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【经验交流】Resident and Fellow Section住院医师教育 来自Neurology&[精华]
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１１６. Massive cerebral edema after CT myelography脊髓造影后显著的脑水肿An optical illusionCorrespondence to Dr. Rabinstein: rabinstein.alejandro{at}mayo.eduA 74-year-old woman underwent myelography with iohexol to exclude a CSF leak. Three days later, her son noticed mild facial asymmetry and took her back to the hospital. Neurologic status was at baseline except for minimal left nasolabial flattening. Initial head CT appeared to show diffuse cerebral edema (figure, A), but the following morning the appearance had normalized (figure, B). Based on the spontaneous clinicoradiologic improvement, we hypothesize that an illusion of cerebral edema was caused by residual iohexol. Although not reported with iohexol, older agents can cause hyperdense gray matter and can accumulate in sulci.1一名７４岁的女性患者使用碘海醇进行脊髓造影除外脑脊液漏。３天后，患者儿子注意到她轻微的面部不称，遂再次入院。神经检查除外轻微的左侧鼻唇沟变浅外与之前一样。初步的头ＣＴ显示广泛的脑水肿（图Ａ），但是第二天早上即恢复正常（图Ｂ）。基于临床放射影像的自发改善，我们假设脑水肿是由于残存的碘海醇导致。尽管使用碘海醇没有这方面的报道，但是之前几代的造影剂可能导致灰质高密度以及可能在脑沟聚集。
FIGURE Neuroimaging findings at presentation and the following dayNoncontrast CT scan at presentation (A) shows loss of the sulcal pattern and gray–white distinction, relatively decreased attenuation of the white matter, deep gray structures, and brainstem, and effacement of the basal cisterns and fourth ventricle. Repeat noncontrast CT scan (B) shows marked improvement. Also evident are bilateral deep brain stimulator leads and an old right retinal detachment.非增强ＣＴ显示脑沟和灰白质界限模糊，相对的白质区、深部灰质结构、脑干衰减下降和基底池和四脑室的减小（Ａ）。复查ＣＴ显示显著地改善（B），附见双侧脑深部刺激电极和右侧视网膜剥离。http://www.neurology.org/content/83/18/e170.full?sid=27dad5db-9a91-4d
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117. Pisa syndrome in Parkinson disease见于帕金森病患者的比萨综合征A 68-year-old man with Parkinson disease of 5 years' duration treated with levodopa/carbidopa and ropinirole developed progressive lateral postural flexion of the trunk in the last 6 months, despite adequate control of symptoms. A spinogram showed curvature of more than 10°. The patient was diagnosed elsewhere with scoliosis. On examination in supine position, abnormal trunk posture disappeared almost entirely, ruling out fixed bony deformity. Supine X-ray confirmed significant improvement (figure). In this context, Pisa syndrome may correspond to axial dystonia.1 Scoliosis and Pisa syndrome are not synonymous, as the latter is typically reducible by passive mobilization or when lying down.一名68岁的男性有帕金森病史的患者在使用5年左旋多巴/卡比多巴和罗匹尼罗后出现进展性躯干侧弯6个月，经管帕金森症状得到了适当的控制。脊柱x线显示侧弯超过10度。患者在其他医院诊断为脊柱侧凸。在仰卧位时异常的躯干姿势完全消失，这帮助排除了骨性畸形。卧位的x线证实了这种卧位的显著改善。在这种情况下，比萨综合征可能符合这种轴性的肌张力障碍。脊柱侧凸和比萨综合征并非同义词，后者通常通过被动运动或平躺能够减轻。 FIGURE Spinogram and supine X-ray(A) Spinogram while standing shows curvature of over 10°. (B) Supine X-ray confirms sig however, supine films are still abnormal.A、脊柱x线显示站立位弯曲度超过10度。B、平卧x线显示显著地改善，然而卧位x线仍然是异常的。
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118. Magnetic resonance susceptibility effect for acute isolated cortical vein thrombosis急性孤立性皮层静脉血栓磁敏感效应A 38-year-old woman using hormonal contraception presented with right-sided abdominal and arm clonic seizures, right hemiparesis, hypesthesia, and nausea. She denied headache. D-dimers were 350 ng/mL (laboratory reference ≤500 ng/mL). CT showed a small left parietal hemorrhagic infarct with adjacent hyperdense cortical vein (figure 1). MRI 15 hours after onset showed magnetic susceptibility effect on susceptibility-weighted imaging (SWI, figure 2). Isolated cortical vein thrombosis is present in 5% of patients with cerebral venous thrombosis (CVT).1 Negative D-dimer and absence of headache does not exclude CVT1 and SWI is useful for confirmation of the diagnosis.一名38岁使用激素避孕的女性患者出现右侧腹部和手臂肌阵挛，右侧轻瘫和感觉减退，伴恶心。患者否认头痛，D二聚体为350 ng/mL (正常参考≤500 ng/mL)。CT显示左侧顶叶出血性梗死伴相邻皮层静脉高密度。发病15小时后MRI显示SWI相异常。孤立的皮层静脉血栓占脑静脉窦血栓的5%。D二聚体阴性和无头痛不能除外静脉窦血栓，SWI有助于确定诊断。 FIGURE 1 Baseline noncontrast CT scanAxial CT scan shows subtle hemorrhagic infarction in left parietal region ( A) with associated hyperdense cortical vein ( B).轴位CT显示左侧顶叶轻微的出血性脑梗死（白箭A），伴皮层静脉的高密度征（黑箭B）。 FIGURE 2 MRI susceptibility-weighted imaging 15 hours after onsetAxial MRI with susceptibility-weighted imaging shows magnetic susceptibility effect in left parietal area (A) and ipsilateral cortical vein ( B). MRI time-of-flight venography was negative (not shown).轴位MRI SWI相显示左侧顶叶区和同侧皮层静脉磁敏感异常（箭，B图），TOFMRV没有发现异常（没有提供图片）。http://www.neurology.org/content/83/20/e178.full?sid=aae9d34f-20b5-4435-bf85-b7e
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119. Hyperdense posterior cerebral artery sign大脑后动脉高密度征A 38-year-old man presented to the emergency department with right-sided weakness, sensory loss, and hemianopsia. CT head showed a left hyperdense posterior cerebral artery sign (HPCAS) (figure 1). CT angiogram of the head confirmed a left posterior cerebral artery (PCA) occlusion (figure 2). MRI demonstrated a left PCA infarct. Transesophageal echocardiogram revealed an atrial septal aneurysm and patent foramen ovale. He was treated with antiplatelet therapy. The HPCAS has been considered a marker for acute ischemia in the PCA territory.1 Recognizing the HPCAS on CT in acute stroke may help in the diagnosis and treatment of thromboembolic PCA branch occlusion.38岁男性出现右侧肢体无力、感觉缺失和偏盲入急诊。CT显示左侧大脑后动脉高密度征。CT血管造影证实左侧大脑后动脉闭塞。MRI显示左侧大脑后动脉区梗死。经食道超声发现房间隔瘤和卵圆孔未闭。给予患者抗血小板治疗。大脑后动脉高密度征被认为是大脑后动脉分布区急性梗死的标志，在急性卒中患者识别大脑中动脉高密度征有助于确立诊断和帮助治疗。 FIGURE 1 CT headCT head shows a left hyperdense posterior cerebral artery sign (red arrows).CT显示左侧大脑后动脉高密度征（红箭）。 FIGURE 2 CT angiographyCT angiography of the head demonstrates occlusion of the left posterior cerebral artery (red arrows).CT血管造影显示左侧大脑后动脉闭塞（红箭）。
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120. Spontaneous amyloid-related imaging abnormalities in a cognitively normal adult认知功能正常的成年人中出现的自发性淀粉样变类似影像学异常表现An 81-year-old cognitively normal man (APOE ε3/ε3) without risk factors had amyloid-related imaging abnormalities with sulcal effusions and edema (ARIA-E), siderosis, and microhemorrhages (ARIA-H) on MRI (figure 1).1 He was identified from 1,006 participants followed with 3,385 MRIs in
in Alzheimer's Disease Neuroimaging Initiative (ADNI) 2/ADNI Grand Opportunities. Amyloid standard uptake value ratio on PET was 1.85 (positive) (figure 2). ARIA-E and associated ARIA-H can be observed in cognitively normal elderly without the APOE ε4 risk allele, who have no prior microhemorrhages, and who are not receiving amyloid-modifying treatments. Focal amyloid deposits around the region of ARIA-H suggest that cerebral amyloid angiopathy may be responsible for the occurrence of ARIA in this case.一名81岁没有风险因素的认知功能正常男性(APOE ε3/ε3) 在MRI上存在淀粉样变类似影像学异常，并脑沟渗出和水肿(ARIA-E译者注：Amyloid-related imaging abnormalities (ARIA)
