系统性硬化症伴小脑液化性坏死。
Systemic sclerosis associated with colliquative necrosis in the cerebellum.
作者信息
Ricci Alessandro, Di Vitantonio Hambra, De Paulis Danilo, Del Maestro Mattia, Dehcordi Soheila Raysi, Murrone Domenico, Coletti Gino, Calvisi Giuseppe, Galzio Renato Juan
机构信息
Department of Neurosurgery, San Salvatore city Hospital, L'Aquila, Italy.
Department of Life, Health & Environmental Sciences (MESVA), University of L'Aquila, Italy.
出版信息
Surg Neurol Int. 2017 Apr 5;8:44. doi: 10.4103/sni.sni_401_16. eCollection 2017.
BACKGROUND
The scleroderma is a complex autoimmune collagen disorder that can affect many organs simultaneously, as it occurs in the systemic sclerosis (SS), or only the skin, as it occurs in the localized scleroderma (LS). The neurological presentation is extremely uncommon, and even more uncommon are the symptoms of the scleroderma in the cerebellum.
CASE DESCRIPTION
We report the case of a 56-year-old male with cerebellar lesions mimicking a brain abscess. After surgical excision, the histopathological diagnosis deposed for an ischemic necrosis caused by a vasculopathy. All the bacteriological and viral exams were negative, whereas the rheumatologic tests were compatible with the scleroderma pattern.
CONCLUSION
Up to now, the literature has described only 5 cases of scleroderma in the posterior cranial fossa. The authors report a case of SS causing colliquative necrosis in the cerebellum. Pathogenetic mechanisms, clinical aspects, and radiological features are discussed along with the pertinent literature.
背景
硬皮病是一种复杂的自身免疫性胶原疾病，可同时影响多个器官，如系统性硬化症（SS）；或仅累及皮肤，如局限性硬皮病（LS）。神经系统表现极为罕见，而硬皮病累及小脑的症状则更为罕见。
病例描述
我们报告一例56岁男性，其小脑病变酷似脑脓肿。手术切除后，组织病理学诊断为血管病变导致的缺血性坏死。所有细菌学和病毒学检查均为阴性，而风湿学检查结果符合硬皮病类型。
结论
迄今为止，文献仅描述了5例后颅窝硬皮病。作者报告了一例系统性硬化症导致小脑液化性坏死的病例。结合相关文献对发病机制、临床特征及影像学表现进行了讨论。
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