Uchida Daiki, Nakatogawa Hirokazu, Inenaga Chikanori, Tanaka Tokutaro
Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Department of Neurosurgery, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
World Neurosurg. 2018 Jun;114:326-329. doi: 10.1016/j.wneu.2018.03.184. Epub 2018 Apr 3.
Lhermitte-Duclos disease (LDD) is a rare neurologic disease that causes a hamartomatous lesion in the cerebellum. LDD is usually associated with mass lesion effects, but with appropriate surgical treatment, the clinical course is usually benign. We report a rare case of a patient with LDD with contrast enhancement on magnetic resonance imaging (MRI) who died as a result of intratumoral hemorrhage.
LDD was diagnosed in a 59-year-old woman after a routine MRI. She did not present with any symptoms initially or after follow-up MRI suggested minor hemorrhage in the tumor. Eleven months after her first visit to our department, she suddenly lost consciousness, and computed tomography revealed massive intratumoral hemorrhage. Surgical decompression was done, but she died 17 days after surgery. Histopathologic findings were consistent with LDD.
Hemorrhagic events can occur with LDD, even though the growth of the lesion is slow. Long-term close follow-up of patients with LDD is needed, especially patients in whom MRI shows atypical enhancement. To avoid possible hemorrhagic events, surgical treatment should be considered even if only subtle changes are seen on neuroimaging.
Lhermitte-Duclos病（LDD）是一种罕见的神经系统疾病，可导致小脑出现错构瘤样病变。LDD通常伴有占位性病变效应，但经过适当的手术治疗，临床病程通常为良性。我们报告一例罕见的LDD患者，其磁共振成像（MRI）显示有对比增强，最终因肿瘤内出血死亡。
一名59岁女性经常规MRI检查后被诊断为LDD。最初她没有任何症状，后续MRI提示肿瘤内有少量出血时她仍无症状。首次就诊于我科11个月后，她突然失去意识，计算机断层扫描显示肿瘤内大量出血。进行了手术减压，但她在术后17天死亡。组织病理学检查结果与LDD相符。
尽管LDD病变生长缓慢，但仍可能发生出血事件。需要对LDD患者进行长期密切随访，尤其是MRI显示非典型强化的患者。为避免可能的出血事件，即使神经影像学仅显示细微变化，也应考虑手术治疗。
