Awang Saufi M, Saiful Nayan M, Madhavan Mohan, Abdullah Jafri, Tharakan John K
J Med Case Rep. 2009 Aug 5;3:7407. doi: 10.4076/1752-1947-3-7407.
We report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease.
The first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale. The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes. The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent.
These cases illustrate the importance of considering a demyelinating disease in the differential diagnosis of a mass lesion. Critical analyses of clinical presentations coupled with good physical examination are vital in assisting clinicians to reach the correct diagnosis.
我们报告了3例具有肿瘤样表现的脱髓鞘疾病病例。这一信息对于神经外科医生和神经内科医生尤为重要,他们应意识到炎性脱髓鞘疾病可能表现为肿块样病变,在临床和放射学上与肿瘤难以区分,尤其是当没有该疾病时间上播散的证据时。
首例患者是一名42岁的马来族女性,出现亚急性起病的进行性四肢瘫并伴有尿失禁。其脊柱磁共振成像显示颈5至颈7水平髓内有一病变。她接受了手术,活检提示为脱髓鞘疾病。回顾病史发现有两次急性起病的神经功能缺损发作且部分恢复,其脑部磁共振成像显示半卵圆中心有脱髓鞘斑块。第二例患者是一名16岁的马来族男孩,表现为颅内压升高的症状。脑部计算机断层扫描显示梗阻性脑积水,第四脑室附近有一病变。插入了外部脑室引流管。随后进行了立体定向活检,组织病理学报告为脱髓鞘。回顾病史发现有类似发作,发作间期完全恢复。第三例患者是一名28岁的马来族男性,出现急性双侧视力丧失和意识模糊。其脑部磁共振成像显示右侧颞顶叶区域有一个大的肿块样病变。活检结果与脱髓鞘疾病相符。对该患者的复查发现是双侧视乳头炎而非视乳头水肿。双侧视觉诱发电位延长。在所有3例病例中,由于患者不同意,未进行腰椎穿刺以研究脑脊液。
这些病例说明了在肿块样病变的鉴别诊断中考虑脱髓鞘疾病的重要性。对临床表现进行批判性分析并结合良好的体格检查对于帮助临床医生做出正确诊断至关重要。