include MRI signal abnormalities suggestive of vasogenic oedema and sulcal effusions (ARIA-E) and microhaemorrhages and haemosiderin deposits (ARIA-H))，铁沉积和微出血(ARIA-E)。患者是从3385名参加阿尔茨海默病神经影像研究计划2/ADNI大战略研究中1,006随访者中发现的。淀粉样变在PET上的标准摄取值比率是1.85（阳性）。ARIA-E 和 ARIA-H在认知功能正常、没有APOE ε4风险等位基因的高龄人群中也可能出现，这些人群之前没有微出血，也没有接受淀粉样变修饰治疗。在ARIA-H周围区域局部的淀粉样沉积提示脑淀粉样血管病可能是这个病例发生ARIA的原因。
FIGURE 1 MRI of amyloid-related imaging abnormalities with microhemorrhages and amyloid-related imaging abnormalities with sulcal effusions and edemaAmyloid-related imaging abnormalities with microhemorrhages (arrow) on T2* gradient recalled echo (A), amyloid-related imaging abnormalities with sulcal effusions and edema (arrows) on fluid-attenuated inversion recovery MRI (B) at month 24, which were not present on the 12-month MRI.A、在24个月时T2梯度回波系列显示淀粉样类似影像学异常微出血（箭），B、在FLAIR系列显示脑沟渗出和水肿（箭），而这些在12个月时的MRI并没有发现。 FIGURE 2 Amyloid PET of a patient with spontaneous amyloid-related imaging abnormalities with microhemorrhages and amyloid-related imaging abnormalities with sulcal effusions and edemaC-11 Pittsburgh compound B PET (month 24) shows focal amyloid deposits in the left temporal and occipital lobe. Arrow indicates increased uptake in the location of the microhemorrhages. 11C-匹兹堡化合物B PET （24个月时）显示在左侧颞枕叶淀粉样沉积，箭头提示在微出血部位摄取的增高。
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121. IgG4-related diffuse perineural disease IgG4相关性弥漫性周围神经病A 55-year-old woman had right exophthalmia. Eleven years previously, she had orbital irradiation for refractory nonspecific orbital inflammation. PET/CT revealed FDG uptake in the right orbit and paravertebral masses (figure 1, A and C). MRI showed an enlargement of the right optic nerve and orbital muscles, and a diffuse infiltration involving lumbodorsal and sacral nerve roots (figure 1, B and D). The orbital biopsy demonstrated immunoglobulin G4 (IgG4)+ plasma cell infiltrate and a storiform fibrosis (figure 2), identical to the histopathologic features of the nerve root biopsy, and suggestive of IgG4-related diffuse perineural disease.1,2 No treatment was started in the absence of neurologic symptoms. One year later, the patient had no further symptoms.一名55岁的女性出现右侧眼球突出。11年前患者因难治性非特异性眼眶炎行眼眶放疗史。PET/CT显示在右侧眼眶和椎旁团块样FDG摄取增高。MRI显示右侧视神经和眼眶肌增粗，并弥漫性累及腰骶神经根。眼眶活检显示IgG4阳性浆细胞浸润和席纹样纤维化，神经根活检也见同样的组织病理特点，提示IgG4相关性弥漫周围神经病。由于缺乏神经系统症状，没有进行治疗。1年后，患者的没有出现新症状。 FIGURE 1 Imaging findings of nerve rootsRight orbit (A) and paravertebral (C) FDG uptakes (SUV max = 7). MRI shows meningeal infiltration of the right optic nerve (B, black arrow), a thickening of inferior orbital muscle (B, white arrow), and a diffuse infiltration of paravertebral nerve roots (D).A与C：右侧眼眶和椎旁FDG摄取增高(SUV max = 7)。MRI显示右侧视神经脑膜侵润（B，黑箭头），下直肌增粗（B，白箭）,椎旁神经根的弥漫性侵润（D）。 FIGURE 2 Pathologic findings of the biopsy from the paravertebral massA perineural lymphoplamacytic infiltrate was observed. Note that endoneurium is unremarkable without inflammatory cell infiltration (A, hematoxylin & eosin × 40). Immunohistochemical staining for immunoglobulin G4 (IgG4) revealed approximatively 50 IgG4-positive plasma cells/high-power field (B, immunoperoxidase × 40).周围神经淋巴细胞浸润，神经内膜没有炎性细胞浸润（A，HE染色× 40)。IgG4免疫组化染色显示高倍视野下近50个IgG4阳性浆细胞（B，免疫过氧化物酶染色× 40)
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122.IVth ventricular neurocysticercal cyst第四脑室脑囊虫病A rare cause of acute hydrocephalus一种罕见的急性脑积水原因An 11-year-old boy presented with acute-onset projectile vomiting, bifrontal headache, and altered sensorium. Contrast-enhanced MRI brain was suggestive of intraparenchymal and intraventricular neurocysticercosis, with an intraparenchymal ring-enhancing lesion with central scolex in the left cingulate gyrus and a large cyst enlarging the IVth ventricle, with obstructive hydrocephalus (figure).1 Intraventricular cysticerci with acute hydrocephalus are rare.1In contrast to intraparenchymal cysts, intraventricular cysts are large and typically lack an identifiable scolex.2Confirmation of cysticerci is based upon identification of scolex in the intraparenchymal lesion or use of high-resolution T2 MRI sequences, which are superior in identifying scolex in intraventricular cysts.11岁的男孩出现急性喷射性呕吐，双额头痛，感觉异常。MRI强化提示脑实质和脑室囊虫，左侧扣带回内见头节和戒指样增强病灶，第四脑室显著扩大，阻塞性脑积水。脑室囊虫伴急性脑积水是非常罕见的。与脑实质囊虫比较，脑室囊虫通常更大、缺乏特征性头节征象。囊虫的诊断建立在脑实质病灶内发现头节，或者使用高分辨率MRI T2系列，后者有利于脑室内囊虫头节的识别。 FIGURE MRI brain with IVth ventricular and left parietal neurocysticerciAxial T1 (A) and axial T2 (B) MRI show a small cystic lesion with eccentric scolex (arrow) without perilesional edema in the left cingulate gyrus. Supratentorial ventricle is dilated without any periventricular ooze. Axial fluid-attenuated inversion recovery (C), axial T2 (D), and postcontrast sagittal T1 (E) MRI show ballooned IVth ventricle with intraventricular cystic lesion (arrows), which is better appreciated on thin-cut high-resolution T2 image (D) (arrows); however, scolex was not identifiable in the intraventricular cyst. Axial T2 (F) MRI shows a normal outlet foramina of the IVth ventricle.轴位T1和T2MRI显示左侧扣带回内小囊虫病灶伴偏心性头节（箭）,没有灶周水肿。摹上脑室扩张，没有脑室周围渗出。轴位Flair、轴位T2、矢状位增强T1MRI显示第四脑室球状扩张，伴脑室囊虫病灶（箭），在薄层高分辨率率T2相更易识别（D，箭）。然而，在脑室囊虫病灶没有发现头节。轴位T2（F）MRI显示一四脑室正常出口形态作为对照。
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123. CNS siderosis and orthostatic headache as a delayed complication of spinal surgery脊柱手术后迟发性中枢神经系统铁沉积和体位性头痛A 61-year-old man presented with orthostatic headaches and asymmetric sensorineural hearing loss (ASNHL) 8 years after cervical laminoplasty. Percussion of his cervical spine elicited Lhermitte sign. Brain MRI demonstrated trace intraventricular blood and mild superficial siderosis (figure 1); digital subtraction angiogram had normal results. CT myelogram revealed fractured hardware puncturing adjacent dura with resultant CSF leak (figure 2).61岁的男性患者在颈椎椎体成形术后8年出现体位性头痛和不对称性感音神经性听力减退（ASNHL）。叩诊患者颈椎能诱发出Lhermitte征。头MRI显示脑室微出血和轻微的表面铁沉积。DSA检查正常。CT脊髓造影揭示骨裂刺破邻近的脑膜，导致脑脊液漏。 FIGURE 1 Brain MRITrace intraventricular blood in the right occipital horn is seen as a fluid–fluid level on T2 fast spin echo sequence (A, B). T2* gradient echo sequence shows susceptibility artifact related to intraventricular blood (C) in addition to mild superficial siderosis, most prominently noted along the falx and sylvian fissures (D, arrows).在T2快速回波系列上可见右侧枕角脑室微出血（ Ａ、Ｂ）。T2梯度回波系列显示在轻微的浅表铁沉积外，还可见与脑室血流有关的磁敏感伪影（C），大部分位于大脑镰和外侧裂（D，箭）。 FIGURE 2 Cervical CT myelogram(A) Distal aspect of fractured and medially displaced laminoplasty plate is visible at C7. Its tip pierces the dura and approximates the dorsolateral spinal cord with resultant CSF leak (between arrows), accounting for patient's orthostatic headaches and Lhermitte sign on spine percussion. (B) 3D reconstruction of the dislodged plate. A、C7骨裂的远侧面和内侧被替换了的关节盘，骨裂的头端刺破了硬膜，邻近脊髓的背外侧，伴脑脊液漏（箭头之间），导致了患者的体位性头痛和脊柱叩诊时出现Lhermitte征。B、移位的关节盘3D重建影像。ASNHL or ataxia are typical presenting features of CNS siderosis, resulting from chronic cerebral or spinal subarachnoid hemorrhage, with characteristic brain MRI findings.1,2 CT myelogram in this patient identified the bleeding source and also explained his associated orthostatic headaches and Lhermitte sign.ASNHL或者共济失调时中枢神经系统铁沉积的典型特点，源于慢性的脑或脊髓蛛网膜下腔出血，并有特征性头ＭＲＩ表现。CT脊髓造影在这个患者中发现了出血原因，同时也解释了患者的体位性头痛和Lhermitte征。http://www.neurology.org/content/83/22/2097.full?sid=b0ee-dea92cbd52
