> 的Acute Necrotizing Encephalopathy 文件夹下。
Acute Necrotizing Encephalopathy Associated With Human Herpesvirus-6 Infection
人类疱疹病毒6型感染相关性急性坏死性脑病
An extremely rare case of acute necrotizing encephalopathy caused by human herpesvirus-6 variant type B infection is reported. The patient, a 14-month-old previously healthy female, presented with high fever and generalized tonic convulsion followed by rapid deterioration of consciousness. On the second day of the illness, the protein level of the cerebrospinal fluid increased without pleocytosis. On the third day, magnetic resonance images demonstrated symmetric, abnormal signal intensity lesions in the bilateral thalamus, cerebellum, and brainstem.On the fourth day, characteristic maculopapular
rash of exanthema subitum appeared on the trunk. Human herpesvirus-6 deoxyribonucleic acid was detected by the polymerase chain reaction in the serum, and immunoglobulin G and immunoglobulin M of serum human herpesvirus-6 were positive. On the twelfth day of illness, the patient died as a result of severe brain damage. Acute necrotizing encephalopathy should be included in the differential diagnosis when examining infants and young children with fulminating consciousness disturbance and intractable convulsion. In addition, as a causative virus, human herpesvirus-6 has to be considered at
the pre-eruptive stage of exanthema subitum. Magnetic resonance images are useful because they reveal the characteristic distribution of lesions specific to acute necrotizing encephalopathy.
Figure 1. T2-weighted magnetic resonance image on day 3 at the level of the thalamus (turbo spin echo) reveals symmetrical high signal intensity lesion in the bilateral thalamus and surrounding white matter, including the internal capsule. TR/TE ＝ 4000 ms/100 ms.
Figure 2. Diffusion-weighted image (single shot echo planar image) reveals significantly decreased diffusion in the bilateral thalamus. TR/TE ＝ 6000 ms/120 ms; b-value ＝ 1000.
Figure 3. Apparent diffusion coefficient image reveals a specific pattern, namely, slightly high signal in the center of the thalamic lesion, low signal in the surrounding thalamus, and high signal in the peripheral white matter.
这是一篇关于人类疱疹病毒6型感染相关性急性坏死性脑病的报道。一个14个月的女婴高热，全身性僵直阵挛型发作伴意识丧失。第二天腰穿检查示脑脊液蛋白增多，但没有脑脊液细胞增多。第三天MRI检查示对称的，双侧对称的丘脑、小脑、脑干异常密度信号病变影。第四天典型的婴儿玫瑰疹的斑丘疹在躯干出现。在血清中用PCR方法检查出人类疱疹病毒6型脱氧核糖核酸，且人类疱疹病毒6型IgG、IgM均阳性。病程12天后，患儿死于严重的脑损伤。急性坏死性脑病应该成为婴幼儿意识丧失、顽固性抽搐的一个重要鉴别诊断要点。另外对于病原菌，人类疱疹病毒6型在幼儿急疹出现的潜伏期应该考虑到。MRI在急性坏死性脑病的诊断中特征性分布的坏死有极大的帮助。
Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan
儿童急性坏死性脑病：一种新的日本和台湾流行的急性脑病
这是Mizuguchi M发表的儿童急性坏死性脑病的第二篇文章，发表在Brain & Development 19 (1997) 81-92，是篇综述。对ANEC的症状、发病机制、病原学、病理特征、鉴别诊断进行一系列讲述。
Diagnostic criteria for ANE：
ANE诊断标准：
1. Acute encephalopathy following a viral febrile disease. Rapid deterioration in the level of consciousness. Convulsions.
2. No CSF pleocytosis. Increase in CSF protein commonly observed.
3. CT or MRI evidence for symmetric, multifocal brain lesions. Involvement of the bilateral thalami. Lesions also common in the cerebral periventiricular white matter, internal capsule, putarnen, upper brain stem tegmentum and cerebellar medulla. No involvement of other CNS regions.
4. Elevation of serum aminotransferases of variable degrees. No increase in blood ammonia.
5. Exclusion of resembling diseases. A. Differential diagnosis from clinical viewpoints.Overwhelming bacterial and viral infections, and fulminant hepatitis; toxic shock, hemolytic uremic syndrome, and other toxin-induced diseases; Reye syndrome, hemorrhagic shock and encephalopathy syndrome, and heat stroke. B. Differential diagnosis from radiological (or pathological) viewpoints.Leigh encephalopathy and related mitochondrial cytopathies; glutaric acidemia, methylmalonic acidemia, and infantile bilateral striatal necrosis; Wernicke encephalopathy, and carbon monoxide poisoning; acute disseminated encephalomyelitis, acute hemorrhagic leucoencephalitis, and other types of encephalitis and vasculitis; arterial or venous infarction, and the effects of severe hypoxia or head trauma.
