Int. 2018 Jun;68(6):382-387. doi: 10.1111/pin.12668. Epub 2018 Apr 6.
Multiple system atrophy (MSA) is a spinocerebellar degenerative disease characterized by cerebellar ataxia, parkinsonism, and autonomic failure. A 75-year-old woman who had suffered from dysphagia and dysuria under a diagnosis of probable MSA with predominant cerebellar ataxia underwent autopsy. Eosinophilia was seen extensively in the muscle layer of the esophagus and urinary bladder. Eosinophilic infiltration to the esophagus was localized in the smooth muscle layer and could be considered as "eosinophilic esophageal myositis" identified in patients with nutcracker esophagus and jackhammer esophagus. Dense eosinophilia was present within the smooth muscle layer of the urinary bladder along with muscle fiber degeneration. We suspected a neuropathic etiology associated with MSA as the cause of the histological changes in the esophagus and urinary bladder; however, the possibility that some other disease might also have been responsible for the eosinophilic infiltration of the muscle layer cannot be denied. To our knowledge, this is the first report showing localized eosinophilia in the muscle layers of the esophagus and urinary bladder in the same patient. Although localized eosinophilia in visceral muscle has not been understood well, our case suggests the possibility that it is a feature of functional motility disorders and may have a neuropathic etiology.
多系统萎缩(MSA)是一种脊髓小脑退行性疾病,其特征为小脑共济失调、帕金森综合征和自主神经功能衰竭。一名75岁女性,诊断为可能的以小脑性共济失调为主的MSA,伴有吞咽困难和排尿困难,接受了尸检。在食管和膀胱的肌层广泛可见嗜酸性粒细胞增多。食管的嗜酸性粒细胞浸润局限于平滑肌层,可被视为在胡桃夹食管和手风琴样食管患者中发现的“嗜酸性粒细胞性食管肌炎”。膀胱平滑肌层内存在密集的嗜酸性粒细胞增多,同时伴有肌纤维变性。我们怀疑与MSA相关的神经源性病因是食管和膀胱组织学改变的原因;然而,不能排除其他一些疾病也可能导致肌层嗜酸性粒细胞浸润的可能性。据我们所知,这是第一份报告显示同一患者食管和膀胱肌层出现局限性嗜酸性粒细胞增多。尽管内脏肌的局限性嗜酸性粒细胞增多尚未被很好地理解,但我们的病例提示其可能是功能性运动障碍的一个特征,并且可能有神经源性病因。