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124.Late-onset Alexander disease迟发性亚历山大病A 38-year-old woman presented with an 8-year history of progressive dysarthria, gait disturbance, and hyperreflexia. MRI revealed leukodystrophy involving brainstem with pial signal changes and spinal cord atrophy (figure 1). Brain biopsy showed Rosenthal fibers (figure 2). She had a de novo mutation of the glial fibrillary acidic protein (GFAP) gene (c.799G&C causing p.Ala267Pro).38岁的女性出现进展性构音障碍、步态障碍和反射亢进8年。MRI提示累及脑干的脑白质营养不良，伴脑膜信号改变和脊髓萎缩。脑活检显示Rosenthal纤维。患者检出胶质纤维酸性蛋白基因突变(c.799G&C causing p.Ala267Pro)。 FIGURE 1 Brain and spine MRIFluid-attenuated inversion recovery images show diffuse hyperintensities involving periventricular and subcortical white matter (A–C). Pial signal changes around pons (D) and medulla (E) as well as diffuse atrophies of spinal cord (F) are diagnostic clues.Flair显示弥漫性高信号，累及脑室旁和皮层下白质（A-C）。脑桥和延髓周围软脑膜信号异常（D），并脊髓显著地萎缩（F），这提供了诊断线索。 FIGURE 2 Rosenthal fibersHematoxylin & eosin staining of the white matter biopsy showed typical morphology of Rosenthal fibers (arrowheads). Rosenthal fibers are beaded, elongated, or corkscrew-shaped intracytoplasmic inclusions that represent accumulation of intermediate filament. Rosenthal fibers are seen in neoplasms (such as pilocytic astrocytomas), Alexander disease, and reactive tissues with gliosis.脑白质活检HE染色显示典型的Rosenthal纤维（箭头）。Rosenthal纤维为串珠状、拉长，或螺旋形胞浆内含物，代表了中间丝聚集。Rosenthal纤维见于肿瘤（如：毛细胞型星形胶质细胞瘤），亚历山大病，胶质增生伴随的组织反应。Alexander disease is caused by gain-of-function mutation of the GFAP gene. GFAP is an intermediate filament, and mutations result in astrocytic accumulation of eosinophilic inclusions known as Rosenthal fibers.1 Late-onset patients show brainstem features (ataxia, dysphagia, dysphonia, and palatal myoclonus) with hindbrain-predominant leukodystrophy and spinal cord atrophy.亚历山大病是由于GFAP基因获得性突变导致。GFAP为一种中间丝，其突变导致了星形胶质细胞内含嗜酸性物质的聚集，此即为Rosenthal纤维。迟发性患者表现出脑干特征（共济失调、构音障碍、软腭阵挛），伴后脑为主的脑白质营养不良、脊髓萎缩。1,2http://www.neurology.org/content/83/22/e197.full?sid=b0ee-dea92cbd52
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125. Neurogenic muscle hypertrophy神经源性肌肥大A 42-year-old man had a 2-year history of progressive asymptomatic left calf enlargement. MRI showed diffuse swelling of left calf muscles (figure, A and B); biopsy displayed neurogenic changes (figure, C and D). Electrodiagnostic examination results were compatible with left chronic S1 radiculopathy and lumbosacral MRI revealed an ipsilateral L5-S1 disc protrusion encroaching on the foraminal space.42岁的男性患者出现进展性无症状性左侧小腿增粗2年。MRI显示左侧小腿肌肉增粗（图A、B）。活检显示神经源性改变（图C、D）。肌电图符合慢性左侧S1神经根病变，腰骶MRI揭示同侧Ｌ５-S1椎间盘膨出挤压椎间孔。 FIGURE Left calf findingsCoronal T2-weighted (A) and axial T2-weighted fat-saturated (B) MRI show diffuse swelling of gastrocnemius and soleus muscles. Gastrocnemius medialis biopsy: hematoxylin & eosin (×10) (C) and nicotinamide adenine dinucleotide tetrazolium reductase (×20) (D) stains. Note variability in fiber diameters with predominance of hypertrophic fibers, internal nucleation, fiber splitting, disruption of intermyofibrillar architecture, and target fibers corresponding to denervation.A、B:冠状位T2相和轴位T2脂质饱和相显示腓肠肌和比目鱼肌的增粗。腓肠肌活检：HE染色（×10)（C）和还原型辅酶Ⅰ四氮唑还原酶染色(×20) (D)。注意肌纤维直径的变化，伴有肥大的肌纤维为主，内核形成，纤维断裂，肌纤维间结构和靶纤维的破坏，符合去神经病变表现。Unilateral calf swelling may be associated with deep vein thrombosis, ruptured popliteal cyst, tumors, trauma, autoimmune disorders, vascular anomalies, focal myositis, and radiculopathy. Neurogenic disorders usually
hypertrophic changes may result from compensatory work of nondenervated muscle fibers and spontaneous electrical activity of denervated muscle fibers一侧小腿增粗可能原因为下肢深静脉血栓、腘窝囊肿破裂、肿瘤、外伤、自身免疫病、血管畸形、肌炎和神经根病变。神经源性疾病通常导致肌萎缩，肥大性的改变可能是由于没有受累的肌纤维的代偿性肥大，以及去神经后肌纤维的自发电活动导致。.1
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126. Eosinophilic infiltration and necrotizing vasculitis on nerve biopsy in Churg-Strauss syndromeChurg-Strauss综合征神经活检中的嗜酸性细胞浸润和坏死性血管炎A 57-year-old woman with asthma, nasal polyps, and allergies presented with acute lancinating pain and patchy weakness in the distribution of multiple upper and lower limb peripheral nerves. She had peripheral eosinophilia (2 × 109/L). Sural nerve biopsy demonstrated nerve large arteriolar necrotizing vasculitis with eosinophils (figure), consistent with Churg-Strauss syndrome (CSS), renamed eosinophilic granulomatosis with polyangiitis.伴哮喘、鼻息肉和过敏质的57岁的女性出现上下肢周围神经分布区多发急性刺痛和斑片状乏力。患者外周嗜酸性粒细胞增多(2 × 109/L)。腓肠神经活检显示大量的小血管坏死性炎症伴嗜酸性粒细胞增多（图），符合Churg-Strauss综合征表现，也称为嗜酸性粒细胞性肉芽肿性血管炎。 FIGURE Hematoxylin and eosin–stained paraffin cross-sections(A) Low power shows many nerve fascicles, a large epineurial arteriole with fibrinoid necrosis and eosinophilia (arrow), and small vessels with transmural inflammation (arrowhead). (B) High power of large arteriole shows fibrinoid necrosis (arrow) with eosinophils surrounding and invading the vessel wall (arrowhead).A、低倍镜显示大量神经束，一神经外膜小动脉伴纤维蛋白样坏死和嗜酸性粒细胞增多（箭），小血管的透壁性炎症（箭头）。B、小动脉高倍镜下显示纤维蛋白样坏死（箭），伴周围嗜酸性粒细胞浸润和血管壁受累（箭头）。CSS is a systemic vasculitis involving small to medium-sized vessels, associated with asthma, chronic rhinosinusitis, and eosinophilia. Peripheral neuropathy occurs in 60%–70% of cases, and nerve biopsy may show intravascular and extravascular granulomas.1 Eosinophilic inflammation in the nerve is rare, occurring in approximately 15% of biopsies CSS是累及中小血管的系统性血管炎，伴有哮喘、慢性鼻窦炎和嗜酸性粒细胞增多。约60%–70%病例发生周围神经受累，神经活检可以显示血管内或血管外肉芽肿。周围神经嗜酸性粒细胞浸润非常罕见，近15%的CSS活检病例中可出现。2http://www.neurology.org/content/83/23/e202.full?sid=b0ee-dea92cbd52
