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Balo同心圆硬化临床诊治进展（综述） - 丁香园
Balo同心圆硬化临床诊治进展（综述） - 丁香园
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Balo同心圆硬化临床诊治进展（综述）
2014-10-26 09:47
来源：丁香园
作者：幸福的味道
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Balo同心圆硬化常被看作是多发性硬化的一种罕见变异型。该病的患者表现为急性或亚急性神经系统症状的恶化，MRI显示脑白质内一个或多个同心圆性多层环状病灶。以往观点认为，所有Balo同心圆硬化都是致命性的。然而，核磁共振的应用使得对放射学表现为Balo病变的患者不同的自然病史以及其与多发性硬化和其他神经系统疾病之间的相关性有了更好的认识。重大的进展增加了对与Balo病变的形成相关的免疫病理发生机制的理解。然而，如何治疗急性损伤以及何时或是否开始治疗的认识尚不充分，尽管对于那些也符合多发性硬化诊断标准的Balo病变患者，我们的意见是采用多发性硬化疾病修饰治疗似乎也是合理的。简介：Marburg在1906年首次描述了Balo同心圆硬化，但该疾病变得更广为人知是在1928年，匈牙利神经病理学家Josef Baló发表了一例学生患者伴有右侧轻偏瘫之后出现视神经炎的报告，尸检显示为脱髓鞘病变，被称为同心性轴周性脑炎。传统上认为，Balo同心圆硬化是多发性硬化非典型表现型之一，与Marburg病，块状脱髓鞘，Schilder病和急性出血热白质脑炎等一起作为一组疾病，尽管这只是当时的情况，并且这些分类的实用性——除了块状脱髓鞘之外——是有争议的。曾经Balo同心圆硬化是一种尸检诊断，然而，核磁共振成像技术的出现促使对疾病的更好理解，许多Balo同心圆硬化的患者可在一次发病之后完全或几乎完全恢复。Balo同心圆硬化典型表现为脑白质内分散的同心性层状病灶，并通常被描述为一个洋葱皮样或轮状外观。这种独特的表现有助于区分传统的多发性硬化的脱髓鞘病变，以及与块状脱髓鞘病变相鉴别，后者通常大于2cm，伴有环状强化或开环强化，但没有层状结构。许多研究的结果提供了对Balo同心圆硬化放射学和病理学特征的理解；然而，对于当 Balo病变发生后其如何进展以及应该启动什么样的治疗相关的信息是缺乏的。本文的目的是描述这种疾病的临床特征，讨论其免疫发病机理和治疗方面的不确定性。流行病学和临床特征Balo同心圆硬化是一种罕见的疾病，很难精确估计患病率。Balo同心圆硬化患者平均发病年龄为34岁(范围3 – 62岁)。从一个小型系列病例报道的MRI数据显示女性和男性患者比例是2:13，并且该病似乎在东亚患者中更为常见，患病率最高的人群是南方汉族人群，台湾，和菲律宾人群。这些数据可能表明Balo同心圆硬化存在以人群为基础的遗传易感性，尽管我们也不能排除环境的影响。虽然该病发病没有季节性的偏好或与传染性疾病有明确的相关性，但观察性研究数据表明，自上世纪九十年代以后，菲律宾的Balo同心圆硬化的患者数量急剧下降，多发性硬化发病率保持稳定。一些研究人员推测这个数字的下降可能是由于菲律宾的快速“西方化”而致，在此期间考虑环境因素可能是较为重要的。尽管患者可以表现为多发性硬化的一些经典的局灶性症状，如局灶性力弱，共济失调，感觉障碍，或复视，但Balo病灶最常见的症状与其他任何颅内颅内病变的症状类似，包括头痛、认知损害、行为改变、少言、尿失禁、癫痫、失语、轻偏瘫等。在一项17例菲律宾患者的系列病例报道研究中显示，大约一半的患者有前驱期的症状，包括轻度发热，全身不适，头痛等。有时候，患者可能出现更为急性的神经系统障碍，类似于卒中的表现，在极少数情况下可能是无症状性的。Balo病变可被误诊为脑部肿瘤，如多形性胶质母细胞瘤或原发性中枢神经系统淋巴瘤，一些患者进行脑活检可排除肿瘤病变。其他鉴别诊断包括梗塞、脑脓肿、块状脱髓鞘。块状脱髓鞘有时是很难与Balo同心圆硬化相鉴别，因为后者也可发展形成块状病变簇状的外观，虽然块状病变没有簇状典型的Balo病变同心圆样改变。