Acute relapsing encephalopathy mimicking acute necrotizing encephalopathy in a 4-year-old boy
一4岁男孩类似急性坏死性脑病的急性复发性脑病
A 4-year-old boy showed two episodes of encephalitis/encephalopathy involving disturbed consciousness, convulsion, and paresis associated with the elevated levels of protein and myelin basic protein of the cerebrospinal ¯uid. MRI studies of the brain revealed
symmetrical lesions in the brain stem and thalami at the ®rst episode, and additional lesions were found in the cerebellum involving both the gray and white matter in the second episode. The intensities of MRI lesions were low in T1 and high in T2. These episodes were followed by an elevation of the anti-viral antibody titers, for in¯uenza A virus during the ®rst episode and for adenovirus during the second. In the second episode, intravenous methylprednisolone therapy resulted in rapid improvement of his neurological signs.
Fig. 1. MRI findings on day 6 are shown in (A±C), and, those 3 months after the onset are shown in (D±F). At the onset, symmetrical lesions (A,B) and thalami were low in T1-weighted and high in T2-weighted images. High T2- weighted images are shown for the right cerebellar lesion (B). Multi focal high T2-weighted images are shown for small circumscribed lesions in the posterior limb of the internal capsule (small arrow) and the putaminal lesions localized in the very lateral aspect toward the claustrum (large arrow) (C). Three months later, marked atrophy of the pons and some mild change in the related cerebellum were found (D,E), while those at the thalami were diminished (F).
Fig. 2. (A,B) Images on day 6, and (C,D) represent images 3 months later. Symmetrical high intensity lesions in the medulla and white and gray matters of the cerebellum were detected in T2-weighed images (A). Sagittal T1-weighed sections through the cerebellar hemisphere showed diffuse changes of low intensity in the cerebellum (B). Atrophic changes were detected in the images 3 months later (C,D) with a persistent lesion at the ventral pons (C).
这是一篇Original article，讲述急性播散性脑脊髓炎ADEM与急性坏死性脑病ADE的区别和临床分析。一4岁男孩表现出2个阶段的脑炎/脑病，包括神智不清、抽搐，轻瘫，并伴有脑脊液中蛋白及髓鞘碱性蛋白升高。MRI检查可见在第1阶段脑干和丘脑对称病灶，并且在2阶段看到小脑灰白质其它病变。这2次的发作都随之伴有相关病毒抗体的升高，在第一次发作后流感病毒A抗体滴度升高，而第二次发作为腺病毒抗体滴度升高。在第二次发作，静脉的甲强使用迅速的改善了神经症状
文章结尾说道：There are several possible diagnoses for this case. ANE may have a wider clinical spectrum than is currently believed, including a relapsing condition. Otherwise our case could have had a fortuitous combination of two episodes of virus-induced encephalitis, one due to influenza A and the other due to adenovirus. Accumulation of MRI data on children with virus-induced encephalitis/encephalopathy, may reveal relationships among postinfections of encephalitis, ANE and ADEM.
对这个病例的诊断有几种可能，ANE也许有着较我们现在所认识的更广的临床镨，包括这种复发情况。否则我们这个病例是偶然的2次病毒诱导的脑炎发作，一个是流感A病毒，另一个是腺病毒。从儿童病毒性脑炎的MRI资料看来，可能揭示ANE和ADEM2种疾病在感染后的脑炎变化。
Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy
联合低温及抗细胞因子治疗流感病毒A脑病
Two children with influenza A-related encephalopathy were treated with a combination of mild hypothermia (deep body temperature of the forehead: 358C) and anticytokine agents (high-dose methylprednisolone and ulinastatin), while receiving amantadine. One of the cases exhibited acute necrotizing encephalopathy on computed tomography (CT). Although no severe complications occurred, correctable hypokalemia and hyperglycemia occurred in both cases. Both patients recovered without any neurological sequelae. Our therapeutic protocol appears to be effective for managing influenza A-related encephalopathy.
Fig. 1. Clinical course of case 1. The treatment, clinical symptoms, and brain temperature (deep body temperature on the patient's forehead) are summarized. Additional drugs used included famotidine, cefotiam, tocopherol (vitamin E), and furosemide (see text). Methyl PSL, methylprednisolone.
Fig. 2. Clinical course of case 2. The treatment, clinical symptoms, and brain temperature are summarized. Drugs not listed in Fig. 3 included dopamine,famotidine, ceftriaxone, tocopherol (vitamin E), menaquinone (vitamin K2), nafamostat, and heparin (see text). Methyl PSL, methylprednisolone; G/I,glucagon-insulin therapy; TRH, thyrotropin releasing hormone.
