Neurology department, GZA Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Antwerp, Belgium.
Neurology department, GZA Sint-Augustinus Hospital, Oosterveldlaan 24, 2610 Antwerp, Belgium.
Mult Scler Relat Disord. 2020 Jul;42:102060. doi: 10.1016/j.msard.2020.102060. Epub 2020 Mar 16.
Tumefactive demyelination presents as an aggressive, fast progressive focal demyelinating lesion in the central nervous system, with often devastating clinical outcome if not acutely treated. Correct and early treatment is threatened by its real diagnostic challenge.
We describe a 47-year-old man with tumefactive demyelination and testicular seminoma. He presented with aphasia, cognitive impairment and right sided weakness of the arm and lower face. MRI revealed a lesion characteristic of tumefactive demyelination. Cerebral biopsy confirmed this diagnosis. In addition, a testicular seminoma was diagnosed. Temporal association of demyelination and malignancy was highly suggestive for a paraneoplastic syndrome. He responded well to corticosteroid therapy plus orchiectomy, but a behavioral disturbance remained.
A good knowledge of specific imaging characteristics of tumefactive demyelination can help in early diagnosis. Multiple underlying causes should be considered, including, but not limited to multiple sclerosis. A paraneoplastic syndrome should not be overlooked. This is the fifth case in which seminoma is associated with a paraneoplastic tumefactive demyelination lesion.
肿块样脱髓鞘病变表现为中枢神经系统中侵袭性、快速进展的局灶性脱髓鞘病变，如果不及时治疗，常导致严重的临床后果。但由于其真正的诊断挑战，正确和早期的治疗受到了威胁。
我们描述了一例 47 岁男性，患有肿块样脱髓鞘病变和睾丸精原细胞瘤。他表现为失语、认知障碍以及右侧手臂和下部面部无力。MRI 显示符合肿块样脱髓鞘病变的特征性病变。脑活检证实了这一诊断。此外，还诊断出睾丸精原细胞瘤。脱髓鞘和恶性肿瘤的时间关联高度提示为副肿瘤综合征。他对皮质类固醇治疗加睾丸切除术反应良好，但仍存在行为障碍。
对肿块样脱髓鞘病变的特定影像学特征有较好的了解有助于早期诊断。应考虑多种潜在病因，包括但不限于多发性硬化症。不应忽视副肿瘤综合征。这是第五例与副肿瘤性肿块样脱髓鞘病变相关的精原细胞瘤。