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127. Bilateral papilledema associated with spinal schwannoma脊髓神经鞘瘤并双侧视乳头水肿Correspondence to Dr. Matsubara: tomoyasumatsubara{at}yahoo.co.jpA 61-year-old man presented with a 4-month history of progressive vision loss and paresthesia between the right L2-L4 dermatome. Funduscopy revealed severe bilateral papilledema (figure 1). Head CT showed mild hydrocephalus. CSF from a lumbar puncture at the L4-L5 level was xanthochromic with an elevated protein level (4,350 mg/dL). Lumbar spine MRI demonstrated a large intradural tumor at the L3-L4 level (figure 2), which was pathologically identified as schwannoma. Surgical removal resolved all symptoms. Papilledema is a rare complication induced by marked elevation of CSF protein, which may disturb CSF absorption and result in high intracranial pressure.1,261岁的男性患者出现进展性视力减退4个月，并右侧L2-L4节段感觉异常。眼底镜显示双侧严重视乳头水肿（图1）。头颅CT显示轻度脑积水。L4-L5水平腰穿脑脊液检查发现黄色脑脊液，蛋白升高(4,350 mg/dL)。腰髓MRI显示L3-L4水平巨大硬膜内肿瘤，病理证实为神经鞘瘤。外科切除术后所以症状得到缓解。由脑脊液蛋白显著升高引起的视乳头水肿非常罕见，前者可妨碍脑脊液的吸收、导致颅内高压。 FIGURE 1 PapilledemaFunduscopy shows severe bilateral papilledema surrounded by peripapillary hemorrhage. Visual acuity was 20/50 in both eyes.眼底镜显示严重的视乳头水肿并视乳头周围出血，双眼视力仅20/50（0.4）。
FIGURE 2 MRI of the spinal schwannomaT1-weighted gadolinium-enhanced lumbar spine MRI demonstrates a heterogeneous hyperintense mass located in the vertebral canal at the level of the L3-L4 vertebral bodies.T1加权增强序列显示L3-L4水平椎管内异质性高信号肿块。
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128. MRI of brain findings of Wolfram (DIDMOAD) syndrome Wolfram综合征的头部MRI表现Correspondence to Dr. Guler: gulerezgi{A 31-year-old woman was diagnosed with type 1 diabetes mellitus (DM) at age 5 years and subsequently with hypothyroidism at age 16 years. She developed progressive visual loss at age 19 years and progressive hearing loss at age 28 years. She was clinically and radiologically diagnosed with Wolfram syndrome (figures 1 and 2). Wolfram syndrome, first described in 1938, is a rare autosomal recessive disorder.1 It features diabetes insipidus (DI), DM, optic atrophy (OA), and deafness (D) (DIDMOAD).1,2 It is caused by a mutation in the WFS1 gene that encodes wolframin, a transmembrane protein of pancreatic β cells.1 The life expectancy of patients diagnosed with this syndrome is about 30 years.一名31岁的女性患者在5岁时诊断为1型糖尿病，16岁又时出现甲状腺功能减退，19岁视力渐进丧失，28岁听力进行性减退。临床和影像学提示Wolfram综合征，这种疾病首先于1938年报道，是一种罕见的常染色体隐性遗传病，特征表现为尿崩症、糖尿病、视神经萎缩、耳聋(DIDMOAD)。它是由于WFS1基因突变导致合成wolframin蛋白障碍引起，后者系胰腺β细胞跨膜蛋白。这种疾病患者的预期寿命约30岁。 FIGURE 1 Brain MRI findings of a 31-year-old woman with Wolfram syndromeAxial T2-weighted image (A) and sagittal T1-weighted image (B) demonstrate atrophy of brainstem. Note absence of neurohypophyseal “bright signal” on sagittal T1 image (B). T2-weighted image (C) shows atrophy of cerebellum and hyperintense signal at ventral part of the pons.轴位T2加权序列和矢状位T1加权系列显示脑干萎缩。注意B图矢状位T1相上神经垂体高亮信号的缺失。图C T2加权序列显示小脑的萎缩和脑桥腹侧部高信号改变。 FIGURE 2 Optic nerve and optic tract findingsAxial fluid-attenuated inversion recovery image (A) demonstrates increased signal intensity in the bilateral peritrigonal areas/optic tracts. Coronal T2-weighted image (B) shows thinning/atrophy of bilateral optic nerves (arrows).FLAIR序列轴位显示双侧三角部、视觉传导径路高信号影（A）。冠状位T2加权序列显示双侧视神经减少、萎缩。
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129. Drawing perseveration in the behavioral variant of frontotemporal dementia行为变异型额颞叶痴呆中出现的持续性绘画Correspondence to Dr. Ioannidis: ispanagi{at}auth.grThe behavioral variant of frontotemporal dementia (bvFTD) represents a distinct clinical FTD subtype, dominated by progressive deterioration in cognition, personality, and social conduct. Perseverative behavior is a core clinical feature in the proposed diagnostic criteria.1 Two patients with bvFTD exhibited a peculiar form of perseveration, which we named drawing perseveration. During neuropsychometric testing, especially after copying pentagons, they both spontaneously drew animals: a pigeon or a dog (figure). They both used to draw these animals during childhood, but not during adulthood, in contrast to the emergence of artistic talent that can occur in FTD.2行为变异型额颞叶痴呆是一种不同的临床亚型，主要包括进展性认知功能减退，人格改变，社会行为异常。持续性、刻板性行为异常在建议的诊断标准中作为其核心临床特征。2名额颞叶痴呆的患者表现出奇特的持续性行为，我们称其为持续性绘画。在神经心理测试时，特别是在临摹五角形时，他们都自动出现绘画动物：分别是鸽子和狗（图）。他们在儿童期都画过这些动物，但是在成年后没有再画过，这与额颞叶痴呆患者中也可能出现的艺术才能并不同。FIGURE Drawing perseverationThe first patient drew a pigeon and the second drew a dog after they were asked to copy the pentagons.
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130. Medullary cap dysplasia延髓帽状发育异常MRI and diffusion tensor imaging of a hindbrain malformation后脑畸形的MRI和DTI表现Correspondence to Dr. Jurkiewicz: e-jurkiewicz{at}o2.plA 3-year-old boy presented with respiratory distress and apnea, discrete dysmorphic features (facial asymmetry, large dysplastic earlobes, deep-set eyes), absence of the middle and distal phalanges of the hands and feet, gastroesophageal reflux, and hiatal hernia. Auditory brainstem response testing (brainstem auditory evoked response) was abnormal bilaterally with more severe changes on the right side.一名3岁的男孩出现呼吸窘迫和呼吸暂停，伴多发畸形表现（面部不对称，巨大耳垂，眼睛深陷），手足缺中远端指骨，胃食管返流，食管裂孔疝。听觉诱发脑干反应测试（脑干听觉诱发反应）显示双侧异常，右侧更为严重。MRI revealed hypoplastic pons and thickened medulla oblongata with anomalous mass of gray matter signal intensity around it and abnormal signal of the dentate nuclei (figure 1). Diffusion tensor imaging (figure 2) shows lack of pontine fiber crossing in this malformation.1,2 Supratentorial anomalies included hippocampal malrotations and corpus callosum hypogenesis. This malformation has been mentioned previously only by Barkovich and colleagues.1,2MRI提示脑桥发育异常，延髓增粗，并其周围灰质信号中出现异常块状影，齿状核异常信号改变，（图1）。弥散张量成像（图2）显示在畸形区域没有脑桥纤维交叉经过。幕上的异常包括海马旋转不良（？）和胼胝体发育不良。这种畸形之前仅由Barkovich报道过。FIGURE 1 MRI of the brainAbnormal rounded cap over the medulla oblongata (arrows) (A–D). Small pons with abnormal anterior outline, thickened medulla oblongata. Oversized and dysplastic cerebellar vermis. Hypogenesis of the splenium of corpus callosum (D, E). Dysplastic upper part of the cerebellar hemispheres (F).延髓周围异常的圆形帽状结构（A-D）。小的桥脑并异常的腹侧轮廓，延髓增粗。小脑蚓部增大发育异常。胼胝体压部发育异常（D，E）。小脑半球上部发育异常（F）。FIGURE 2
Diffusion tensor imaging and tractographyNo decussating pontine fibers—no red spot (A, B) and absence of the crossing of the corticospinal tracts (C).DTI显示脑桥纤维没有交叉---没有红色点状信号（A、B），皮质脊髓束纤维没有交叉（C）。