这上面2张图分别是2例患者的病程描述，很有特点，大家可以学一下，以后讲课写文章可用这样的表达方法：）
Fig. 3. CT and MRI findings. (A) CT findings in case 1. CT images obtained (a) at the onset of convulsions at the community hospital (26 Jan 1999), (b) on hospital day 1 (27 Jan.), (c) on day 5, and (d) 3 months after the onset of the disease. (B) CT and MRI ®ndings in case 2. CT images were obtained (a) at the onset of consciousness disturbance at the referring hospital (1 Feb 1999), (b) on hospital day 1 (3 Feb) and (c) on day 11. (d) Follow-up MR images (T1-weighted) were obtained 2 months later.
文章最后Influenza encephalopathy often produces severe neurological residua. Approximately 70% of patients with acute necrotizing encephalitis die or develop severe neurological sequelae . Although we are reporting the results of only two cases, both patients recovered without any neurological residua. Our therapy protocol may improve the neurological recovery in patients with influenza A encephalopathy.
Diffusion-Weighted MR Imaging Findings of Acute Necrotizing Encephalopathy
Summary: Multiple, symmetrical brain lesions affecting the bilateral thalami and cerebral white matter, which often show a concentric structure on CT and MR images, characterize acute necrotizing encephalopathy (ANE) of childhood. We describe the imaging findings of a 2-year-old child with ANE obtained with diffusion-weighted MR imaging.
We discuss the significance of these findings, as well as the pathophysiology of ANE lesions, with reference to the appearance of the disease as revealed by diffusionweighted
MR imaging.
FIG 1. Conventional MR image findings in acute-stage ANE in 2-year-old-girl. A, Axial T2-weighted images show symmetric bilateral hyperintensities located deep in the cerebral-cerebellar white matter, bilateral thalami, and pontine tegmenta. B, Contrastenhanced T1-weighted images reveal bilateral symmetric ringlike enhancement around the central hypointense portion of thalamic and central deep cerebral white matter lesions. Homogeneous enhancements in the pontine tegmenta and cerebellar white matter are also seen. C, T2-weighted gradient-echo MR image of the brain shows multiple, small hemorrhagic foci located on the central portion of thalamic lesions.
FIG 2. Diffusion findings in acute-stage ANE in a 2-year-old girl. A, Diffusion-weighted MR images (b 1000) show bilateral symmetric diffuse hyperintense lesions in the cerebellar-cerebral white matter, thalami, and pontine tegmenta. B, The ADC map shows three different patterns of the thalamus and cerebral white matter. The center of the lesions shows higher ADC values than those of normal parenchyma, the peripheral portion of central lesions shows very low ADC values, and outside the thalamus and cerebral white matter are high ADC values, findings compatible with vasogenic edema. Note that the splenium of corpus callosum, pontine tegmanta, optic radiatia, and cerebellar
white matter have low ADC values in the central portion and high ADC values in the peripheral portion. C, Mean ADC values and pattern on the right thalamus (A, center of thalamic lesions; B, periphery of the central thalamic lesions; C, outside portions of the thalamic lesions).
FIG 3. On follow-up MR examination, 25 days after onset of symptoms, T2-weighted (A) and fluid-attenuated inversion recovery (B) MR images of brain show neuronal tissue destruction on the central portion of cerebral white matter, bilateral optic radiation, deep cerebellar white matter, and pontine tegmanta. Necroses are also seen on the central portion of thalamus and deep cerebral white matter. T1-weighted MR image (C) shows subacute hemorrhagic changes in the central necrotic portion of thalamus.
Influenza B Acute Necrotizing Encephalopathy: A Case Report and Literature Review
流感病毒B致急性坏死性脑病：病例报告及文献综述
The case of a 9-year-old girl with acute encephalopathy with evidence of influenza B virus infection and bilateral basal ganglia lesions is reported. The literature on acute necrotizing encephalopathy is reviewed, and other causes of bilateral basal ganglia lesions are discussed.
Figure 1. Magnetic resonance imaging of the brain on day 2 after admission (day 6 of the patient’s illness). (1a) Coronal inversion recovery image (TR7000/TE60) illustrates hypointense lesions involving the putamina and parts of the thalami. (1b) Transverse T2 image (TR3645/TE102) reveals increased signal intensity in the corresponding areas above.
Figure 2. Computed tomography image of the head on day 3 after the patient’s admission (day 7 of her illness). Note symmetrical bilateral basal ganglia lesions with sparing of the caudate nuclei and involvement of the thalami.
