Fudaba Hirotaka, Ooba Hiroshi, Abe Tatsuya, Kamida Tohru, Wakabayashi Yukihiro, Nagatomi Hirofumi, Fujiki Minoru
Department of Neurosurgery, Oita University Faculty of Medicine, Oita 879-5593 Japan.
Department of Neurosurgery, Oita University Faculty of Medicine, Oita 879-5593 Japan.
J Neurol Sci. 2014 Jul 15;342(1-2):192-6. doi: 10.1016/j.jns.2014.05.006. Epub 2014 May 15.
Cerebral malakoplakia is a very rare chronic inflammatory disease. We herein report the case of a 49-year-old female who presented with a slowly progressive speech disturbance and right hemiparesis. Computed tomography and magnetic resonance imaging showed irregular enhanced mass lesions with numerous scattered areas of calcification in the left insula, thalamus and basal ganglia. Histopathologically, the biopsy specimen showed basophilic laminated inclusion bodies and intracellular and extracellular calculospherules, usually with a typical targetoid appearance (Michaelis-Gutmann bodies). Treatment with antibiotics, bethanechol and ascorbic acid improved her symptoms in association with a decrease in the abnormal calcification and enhancement. The cerebral malakoplakia mimicked a brain tumor in terms of the patient's clinical course and neuroradiological image findings; however, it was successfully cured with medical treatment. This case provides evidence that the pathogenesis of cerebral malakoplakia is deeply tied to bacterial infection and that medical treatment is effective in cases of this disease.
脑软斑病是一种非常罕见的慢性炎症性疾病。我们在此报告一例49岁女性病例,该患者表现为缓慢进展的言语障碍和右侧偏瘫。计算机断层扫描和磁共振成像显示左侧岛叶、丘脑和基底神经节有不规则强化的肿块病变,伴有大量散在的钙化区域。组织病理学检查显示,活检标本中有嗜碱性层状包涵体以及细胞内和细胞外钙化球,通常具有典型的靶样外观(米氏小体)。抗生素、氨甲酰甲胆碱和维生素C治疗改善了她的症状,同时异常钙化和强化减少。就患者的临床病程和神经放射学影像表现而言,脑软斑病类似脑肿瘤;然而,通过药物治疗成功治愈。该病例证明脑软斑病的发病机制与细菌感染密切相关,且药物治疗对该病有效。