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右侧肢体无力及反应迟钝20天。。。

2019-08-06 不详 影像园

进行性多灶性脑白质病
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患者20天前突发右侧肢体无力及反应迟钝，无明显麻木及疼痛感。HIV病史2年。血淋巴细胞亚群分析示：辅助/诱导性T淋巴细胞（CD3CD4）↓，抑制/细胞毒性T淋巴细胞（CD3CD8）(%)70.62↑， NK细胞↓ 。

【所属科室】

神经内科

【基本资料】

患者，男，45岁

【主诉】

右侧肢体无力及反应迟钝20天

【现病史】

患者20天前突发右侧肢体无力及反应迟钝，无明显麻木及疼痛感。HIV病史2年。血淋巴细胞亚群分析示：辅助/诱导性T淋巴细胞（CD3CD4）↓，抑制/细胞毒性T淋巴细胞（CD3CD8）(%)70.62↑， NK细胞↓ 。

【影像图片】











【影像表现】

双额叶、左顶叶白质，左侧半卵圆中心及放射冠区多发融合的片状长T1长T2信号影，T2FLAIR上呈高信号。DWI、ADC示病灶边缘弥散轻度受限，增强扫描病灶未见明显强化。MRS显示NAA峰明显下降，Cho峰明显上升。
 
【结果】

临床诊断：艾滋病相关进行性多灶性脑白质病变。

穿刺结果：白质髓鞘多发灶丢失和含有核内嗜酸性物质的增大的少突胶质细胞。电镜下可见在核内边缘的圆形和丝状结构的聚集性JC病毒。

免疫组化：淋巴细胞CD20（+）、CD3（+）；浆细胞CD138（+）；胶质细胞GFAP（+），Oligo-2（+）；IDH-1（-）。
 
【病例小结】

进行性多灶性脑白质病（Progressive multifocal leukoencephalopathy，PML）是一种脱髓鞘疾病，是免疫功能低下者体内JC病毒激活的结果。最常见于HIV感染者，其次是正在接受那他珠单抗治疗的MS患者，JC病毒在人类中广泛存在，潜伏于肾脏和脑，通常无症状。在PML患者，JC病毒感染少突胶质细胞，导致髓鞘形成障碍或局部脱髓鞘 。HIV感染患者一旦CD4计数小于100，发生PML的危险性增高。随着AIDS发病率逐年增多及中晚期AIDS患者的增多，PML在艾滋病患者中也日益增多，国内本病常见于肾移植、抗肿瘤治疗和骨髓异常增生的病人。临床表现根据受累部位、大小不同而多种多样，缺乏特异性，最常见的症状是运动无力，视力障碍和精神状态改变，包括认知异常和行为/性格的改变。JCV是一种嗜神经性病毒，可以专一地作用于少突胶质细胞，引起严重、广泛和多阶段的脱髓鞘病变，典型的病理改变为中枢神经系统白质内进行性脱髓鞘，不伴炎症反应和坏死，镜检可见皮质下白质区的脱髓鞘病变，病变周围有胞核扩大的少突胶质细胞，其内有嗜酸性核包涵体，结构不清。病变区的星形胶质细胞也有不同程度增大，内见分叶状的细胞核和嗜酸性包涵体。

MRI：单发或多发病变，边界不清，不对称分布, 多灶性, 位于皮质下，T1WI上早期病变为稍低信号，随着病情进展，低信号区不断扩大，伴有病变区融合、皮质萎缩和脑室扩大等变化，T2WI及T2flair上为高信号，典型病变通常不对称，且无水肿、占位及增强表现，增强扫描通常无强化，大多数PML 病变出现增强表现时，表明有炎症、血脑屏障破坏和水肿的存在，PML在DWI上的表现因疾病处于不同的阶段而有差异，典型活动期的PML病变表现为中央低信号区和边缘高信号区。这个边缘通常是不完整的，是活动性感染的标志。伴随着病变的进展，DWI上外周病变区的高信号随着病情发展逐渐降低。MRS：NAA峰降低，可能反应神经元的损失，Cho峰升高，表明神经细胞细胞膜和髓鞘的损伤，新发病变部位的Cho上升程度较大。

本病需与以下疾病进行鉴别：HIV脑炎，病灶大多位于脑室旁,对称分布，脑萎缩的程度一般较重，一般呈长T1长T2信号，可见弥散受限。急性播散性脑脊髓炎，患者常有明确的发热前驱疾病或免疫接种病史，呈单相急性起病，灰质和白质常同时受累，急性期常有强化。肾上腺脑白质营养不良，病变特点为从后向前逐渐累及，即从枕、顶、颞叶开始，最后影响额叶，侧脑室三角周围白质病变多对称呈蝶状。

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    2019-11-18 wxl882001

    了解一下

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=375508, encodeId=65663e550818, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Mon Nov 18 19:58:13 CST 2019, time=2019-11-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=374147, encodeId=e3b63e4147fa, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=81, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Sun Oct 20 19:37:27 CST 2019, time=2019-10-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=371368, encodeId=7f953e136842, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=76, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Fri Aug 16 20:53:44 CST 2019, time=2019-08-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=371015, encodeId=e0233e10159d, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Fri Aug 09 20:42:02 CST 2019, time=2019-08-09, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1490602, encodeId=b8001490602a6, content=<a href='/topic/show?id=79d33e740ae' target=_blank style='color:#2F92EE;'>#反应迟钝#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37740, encryptionId=79d33e740ae, topicName=反应迟钝)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=5d7f8793980, createdName=yb6563, createdTime=Thu Aug 08 14:18:00 CST 2019, time=2019-08-08, status=1, ipAttribution=)]
    2019-10-20 wxl882001

    了解一下

    0

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    2019-08-16 wxl882001

    了解一下

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=375508, encodeId=65663e550818, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Mon Nov 18 19:58:13 CST 2019, time=2019-11-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=374147, encodeId=e3b63e4147fa, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=81, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Sun Oct 20 19:37:27 CST 2019, time=2019-10-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=371368, encodeId=7f953e136842, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=76, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Fri Aug 16 20:53:44 CST 2019, time=2019-08-16, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=371015, encodeId=e0233e10159d, content=了解一下, beContent=null, objectType=article, channel=null, level=null, likeNumber=73, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6KcicAVC1MwuOgo3iaicb1Imgk1yIfnvr6oXzibcAFB6puAyNJXbzWhNuLqaFYBVRWjh7rghkvFLPxMAfD5ib3nXwVD/0, createdBy=bed21972958, createdName=wxl882001, createdTime=Fri Aug 09 20:42:02 CST 2019, time=2019-08-09, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1490602, encodeId=b8001490602a6, content=<a href='/topic/show?id=79d33e740ae' target=_blank style='color:#2F92EE;'>#反应迟钝#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=37740, encryptionId=79d33e740ae, topicName=反应迟钝)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=5d7f8793980, createdName=yb6563, createdTime=Thu Aug 08 14:18:00 CST 2019, time=2019-08-08, status=1, ipAttribution=)]
    2019-08-09 wxl882001

    了解一下

    0

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    2019-08-08 yb6563

    #反应迟钝#

    0

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