晨读病例#20200518想象力太匮乏

ear of the lynx

这个耳朵，是怎么像的。

A 37-year-old woman presented with a previous history of learning disability, started when she was 23 years old, with progressive spastic gait, cerebellar signs, cognitive decline, and peripheral neuropathy. Parents, who consented with this publication, were non-consanguineous and the patient had two other affected siblings. Brain MRI disclosed an ultrathin corpus callosum and periventricular white matter abnormalities along with the characterization of the “ear of the lynx” sign [Figure 1]. Molecular analysis of SPG11 revealed a splicing pathogenic variation c.2444 + 1G > C (NM_025137.3), consistent with the diagnosis of spastic paraplegia 11 (SPG11). SPG11 is the most frequent presentation of autosomal recessive hereditary spastic paraplegia (HSP) and has characteristic MRI findings.[1],[2] In investigation HSP, brain MRI is usually more helpful than spinal cord MRI.[3]

还有之前发过的，20岁女性，出生时开始行走困难。查体双下肢痉挛



………………割割割………………………割割割………………………割割割………

说这个lynx耳朵征，相对特异SPG11和15。

仔细看，胼胝体发育不良，是有个尖角的很像这个耳朵。

今天算是对前边知识点的补充了。

………………割割割………………………割割割………………………割割割………

致死性甲硝唑脑病。也有凶残的一面。

………………割割割………………………割割割………………………割割割………

伴有结膜充血和流泪的单侧短暂性神经痛样头痛(short-lasting unilateral neuralgiform headache with conjunctival injection and tearing，SUNCT)是一种罕见的头痛，多见于中老年人，也可见于儿童和青少年。SUNCT和伴有颅自主症状的单侧短暂性神经痛样头痛(short-lasting unilateral neuralgiform headache with cranial autonomic symptoms，SUNA)、丛集性头痛及阵发性偏侧头痛一起归类于三叉自主神经性头痛(trigeminal autonomic cephalagias，TACs)。

2005年Black等总结继发性SUNCT原因：桥小脑角AVM、静脉血管瘤和星形细胞瘤，脑干海绵状血管瘤，眼外伤，艾滋病患者的后颅窝病变，脑血管压迫三叉神经，三叉神经病变，基底压迫(成骨不全症)，脑干背外侧梗死，HIV(颅内无病变)，分泌催乳素的垂体腺瘤，静脉窦平滑肌肉瘤，眶内(颅外)囊状肿瘤，神经纤维瘤病2型，眶内(颅内)肿瘤转移。此后又发现巨泌乳素瘤侵犯海绵窦也可引起SUNCT。文献报道治疗泌乳素瘤患者所用的多巴胺激动剂可以诱发SUNCT，如溴麦角环肽、麦角乙脲、奎高利特和卡麦角林。对于临床诊断为SUNCT的患者，如头颅MRI平扫未发现病变，还应进行头磁共振脑血管造影(MRA)、时间飞跃法磁共振血管成像(3D time of flight magnetic resonance angiography，3D-TOFMRA)和垂体MRI检查，必要时还应进行传染病检查，以除外继发性原因。

就想表达，桥小脑角占位可以表现SUNCT。

表

现SUNCT的鞘瘤。