这种病变之间的相似性某种程度上模糊了两种疾病之间的区别，表明这些疾病是非典型脱髓鞘谱系疾病中的一种。历史的观点认为，Balo同心圆硬化认为是一种在发病后数周或数个月内逐渐进展导致严重残疾或死亡的疾病，诊断是基于尸检组织病理学的报告。然而，由于MRI的出现，Balo病变的自然疾病史可以得到更充分的认识。尽管Balo病变可能是致命的，但逐渐增加的认识表明，许多出现Balo病变症状的患者可以大部分或完全恢复。尽管这可能是因为对这些病变所致的真正临床预后的完全认识，但也可能，至少部分上，是由于早期的识别和治疗。第三个可能考虑的原因是，来自不同的疾病过程的患者预后情况较广泛，真正的Balo同心圆硬化是一种暴发性致命性疾病，Marburg样以及Balo样病变预后较好，如传统的块状脱髓鞘或多发性硬化，有时只是作为其他脱髓鞘过程的一部分出现。Balo病变的发生极少与其他疾病相关。具体地说，在抗水通道蛋白 4抗体血清反应阳性和阴性的视神经脊髓炎以及视神经脊髓炎谱系疾病中可以看到一些Balo病变的病例报道。此外， Balo样的病变也可见于一例进行性多灶性白质脑病患者及一例伴皮质下梗死和脑白质病的常染色体显性遗传脑动脉病（CADASIL）患者中。多个Balo样病变也见于一例有静脉注射毒品史和丙型肝炎的患者中，该患者既往采用过干扰素α治疗；脑脊液（CSF）人类疱疹病毒PCR检测阳性。 CSF中这种病毒在与儿科Balo同心圆硬化相关，并增加了可能的病毒性因素在这些病变发生中的可能性。影像学表现MRI技术的进展使得Balo同心圆硬化能够在患者活着的时候就进行诊断。T1加权成像显示Balo病变典型表现为交替等信号和低信号的同心圆样病灶；T2加权序列显示在所谓的T2高信号的风暴中心周围环绕着高信号的片层样结构；尽管也可见其他复杂的结构描述，包括马赛克式，莲花座状，或康乃馨状，甚至双杠状(见图1)。图1. MRI显示左侧顶叶同心圆硬化病灶。A图为T1加权像，B图为T2加权像。DWI序列可见高信号，通常位于病变的边缘。病变水肿很小，MRI增强更可能出现病变的周围，但偶尔也会多层的强化，相应的T2也出现高信号 层。不像典型的多发性硬化脱髓鞘病变，Balo病变主要发生在脑白质，不累及皮层的U形纤维。其他的病灶部位包括基底神经节、脑桥、小脑，也有报道称脊髓和视神经受累。Balo或Balo样病灶的结构因一个或两个至几个交替的脱髓鞘条带不同而不同，其病变大小可以小至不足 1厘米，也可以大到累及整个大脑半球 (图2、3)。Balo病变可能是多个病灶起病或孤立性病灶。连续的MRI使得能够对新形成的Balo病灶的演变有深入的认识，尽管研究对于洋葱环状结构是同时出现还是从核心遵循一定的半径向外发展尚有争议。图2. MRI显示在采用皮质激素治疗前后左侧顶叶同心圆硬化病灶。A和B图（3倍放大）为T2加权像，C图和D图（4倍放大）为采用静脉甲基强的松龙治疗2个月后的T2像。图3. MRI显示在右侧脑室旁白质的同心圆硬化病灶。A图为Flair像，B图为轴位T2像，C图为矢状位T2像，该例女性患者36岁，主因晨起出现突发左侧偏瘫就诊，起初被认为是卒中。注意在两侧大脑半球深部均出现伴随的更典型的脱髓鞘病变。连续的影像学检查还提供了对Balo病变长期发展的理解和认识。有些病变可能最终失去同心圆环状结构模式，并出现弥漫性的块状脱髓鞘病变或大片斑块。其他还包括超过1年的持续性同心圆外观或回归到最终像一个更典型的脱髓鞘斑块的大小。Balo病变的核磁共振波谱成像显示胆碱/乙酰天门冬氨酸比率有中等程度的增加。磁化传递成像显示急性病变的病灶核心区磁化传递率显著降低，周围同心圆状病灶也有不那么明显的下降，并且随着病变的发展，周围结构的磁化传递率先下降后上升。不像其他的急性脱髓鞘病变或高分化肿瘤，Balo病变不会导致PET吸收的增加，因此这种成像技术可能是在描述诊断不清楚的病灶特征时采用标准成像基础上的一种有用的辅助手段。7 T MRI磁敏感加权成像显示多个低信号的病灶，与微出血以及扩张的静脉表现相一致。尽管这些变化尚未通过病理学方面的确认，但表明微血管受累参与病变的发生。病理学特征和免疫发病机理Balo病变的病理学特征已有很充分的描述，典型的特征包括脑白质少突胶质细胞丢失和脱髓鞘 (类似于多发性硬化免疫病理III型)，皮层灰质并不受累(不像传统的多发性硬化)。