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131. Posttraumatic lumbar epidural hematoma外伤后腰髓硬膜外血肿Correspondence to Dr. van Aalst: j.van.aalst{at}mumc.nlA 36-year-old man presented with pain radiating from his right hip to the knee, after a heavy fall on his back 2 months earlier. At neurologic examination, bilateral Lasegue's sign (straight leg raise test) was present. MRI revealed a ventral epidural space-occupying lesion at L2-L3 (figure). The differential diagnosis consisted of a sequestered disc, a ventral synovial cyst, and an epidural hematoma.一名36岁的男性患者出现右侧髋部至膝部的放射痛，2个月之前患者曾有背部严重跌倒外伤史。神经系统查体发现双侧Lasegue征阳性（即直腿抬高试验）。MRI 显示L2-L3硬膜外腹侧占位病变（图）。鉴别诊断包括游离型椎间盘突出、滑膜囊肿、硬膜为血肿。 FIGURE MRI of the lumbar spineSagittal T2-weighted (A) and T1-weighted (B), detail (C), and transverse T2-weighted (D) MRIs show a convex hyperintense T2 lesion in the ventral epidural space at L2-L3, with slight hyperintensity at T1 without obvious signs of adjacent disc degeneration or hydrops of facet joints. The asterisk in panel D points at the hematoma. One year later, the MRI was normalized (E–G).A、矢状位T2加权序列；B、矢状位T1加权序列；C、B图局部放大；D、横断面T2加权序列。显示在L2-L3水平腹侧硬膜外腔一T2相高信号凸出病灶，在T1相呈稍高信号，邻近椎间盘没有明显退行性变或关节面积液表现。D图星号标示了血肿。一年后，MRI恢复正常（E-G）。The diagnosis of a spinal epidural hematom a posttraumatic lumbar epidural hematoma is even more rare.1,2In our patient, the complaints resolved spontaneously and 1 year later, the lesion had disappeared (figure).脊髓硬膜外血肿的诊断较为困难，外伤后腰髓硬膜外血肿则更为罕见。我们的患者其症状自发得到缓解，1年后影像学上的病变也消失了。
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132.Primary Sj?gren syndrome presenting as isolated lesion of medulla oblongata表现为延髓孤立病变的原发性干燥综合征Correspondence to Dr. Yan: yanzhw{at}mail.A 21-year-old woman presented with 2 weeks of vomiting and a brief episode of sudden loss of consciousness. On examination, she had slurred speech and dysphagia. Fluid-attenuated inversion recovery and T2-weighted MRI of the brain revealed white-matter hyperintensity within the medulla oblongata (figure 1, A–C). Biopsy of the salivary gland was diagnostic of Sj?gren syndrome (SS) (figure 2). After multimodal therapy, lesions of medulla oblongata improved (figure 1D), and the patient largely returned to normal.一名21岁的女性患者出现呕吐2周，并有短暂的意识丧失。查体发现患者言语不清，吞咽困难。FLAIR及T2加权序列MRI显示延髓白质高信号病变（A-C）。唾液腺活检诊断为干燥综合征（图2）。在经过综合治疗后延髓的病变得到改善，病人基本恢复正常。 FIGURE 1 Brain MRI(A, B) T2-weighted axial and sagittal images reveal focal hyperintensity within the dorsal medulla oblongata. (C) Fluid-attenuated inversion recovery coronal MRI shows hyperintense white matter lesions predominantly involving medulla oblongata. (D) Lesions of medulla oblongata were basically normal after therapy (white arrow).A、B：T2加权轴位及矢状位显示延髓背侧局部高信号病变。C：FLAIR冠状位显示高信号的白质病变主要累及延髓。D：在治疗后延髓病变基本恢复正常。（白箭）。 FIGURE 2 Histopathology images(A) Hematoxylin & eosin staining of salivary gland shows acinar atrophy (■), small duct dilation (●), and focal lymphocytic infiltration (▲) (×100). (B) Presence of lymphocytic (white arrow) and plasma cells (yellow arrow) is shown at a higher magnification (×200).A、唾液腺HE染色显示腺泡萎缩(■), 小管扩张(●)，局部淋巴细胞浸润(▲) (×100)。B、在高倍镜下呈现淋巴细胞（白箭头）和浆细胞（黄箭头）(×200)。Primary SS is characterized by chronic inflammation of exocrine glands. Involvement of CNS has been reported occasionally.1,2原发性干燥综合征主要特征为外分泌腺的慢性炎症，累及中枢神经系统者偶有报道。
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133. Excessive brain iron accumulation in spinocerebellar ataxia type 17脊髓小脑性共济失调17型患者脑部铁的过度积聚Correspondence to Dr. Claassen: jens.claassen{A 60-year-old man had a 3-year history of cerebellar ataxia and dementia, without a family history. T1-weighted MRI showed cerebellar atrophy (figure, A). Susceptibility-weighted images (SWI) revealed hypointensities of the basal ganglia and mesencephalic and cerebellar nuclei (figure, B, a–c), suggesting neurodegeneration with brain iron accumulation.1 Serum copper, iron, ferritin, transferrin, and ceruloplasmin levels were normal. Genetic testing revealed a CAG/CAA repeat expansion of 1 allele with 44 repeats (normal range 25–42), within the reduced penetrance range (43–48 repeats) in the TATA box binding protein (TBP) gene.2 In patients with cerebellar atrophy with hypointensities of subcortical and cerebellar nuclei in SWI or gradient echo imaging, diagnostic considerations should include spinocerebellar ataxia 17.一名60岁的男性患者有小脑性共济失调及痴呆史3年，没有家族史。T1加权序列显示小脑萎缩（图A）。SWI序列显示基底节和中脑、小脑核的低信号病变（图B，a-c），提示神经退行性变伴脑部铁沉积。血清铜、铁离子、铁蛋白、转铁蛋白、铜蓝蛋白均正常。基因检测提示一等位基因CAG/CAA序列重复扩增了44次（正常25-42），且TATA盒结合蛋白基因外显率减低（43-48个重复序列）。对于小脑萎缩伴有SWI或梯度回波序列MRI上皮质下及小脑核低信号病变的患者，必须考虑脊髓小脑性共济失调可能。 FIGURE MRI in spinocerebellar ataxia type 17(A) T1-weighted MRI shows cerebellar atrophy. (B) Susceptibility-weighted imaging shows hypointensities in (a) dentate nucleus, (b) red nucleus, and (c) globus pallidus.A、T1加权MRI显示小脑萎缩。B、SWI显示齿状核、红核、苍白球低信号病变。
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134. MRI in X-linked adrenoleukodystrophyX连锁肾上腺脑白质营养不良MRI表现Correspondence to Dr. Siddiqui: siddiquis{at}upmc.eduA 45-year-old man with no medical history presented with behavioral changes. He was withdrawn from his family and friends. Home and finances had fallen into disorder. He was disheveled and incontinent of urine and feces. He lacked insight into his cognitive decline. His skin was bronzed and the examination was notable only for upgoing plantar responses. Peroxisomal fatty acid profile was elevated at C24:0 at 138.9 nmol/mL (normal &91/4), elevated C26:0 at 5.05 (normal &1.39), and elevated C26:0/C22:0 ratio at 0.062 (normal &0.023). Images show abnormal high signal on fluid-attenuated inversion recovery (figure 1) with enhancement (figure 2).一名没有特殊病史的45岁男性出现行为异常。他与家庭和朋友隔离，家庭和经济状况陷入困境，蓬头垢面、尿便失禁。对于其认知功能下降没有自知力。患者皮肤呈古铜色，查体除跖反射活跃外没有明显异常。过氧化物酶脂肪酸检测C24:0升高达138.9 nmol/mL (正常&91/4)，C26:0 升高达 5.05 (正常l &1.39)，C26:0/C22:0 比值为 0.062 (正常&0.023)。影像学显示FLAIR相高信号病变，没有强化。 FIGURE 1 MRI brain axial fluid-attenuated inversion recoveryAxial fluid-attenuated inversion recovery shows hyperintense white matter signal and generalized mild ventricular prominence.轴位FLAIR序列显示白质高信号病变及侧脑室轻度扩张改变。 FIGURE 2 MRI brain coronal imageCoronal T1-weighted fast spoiled gradient-echo image shows symmetrical abnormal linear contrast enhancement at the periphery of the white matter abnormality, characteristic of X-linked adrenoleukodystrophy.冠状位T1加权快速扰相梯度回波显示异常白质区周围线样异常增强信号，为X联锁肾上腺脑白质营养不良的特征改变。