Influenza-associated acute necrotizing encephalopathy in a 2-year-old American child
Increasing numbers of cases of children with encephalopathy, associated with influenza virus infection, have recently been reported from Japan. A subset of these cases demonstrates imaging findings of bilateral thalamic and deep cerebellar nuclei necrosis consistent with acute necrotizing encephalopathy (ANE). This case report describes such a case of ANE in an American child.
Fig. 1. (A) MRI diffusion weighted imaging shows regions of restricted diffusion in the bilateral thalami and deep cerebellar nuclei bilaterally consistent with ischemia or infarction from global hypoxia. (B) MRI T2 weighted image with increased signal intensity in the bilateral thalami.
Acute necrotizing encephalopathy (ANE), first described in 1995, is characterized by
symmetrical brain lesions involving the thalami, cerebral periventricular white matter and cerebellar medulla [1]. It is often associated with influenza infection, but has also been found in infections with HHV-6 and measles. In a recent case series of 148 patients with influenza-associated encephalopathy, Morishima et al. [2] found the majority of patients were healthy children less than five years of age with documented influenza A infection, predominantly of the H3N2 subtype. In this series, 10% of the children demonstrated head imaging findings consistent with ANE.
The pathogenesis of ANE and influenza-associated encephalopathy remains largely unknown. Cerebrospinal fluid indices often do not demonstrate pleocytosis, and as with our patient, viral culture or PCR for influenza virus is often negative [1,2]. Histopathologic
Fig. 1. (A) MRI diffusion weighted imaging shows regions of restricted diffusion in the bilateral thalami and deep cerebellar nuclei bilaterally consistent with ischemia or infarction from global hypoxia. (B) MRI T2 weighted image with increased signal intensity in the bilateral thalami.specimens of the brain from patients with ANE demonstrate diffuse edema, perivascular hemorrhage, and necrosis of neurons and glial cells, and viral antigen is not detected. Cytokines, in particular IL-6, IL-10 and TNF-alpha, have been implicated in the pathogenesis of influenza-associated encephalopathy, although the exact mechanism remains to be elucidated [3].
Given the preponderance of cases in Japan, Asian heritage or local environmental factors may contribute to the development of ANE or encephalopathy [our patient was of Filipino descent]. However, in 2002, eight cases of influenza-related encephalopathy were
reported in the state of Michigan in the United States [4]. The mortality rate of influenzaassociated encephalopathy ranges from 15% to 30%. With the large numbers of cases in Japan, and possible increasing numbers in the United States, ANE and influenzaassociated encephalopathy should be recognized as a serious complication of influenza infection. Further studies to elucidate the pathogenesis of this entity are needed.
During the past several years, influenzaassociated acute necrotizing encephalopathy has been well-recognized in Asia but has not yet been reported in the United States. We describe a 28-month-old patient who displayed the classical clinical features of acute necrotizing encephalopathy in association with a documented influenza A infection. This disease is characterized by fever, a rapid alteration in consciousness and seizures, with radiologic involvement of the bilateral thalami and cerebellum.
FIG. 1. Diffusion weighted imaging shows regions of restricted diffusion in the bilateral thalami and deep cerebellar nuclei bilaterally (A). Fluid-attenuated inversion recovery imaging of the thalamus (B) and cerebellum (C) show regions of hyperintensity corresponding to the areas of restricted diffusion. The above areas enhance with intravenous contrast (D).
开卷有益，我找的文章不一定具有代表性，好几篇是临床病例，希望大家看后对病毒性相关脑炎有进一步的认识。：）
写作中。。。。。。。。。
儿童急性坏死性脑病三例.pdf (169.64k)
谢谢！！！
dreamsound
你是我的偶像
谢谢！！！
首先多谢docwufg战友提供的病例，让我们对儿童急性坏死性脑病有了一定的了解
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多谢您的关注,我也学习到ANEC进一步的进展.
我的病例一开始并不被大家所关注，本来我是想进一步来跟大家谈丘脑病变多种病的影像特点的。跟帖者很少让我失望。只好作罢。
我想一是我们对病理、对影像方面知识的不完整有关。其次，人云亦云，不求深究，缺乏对知识热情亦有关。
dreamsound版主的鼓励让我受宠若惊，晚些时候我将补充．
丘脑病变的常见病:
先天性或代谢病
炎症性疾病
双侧丘脑梗死
一氧化碳中毒
酒精中毒
严重疲劳或营养不良的成人as a result of an osmotic myelinosis
少见的有
肝脏疾病
Addison‘s病
利尿剂的应用
急性弥散性脑脊髓炎
Leigh’s 病
Canavan‘s病
Kearn-Sayre 综合症
另加:急性双侧丘脑病变如：梗塞、子宫内缺血缺氧
慢性双侧丘脑病变如：ＣＪＤ、Ｗernicke-Korsakoff、神经胶质瘤、Ｗilson、KSS、Ｆahr