首页> 外文期刊>Journal of Neurology, Neurosurgery and Psychiatry >Neurological picture. Ascending paralysis from malignant leptomeningeal melanomatosis.
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Neurological picture. Ascending paralysis from malignant leptomeningeal melanomatosis.

机译:神经学图片。恶性软脑膜黑色素瘤病使麻痹上升。

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A 32-year-old male with a past medical history of genital herpes and a 1 week history of headaches, presented with nausea, vomiting and diarrhoea, and had a generalised tonic-clonic seizure. His temperature was 38.1degC and CSF laboratory values were: 14 white blood cells (WBC) (100% monocytes), 16 red blood cells, protein 498 mg/dl and glucose 86 mg/dl; serum WBC 12.7 with 75% neutrophils (polymorphomiclear leucocytes). T1 weighted MRI with gadolinium revealed patchy enhancement of the meninges (figure 1A). Serum herpes simplex virus IgG was positive. Viral, herpes simplex virus and bacterial CSF cultures were negative. Acyclovir was started empirically. Two weeks later he suddenly became unresponsive. MRI revealed worsening meningea/temporal lobe enhancement and hydrocephalus (figure IB). Serum WBC was 22.7. An exhaustive work-up for common and exotic infectious diseases, inflammatory disease and neoplastic disease, including CSF studies, was negative. He improved with CSF diversion via ventriculostomy to a GCS-14 until day 17 when he developed acute paraparesis (2/5) (figure 1C). He was taken to the operating theatre for lumbar leptomeningeal and nerve root biopsy. Immunohistochemical staining of the nerve roots revealed tumour cells staining positively for vimentin, the melanoma specific markers HMB45 and Mart-1, and S-100 protein, confirming the diagnosis of meningeal melanomatosis (figure 2). No cutaneous/ocular nevi or melanoma were found on further physical examination conducted by the dermatology and ophthalmology departments. The patient developed ascending paralysis of the upper extremities and then of the cranial nerves (figure ID). The family withdrew medical support and declined a request for autopsy.
机译:一名32岁的男性,曾有生殖器疱疹的病史,头痛的病史为1周,表现为恶心,呕吐和腹泻,并普遍性强直阵挛性癫痫发作。他的体温为38.1摄氏度,脑脊液实验室值为:14个白细胞(WBC)(100%单核细胞),16个红细胞​​,蛋白质498 mg / dl和葡萄糖86 mg / dl;血清白细胞12.7,中性粒细胞含量为75%(多形核白细胞)。带有g的T1加权MRI显示脑膜的斑片状增强(图1A)。血清单纯疱疹病毒IgG为阳性。病毒,单纯疱疹病毒和细菌脑脊液培养均为阴性。阿昔洛韦是凭经验开始的。两周后,他突然变得反应迟钝。 MRI显示脑膜/颞叶增强和脑积水恶化(图IB)。血清白细胞为22.7。对常见和外来传染病,炎性疾病和赘生性疾病(包括CSF研究)进行的详尽检查是阴性的。他通过脑室造口术将CSF转移到GCS-14方面有所改善,直到第17天出现急性轻瘫(2/5)(图1C)。他被带到手术室进行腰部软脑膜和神经根活检。神经根的免疫组织化学染色显示,瘤细胞对波形蛋白,黑色素瘤特异性标志物HMB45和Mart-1以及S-100蛋白呈阳性染色,证实了对脑膜黑素瘤病的诊断(图2)。皮肤科和眼科部门进行的进一步身体检查未发现皮肤/眼痣或黑色素瘤。该患者上肢,然后是颅神经逐渐麻痹(图ID)。该家庭撤回了医疗支持,并拒绝了尸检请求。

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