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135. Meningioangiomatosis脑膜血管瘤病A 23-month-old full-term boy presented with tonic-clonic seizures. Lumbar puncture and routine laboratory tests were unremarkable. CT (figure 1A) showed bifrontal parasagittal calcification. Brain MRI revealed corresponding signal abnormalities with gradient susceptibility (figure 1D) and enhancement (figure 1F). Differential diagnoses included prior infection, vascular malformation, and tumor. Biopsy (figure 2) was consistent with meningioangiomatosis.一名23个月的足月产男孩出现强直阵挛性癫痫发作。腰穿和常规实验室检查没有明显异常。CT显示双额矢状窦旁钙化。MRI显示在梯度磁敏感相和强化相异常信号。鉴别诊断包括既往感染、血管畸形、肿瘤。活检结果符合脑膜血管瘤病表现。 FIGURE 1 CT and MRI of bifrontal parasagittal lesion noncontrast axial CT(A) Amorphous high density indicative of mineralization with corresponding subtle low signal on susceptibility MRI (B). (C) Precontrast T1 shows isointense but thickened cortex. (D) Postcontrast T1 shows robust leptomeningeal enhancement, presumably due to vascular proliferation, and subtle nonspecific cortical enhancement. Subcortical white matter signal changes, characteristic of meningioangiomatosis, were initially difficult to appreciate (E) but were more apparent on follow up T2–fluid-attenuated inversion recovery image (F).A、模糊的高密度信号提示矿化物质，在磁敏感MRI（B）显示相应的轻微低信号。C、T1平扫显示等信号，但是皮质增厚。D、T1强化相显示明显的柔脑膜强化，可能是由于血管的增生；并轻微的皮层强化。皮层下白质信号的异常—这是脑膜血管瘤病的特征，在E图上难以识别，但是在T2 Flair 相（F）则较为明显。 FIGURE 2 Histology of meningioangiomatosisHematoxylin & eosin staining at 4× (A) and 20× (B) magnification shows whorls of meningothelial-like spindle cells proliferating around blood vessels with numerous calcified psammoma bodies. CD31 staining (C) highlights the numerous blood vessels around which the meningothelial-like cells proliferate.HE染色4× (A)和 20× (B)倍放大图显示环绕血管壁的螺旋型脑膜上皮样纺锤细胞增生，并大量钙化的砂粒体。CD31染色（C）突出显示环绕大量血管的脑膜上皮样细胞增生。Meningioangiomatosis is a rare epileptogenic lesion involving the meninges and cortex characterized by vascular proliferation and calcifications. Meningioangiomatosis occurs sporadically or in association with neurofibromatosis 2 (NF2).1,2 Testing for NF2 proved positive in this patient, despite no other clinical stigmata.脑膜血管瘤病一种罕见的累及脑膜和皮质的致痫病变，特征为血管增生和钙化。脑膜血管瘤病可散发或与神经纤维瘤病2型并发。本患者NF2检测为阳性，尽管没有临床表现。
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136．Striatocapsular infarct presumed due to intracranial lipoma推测为颅内脂肪瘤导致的纹状体内囊梗死A 27-year-old woman with no known risk factors for stroke presented with right hemiparesis. There was infarction of left corpus striatum on brain CT and MRI (figure 1). Imaging also showed an intracranial lipoma adjacent to the middle cerebral artery (MCA) (figure 2).一名没有已知卒中危险因素的27岁女性患者出现右侧偏瘫。在左侧纹状体区CT和MRI提示梗死灶。影像学同时显示邻近大脑中动脉的颅内脂肪瘤。 FIGURE 1 Striatocapsular infarction on the left side(A) Brain CT images, (B) MRI T2-weighted images, (C) diffusion-weighted images, and (D) apparent diffusion coefficient maps show chronic infarction.CT和MRI显示左侧纹状体区一陈旧梗死灶。 FIGURE 2 Intracranial lipoma (arrow) located adjacent to the middle cerebral artery bifurcation颅内脂肪瘤邻近大脑中动脉分叉部（箭头）。(A) The brain CT density of this hypodense lesion was measured as ?80 HU. (B) T1-weighted MRI shows the hyperintense lipoma. (C) On T1-weighted image with fat suppression, intensity of the lesion is homogeneously decreased. (D) Reformatted time-of-flight magnetic resonance angiogram image shows lipoma caused chemical shift artifact at the origin of the lenticulostriate arteries.A、低信号病变CT值为-80HU。B、T1 MRI显示高信号的脂肪瘤。C、T1脂肪抑制相见病变信号均质降低。D、TOF MRA 显示在豆纹动脉起始部脂肪瘤导致的化学位移伪影。We presumed that striatocapsular infarction was due to occlusion of the orifice of a lenticulostriate artery originating from the MCA.1 Intracranial lipomas may rarely occur adjacent to the MCA and may be associated with striatocapsular infarction.2我们推测纹状体内囊梗死是由于起自大脑中动脉的豆纹动脉开口闭塞，颅内脂肪瘤可罕见出现于邻近大脑中动脉区，与纹状体内囊梗死相关。http://www.neurology.org/content/84/2/e11.full?sid=768b767b-c96b-45b9-8e4a-10c8c8a023eb
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137.Levamisole-induced leukoencephalopathy mimicking Baló disease类似同心圆硬化的由左旋咪唑诱发的白质脑病A 42-year-old man presented with subacute onset of right hemiparesis and mild cognitive impairment, who had taken 600 mg oral levamisole for ascariasis 30 days prior. Neuroimaging revealed 2 irregular lesions in the white matter of bilateral frontal lobes. The left larger lesion showed the shape of a concentric ring (figure, A and B), and after Gd-DTPA administration, both lesions demonstrated prominent ring enhancement1 (figure, C). The patient was diagnosed with levamisole-induced leukoencephalopathy, mimicking Baló disease, and treated with high-dose IV glucocorticoid therapy.2Within 3 months, the patient's symptoms improved and a repeat MRI scan showed that the previous lesions had decreased in size.一名42岁的男性出现亚急性右侧偏瘫和轻度认知功能损害，30天前患者曾因蛔虫病口服600mg左旋咪唑。神经影像检查显示双侧前额区白质不规则病变，左侧较大的病变显示同心圆环，在增强后双侧病变都显示明显环状强化。患者诊断为左旋咪唑诱发的白质脑病，影像学类似同心圆硬化，患者予以高剂量静脉糖皮质激素治疗。3个月后，患者症状得到改善，复查MRI显示之前的病变已经减小。 FIGURE Brain MRI in a patient with levamisole-induced leukoencephalopathyAxial views show 2 irregular oval lesions (T1 hypointense and T2 hyperintense) in the white matter of bilateral frontal lobes, with the larger left lesion shaped in a concentric ring (A, B). Sagittal view shows ring enhancement after Gd-DTPA administration (C).A及B：轴位相显示双侧前额区2个不规则的椭圆形病变（T1低信号、T2高信号），较大的病变呈同心圆环。C 、矢状位增强相显示环状强化。
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138. Disseminated cysticercosis播散性囊虫病（译者注：图片看了发麻、勿喷）An 18-year-old boy presentedwith headaches, vomiting, recurrent seizures, and altered sensorium.He had extensive muscle hypertrophy on examination. MRI revealed multiple cystsin different stages in brain parenchyma, scalp, extraocular muscles, neck, chestwall, abdominal wall, paraspinal, gluteal, pelvic, and limb muscles (). The patient received steroids and antiepilepticdrugs and had a good recovery. The patient is seizure-free at 6 months. Indisseminated neurocysticercosis, symptomsare related to space-occupying effect rather thaninflammation caused by dying parasites, and in this situationcysticidal drugs may exacerbate the syndrome of intracranialhypertension.一名18岁的男孩出现头痛、呕吐、反复抽搐和感觉异常。查体发现患者肌肉肥大。MRI显示脑实质、头皮、眶外肌、颈、胸壁、腹壁、椎旁、臀部、骨盆和四肢肌肉多发不同时期囊虫影像。患者接受激素及抗癫痫治疗，预后良好，6个月随访时患者无癫痫发作。在播散性脑囊虫患者，症状主要系空间占位效应引起，而不是由于虫体死亡诱发的炎症引起，此时使用驱虫药可能会加重颅内高压症状。FIGURE MRI shows disseminated cysticercus cysts in brain,extraocular muscles, and skeletal muscles(A) Axial T2-weightedimages show multiple cysticercus cysts in bilateral brain parenchyma, scalp,and extraocular muscles. (B–D) Coronal STIR images show extensive cysticercus cysts in neck, chest wall, abdominal wall,paraspinal, gluteal, pelvic, and limb muscles.A、轴位T2加权相显示双侧脑实质、头皮、眶外肌肉多发囊虫影像。B-D、冠状位短时反转恢复序列(STIR)显示颈部、胸壁、腹壁、椎旁、臀部、骨盆和四肢肌肉广泛囊虫影像。
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139. The lentiform fork sign豆状核镜像征An MRI pattern of metformin-associated encephalopathy二甲双胍相关性脑病的MRI影像A 63-year-old woman with type 2 diabetes mellitus on hemodialysis presented with subacute onset of slurred speech and difficulty walking after 1 week of inadvertent metformin use.一名63岁有2型糖尿病并血透史的女性患者在无意中使用二甲双胍后1周出现亚急性起病的言语含糊和行走困难。On examination, she had a wide-based gait, symmetric upper limb rigidity, bradykinesia, and slurred speech.查体发现其存在宽基底步态，对称性上肢肌强直，行动迟缓，以及言语不清。Brain MRI revealed a pattern of vasogenic edema with T2/fluid-attenuated inversion recovery hyperintensity in the basal ganglia compatible with the lentiform fork sign1 (figure).MRI显示基底节区T2/FLAIR相血管源性高信号病变，符合豆状核镜像征表现。 FIGURE The lentiform fork sign(A)
Axial noncontrast CT shows bilateral hypodensities in the basal ganglia. (B) Brain MRI reveals a pattern of vasogenic edema with T2/fluid-attenuated inversion recovery hyperintensity in the basal ganglia compatible with the lentiform fork sign.A、轴位非增强CT显示双侧基底节区低密度影。B、MRI显示基底节区T2/FLAIR相血管源性高信号病变，符合豆状核镜像征表现。Basal ganglia pathology has been described on the setting of metabolic acidosis and metformin use in hemodialysis patients, resulting in a parkinsonian syndrome. 基底节区的这种病变曾报道见于代谢性酸中毒和使用二甲双胍的血透患者，多表现为帕金森综合征。http://www.neurology.org/content/84/3/e15/F1.expansion.html
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140. Frog in the throat
喉部的青蛙A 27-year-old man presented with pain in his throat after a generalized seizure. He had a 2-year history of nighttime seizures and wore dental prosthetics. Upon awakening after the seizure, the patient complained of chest pain and pain in his throat and coughed up some blood. He could not find his front teeth. Physical examination revealed subcutaneous emphysema around his trachea. X-ray of the cervical spine showed the prosthetic device in his esophagus (figure). Endoscopic examination showed no perforation of the esophagus. The prosthetic was removed and the patient resumed his antiepileptic drugs.一27岁的男性患者癫痫全面性发作后出现喉部疼痛。患者有2年的夜间抽搐史，装配义齿。抽搐清醒后患者诉胸痛和喉部疼痛，咳血，并且其前牙不见了。体检发现气管旁皮下气肿。X线发现食管内义齿。内镜显示食管没有穿孔，取出义齿后患者恢复了抗癫痫治疗。 FIGURE X-ray of the cervical spine shows dental device颈椎X线显示义齿，形似青蛙。w.neurology.org/content/84/4/433/F1.expansion.html
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141. Cerebral myiasis脑蝇蛆病An 85-year-old patient was admitted to the emergency room septic with fetid odor in the right orbit, where enucleation of the right eye had been performed 8 years prior. Physical inspection noted the presence of larvae within the right orbit. After a CT scan (figure 1), the patient underwent surgical treatment (figure 2). Intraoperative cultures revealed encephalitis caused by myiasis. The patient died due to complications caused by sepsis 2 weeks after the procedure.一名85岁患者因右侧眼眶脓臭味入急诊室，其曾在8年前行右侧眼球摘除术。查体发现右侧眼眶有幼虫。CT检查后患者进行了外科治疗。术中培养提示为蝇蛆导致的脑炎。在术后2周患者死于脓毒血症并发症。 FIGURE 1 Preoperative images(A) Axial CT image demonstrates a bone lysis in the skull base. (B, C) Axial CT image reveals density changes in the right frontotemporal region, which is suggestive of pneumocephalus due to lysis of the wall of the paranasal sinuses and ischemic areas due to myiasis.A、轴位CT显示颅底骨侵蚀。B、C：轴位CT显示右侧额颞区低密度影，提示鼻窦旁骨侵蚀后出现颅脑积气和蝇蛆导致的炎症缺血。 FIGURE 2 Operative findings(A) Physical inspection noted the presence of larvae in the right orbit. (B) Image after surgical skin incision. (C) Image of the brain parenchyma after cleaning and removal of larvae.A、查体发现右侧眼眶有幼虫存在。B、外科皮肤切口图像。C、在清除幼虫后脑实质的图像。Among the 11 cases with cerebral myiasis reported in the literature,1 we found no history of infection by contiguity to the brain with a skull base bone lysis.在文献报道的11名脑蝇蛆病病例中，没有类似文中患者脑实质感染并颅底骨侵蚀的病例。
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142. Eosinophilic CNS vasculitis can mimic demyelinating disease of the brain and spinal cord嗜酸性粒细胞中枢神经系统血管炎可类似于脑脊髓脱髓鞘疾病A 61-year-old man developed progressive ascending sensory loss to T8 and paraparesis over 4 weeks. MRI revealed ovoid T2-hyperintense lesions in juxtacortical and periventricular areas, and in the T7-T10 spinal segment, with varying degrees of peripheral gadolinium enhancement (figure 1). He received 5 days of high-dose corticosteroids and plasmapheresis for presumed demyelination, without clinical response. Brain biopsy showed vasculitis involving small arteries with transmural inflammatory cell infiltrates including numerous eosinophils (figure 2). Eosinophilic vasculitis can cause ischemic strokes1; it rarely involves the spinal cord.2 Our case illustrates that it should be considered in the differential diagnosis of CNS demyelination.一名61岁的男性患者出现进行性的上升性感觉缺失、高达T8平面，并下肢瘫4周余。MRI显示近皮层和脑室周围，以及T7-10节段卵圆形T2高信号病变，存在不同程度周边强化。考虑为脱髓鞘疾病，患者接受了5天高剂量的皮质激素和血浆置换治疗，但是没有效果。脑活检显示累及小动脉的血管炎，伴炎性细胞跨壁浸润、包括大量的嗜酸性粒细胞。嗜酸性粒细胞血管炎可导致缺血性卒中，但累及脊髓罕见。我们的病例显示中枢神经系统脱髓鞘疾病的鉴别诊断包括嗜酸性粒细胞中枢神经系统血管炎。
FIGURE 1 MRI of the brain and spinal cordJuxtacortical and periventricular (A, C, E) T2 hyperintensities with varying degrees of enhancement (B, D, F). Note T2 hyperintensity at T7-T10 with edema (G, arrows) and gadolinium enhancement (H, arrows).近皮层和脑室旁T2高信号病变伴不同程度强化，T7-10 T2高信号病变并水肿、强化。
FIGURE 2 Brain biopsy(A, hematoxylin & eosin) Vasculitis (black arrow), infarcts (white arrow), inset with macrophages. Transmural infiltration by macrophages (B, CD163), lymphocytes, polymorphonuclear leukocytes (C, trichrome), and numerous eosinophils (D, arrows, hematoxylin & eosin), destroying the vessel wall (E, arrow, smooth muscle myosin). (F, hematoxy G, trichrome) Chronic, scarring phase. (H) Minimal perivascular myelin loss.A、HE染色，显示血管炎（黑箭头），梗死（白箭头），并巨噬细胞浸润。B、CD163染色，显示跨壁炎性细胞浸润，包括巨噬细胞、淋巴细胞。C、三色染色，显示中性粒细胞浸润。D、HE染色，显示大量嗜酸性粒细胞浸润（箭头）。E、平滑肌球染色，显示血管壁破坏（箭头）。F、苏木精根皮红藏红花染色，G、三色染色，显示慢性疤痕期。H、劳克坚牢蓝染色，显示血管周围少量髓鞘丢失。
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143. Variant of Guillain-Barré syndrome with spinal cord involvement 并脊髓受累的变异吉兰巴雷综合症A 48-year-old man presented with ascending sensory deficits over 12 hours, followed by urinary retention. He had areflexia, mild lower extremity weakness, sensory ataxia, and a T2 sensory level. Smooth pursuit was impaired, but cranial nerves were otherwise normal. Diagnostic evaluation demonstrated CSF cytoalbuminologic dissociation and demyelinating polyneuropathy fulfilling the electrodiagnostic criteria for Guillain-Barré syndrome (GBS).1 Laboratory evaluation had normal results, including vitamin B12; anti–neuromyelitis optica, antineuronal, and ga and oligoclonal bands. Myelopathy was confirmed on MRI (figure). This case highlights that acquired acute demyelination may rarely affect the peripheral and CNS simultaneously (GBS–transverse myelitis overlap syndrome), likely related to common autoimmune-mediated pathomechanisms.一名48岁的男性患者过去12小时出现上升性感觉缺失，随后出现尿潴留。他还出现反射消失，轻度下肢无力，感觉性共济失调，T2水平存在感觉平面。平滑追踪受损，而其他颅神经则正常。实验室检查显示脑脊液蛋白细胞分离，以及脱髓鞘性多发神经炎--符合吉兰巴雷综合症的电生理诊断标准。其他实验室检查正常，包括维生素B12、NMO抗体、神经元抗体、神经节苷酯抗体，及寡克隆带。MRI证实存在脊髓病。这个病例强调获得性急性脱髓鞘疾病可以少见的同时累及周围和中枢神经系统（吉兰巴雷横断脊髓炎重叠综合征），可能与共同的免疫介导机制相关。 FIGURE Spinal MRI 1 week after symptom onsetMRI of the spine demonstrates longitudinal (A, sagittal plane) T2 hyperintensities (C7/T1 to T3) affecting the dorsal columns more than the lateral columns (B, axial image at the level of T2).MRI显示T2相C7/T1至T3的长段高信号病变，T2椎体水平横断面显示更多的累及背侧而不是外侧。
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144. Radiation-associated symptomatic carotid artery disease with ipsilateral radiodermatitis放射性症状性颈动脉病变伴同侧放射性皮炎A 68-year-old man with a history of squamous cell carcinoma of the tongue treated with external beam radiation therapy in 2007 presented in 2013 with acute onset expressive aphasia and right lower facial weakness. MRI confirmed an acute infarct in the left frontal cortex. CT angiography revealed occlusion vs critical stenosis of the left internal carotid artery, new from 2007. Examination of the patient's neck revealed ipsilateral radiodermatitis (figure). Therapeutic neck irradiation has been associated with accelerated atheromatous disease, carotid artery stenosis, and increased risk of stroke.1,2 Ipsilateral radiodermatitis may be a sign of underlying carotid artery stenosis.一名68岁男性，2007年舌鳞状细胞癌行外放射治疗，2013年出血急性表达性失语及右侧中枢性面瘫。MRI证实左侧额叶急性脑梗塞。CTA显示左侧颈内动脉闭塞，与2007年比较、考虑新发。患者颈部检查显示同侧放射性皮炎。颈部放疗与动脉粥样硬化、颈动脉狭窄、卒中相关。同侧放射性皮炎可作为潜在颈动脉狭窄的提示征。 FIGURE Unilateral carotid artery disease with ipsilateral radiodermatitisNeck CT angiography maximum intensity projection images show patent right internal carotid artery (A) and left internal carotid artery (B) with focal tapered occlusion vs critical stenosis distal to the bifurcation. Clinical pictures of the patient's neck show (C) normal skin on the right and (D) radiodermatitis on the left.A、颈部CTA MIP相显示右侧颈内动脉通畅，B、左侧颈内动脉局限性锥形闭塞，分叉远端重度狭窄。C、患者右侧正常颈部皮肤，D、左侧放射性皮炎表现。http://www.neurology.org/content/84/5/e31.full?sid=ce3f36f3-248c-444e-aa02-e31
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先做个标记，有空再来细细研究
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145. Extracorporeal membrane oxygenation体外膜肺氧合—不常见的胼胝体微出血原因Uncommoncause of corpus callosalmicrohemorrhageA 47-year-oldman presented with respiratory failure secondary to influenza requiring venovenousextracorporealmechanical oxygenation (ECMO) for 10 days. MRI performed for mentalstatus changes revealed numerous microhemorrhages predominantly involving thecorpus callosum (). ECMO was the o there wasno history of substance abuse, hypertension, or other explanation.一名47岁的男性患者流感后出现呼吸衰竭，经静脉-静脉转流体外机械氧合（ECMO）10天。因为智能状态改变行MRI检查发现累及胼胝体为主的多发微出血。ECMO是仅有的可能原因，患者没有药物滥用、高血压和其他可能的因素。 Neurologic injury occurs in half of ECMO-treated patients andincludes anoxic injury, various hemorrhages, and infarctions. Callosal microhemorrhage is a rareECMO complication and may be a consequence of continuous heparin infusionduring ECMO and hemorrhagic diathesis. Neurologic complications are morecommon with venoarterial ECMO than venovenousECMO.一半ECMO治疗患者出现神经系统损伤，包括缺氧损伤、各种出血及梗死。胼胝体微出血是ECMO的一种罕见并发症，可能是ECMO治疗期间持续使用肝素及出血潜质的结果。静脉-动脉转流ECMO较静脉-静脉转流ECMO更常出现神经系统并发症。.FIGURE MRI brain shows corpus callosum microhemorrhagefollowing extracorporeal mechanical oxygenation(A, B) Noncontrast axial susceptibility-weighted imagesdemonstrate widespread microhemorrhages within splenium and genu of the corpuscallosum. There is also involvement within subcortical and periventricularwhite matter. 非增强轴位SWI相显示胼胝体压部和膝部广泛的微出血。同时累及皮层下及脑室旁白质。(C)Noncontrast fluid-attenuated inversion recovery images demonstratehyperintensity within the corpus callosum.非增强Flair显示胼胝体高信号病变。
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146. High-resolutionMRI of radiation-induced intracranial vasculopathy放疗诱发的颅内血管病变的高分辨MRI表现A 46-year-old womanwithout traditional stroke risk factors presented with acute left limbsnumbness. Two years prior, she had a right cranio-orbital junctionmeningioma resected and received radiation therapy (target dose DT 60 Gy/30 F, 5 F/W, 2 Gy/F). MRI revealed a diffusion-positive focuswithin the anterior periventricular white matter. CT angiography showed &50%right middle cerebral artery (MCA) stenosis. High-resolution MRI (HRMRI)suggested concentric vessel wall thickeningwith contrast enhancement ().一名46岁没有传统的卒中危险因素的女性患者出现急性左侧肢体麻木。患者2年前有右侧颅眶部脑膜瘤切除术史及放疗史（治疗方案:60Gy/30次，每周5次，每次2Gy）。MRI显示脑室旁前部一弥散受限病灶。CTA显示右侧大脑中动脉&50%的狭窄。高分辨MRI提示同心性血管壁增厚并强化。FIGURE MRI, CT angiography, and high-resolution MRIfindings of the patient(A) Brain MRI shows anacute lesion within the anterior periventricular white matter (arrow).MRI显示脑室旁前部白质一急性病灶。 (B) CT angiography shows right middle cerebral arterystenosis (arrow). CTA显示右侧大脑中动脉狭窄（箭头）。(C) On T1-weighted contrast-enhanced high-resolution MRI,a concentric vessel wall enhancement is revealed (arrow, and on the magnifiedimage).高分辨MRI T1加权增强相显示同心性血管壁增厚（箭头，及放大图）。The precise mechanism ofradiation-induced intracranial large vessel arteriopathy is not understood.Although radiation is more commonly associated with arteritis, radiation maycontribute to carotid atherosclerosis and stenosis. In our patient, the features of MCAwall lesions on HRMRI are consistent with cerebral vasculitis放疗导致的颅内大动脉病变的确切机制并不清楚。放疗通常导致动脉炎，但是放疗也可能导致动脉硬化及狭窄。在我们的病人，高分辨MRI显示大脑中动脉血管壁同心性增厚的特点与脑动脉炎的表现相符。.
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147. TeachingNeuroImages: Cortical blindness following acuteobstructive hydrocephalus by a colloid cyst胶样囊肿导致的急性阻塞性脑积水及皮质盲A 46-year-old woman wasadmitted for acute headache, postseizure confusion, and visualloss. Urgent head CT scan showed obstructive hydrocephalus due to a colloidcyst (). External ventricular drains insertedemergently demonstrated CSF under pressure, above 40 mm H2O. MRI confirmed the suspected diagnosis of a colloidcyst and highlighted bilateral occipital lobe infarcts (). CT angiography showed no thrombosisof the posterior cerebral arteries (PCAs). After neurosurgical excision of thecolloid cyst, the patient remained blind. The presumed mechanism of infarctionwas acute compression of the PCAs against the edge of the tentorium cerebelli.一名46岁的女性患者因急性头痛、抽搐后意识障碍和视力丧失入院。急诊CT显示胶样囊肿导致的急性阻塞性脑积水。急诊置入脑室外引流显示脑脊液正压，超过40mmH20。MRI证实怀疑的胶样囊肿，并显示双侧枕叶梗死。CTA显示大脑后动脉没有血栓。囊肿在神经外科切除后，患者仍然失明。推测梗死的机制是靠近小脑幕边缘的大脑后动脉急性受压导致。FIGURE 1 Axial CT scan images without iodinated contrastmedia show enlarged occipital lobeHorns of the lateralventricles consistent with obstructive hydrocephalus secondary to the presenceof a colloid cyst in the foramen of Monro.轴位非增强CT显示侧脑室枕角的扩大，符合室间孔胶样囊肿继发急性梗阻性脑积水表现。FIGURE 2 MRI scan images(A) Sagittal T1without gadolinium contrast injection shows a colloid cyst of 14 mm.非增强矢状位T1相显示囊肿大小14mm。(B) Diffusion-weighted image depicts restricted diffusionin both occipital lobes. DWI显示双侧枕叶弥散受限。
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