宏观上看，病变通常比传统的多发性硬化病变体积大，其交替的环状外观可归因于相对髓鞘保留和缺失以及相对轴索不受累，可以形成所谓的洋葱皮样外观。相对髓鞘保留很少包含正常的髓鞘，而是早期或部分的髓鞘脱失，这也许是持续的髓磷脂分解的迹象，既往脱髓鞘的区域重新形成新的髓鞘。然而，来自新生典型多发性硬化病变的尸检结果研究也可出现几乎完整的髓磷脂与脱髓鞘脱交替出现，类似于另一种更典型II型多发性硬化患者中Balo或Balo样病变，其特征包括在退化的髓鞘上补体沉积，伴有髓鞘重新形成的区域。星型胶质细胞病变也被作为一个Balo病变的标志性特征。 肥大的星形胶质细胞分布于整个病灶中，并且其出现与少突胶质细胞有密切的关系。为什么Balo病变发展称片状或同心圆状？病变似乎是因为对未知刺激的反应之后在血管周围区域产生的，导致巨噬细胞和激活的小胶质细胞产生细胞因子，氧自由基或其他神经介质，诱导脱髓鞘形成。这种同心圆形的模式代表着化学介质从核心区像波浪一样像外传播 (图4)。图4. Balo病变形成的假设模型损伤可能是由于缺血缺氧所致，可能的机制包括线粒体呼吸链复合体IV及其催化组件细胞色素C氧化酶受抑制。当病灶形成时在其周围发现低氧诱导因子，D – 110，缺氧诱导因子1α和热休克蛋白70等，这在一定程度上发挥了神经保护作用，使得在环状的脱髓鞘之间有部分髓鞘得以保留。数学模型表明，同心性分层中还有可能是由于非线性的单核细胞趋化和小神经胶质细胞对非特异性化学诱导物反应，导致在这些细胞聚集的区域脱髓鞘。髓鞘脱失也可能与星型胶质细胞病变有关。连接素形成星形胶质细胞之间以及其与少突胶质细胞之间缝隙连接。在扩大的Balo病变的前缘，星形胶质细胞水通道蛋白4和连接素(如Cx43)明显减少，并且与少突胶质细胞和髓鞘密切相关。这个观察表明，星形胶质细胞和少突细胞相互作用的破坏导致脱髓鞘。相关补体，免疫球蛋白沉积或抗水通道蛋白 4抗体的缺乏使得该病的免疫病理学与视神经脊髓炎截然不同。与多发性硬化有怎样的临床相关性?Balo病变和多发性硬化之间的关系还不清楚。Balo同心圆硬化可能是多发性硬化的一个变异型或一个单独的但又相关性的疾病。显然，传统的多发性硬化和Balo病变不仅仅只是病理学表现方面的重合。Balo病变可以发生在除复发缓解型之外传统的多发性硬化发生的临床过程中。至少有55%存在Balo或Balo样病变的患者表现为MRI显示的典型多发性硬化病变(图2)。在2例由Chaodong及其同事报道的患者中，患者起初发生的Balo病变复发的时候伴有典型的多发性硬化脱髓鞘病变。此外，一些脑脊液寡克隆带阳性的Balo病变的患者可能继续发展为多发性硬化，这就提出了一个问题：寡克隆带阳性是否可能预测进展为多发性硬化的风险？并不是所有Balo病变的患者都出现寡克隆带，在一项纳入11例 Balo样病变患者研究中，只有1例患者出现脑脊液寡克隆带的生成。治疗急性病灶用来指导治疗急性症状性Balo病变的随机对照临床研究十分稀少，考虑到其病变的罕见性以及临床表现的异质性，这样的结果并不出人意料。大多数作者和临床医生的共识均是基于病例报告，同时也是根据我们的经验，糖皮质激素是推荐的一线治疗方法。对于那些对糖皮质激素治疗不完全反映或者无反应的病变以及相关的临床综合征，治疗方法尚不清楚。一般来说，我们认为血浆置换似乎是合理的二线治疗选择，因为这种治疗通常是作为更典型的及块状脱髓鞘病变患者的抢救治疗。环磷酰胺，静脉注射免疫球蛋白以及免疫吸附等治疗也可用于个体患者中，有时可结合更长期的治疗方法，如硫唑嘌呤或米托蒽醌治疗。然而，这些治疗方法的可能获益或者哪一种更佳的数据尚不充分。研究报道，在一例临床和影像不断进展的Balo同心圆硬化患者中，糖皮质激素，血浆置换，或静脉注射环磷酰胺等治疗后仍效果不佳后可尝试使用阿仑单抗治疗。尽管在该例患者中没有病理学检查证实是Balo髓鞘脱失，但MRI已经提示是该病。到使用阿仑单抗进行免疫诱导治疗时，患者的扩展残疾状态量表得分为6.5分，尽管已经开始了治疗，患者仍继续恶化，并且在阿仑单抗治疗6个月后死亡。研究人员推测如果更早期干预是否会有较好的预后。多发性硬化疾病修饰治疗会发挥作用吗?用于指导已发生Balo病变的患者持续治疗的信息十分稀缺。我们的意见是，如果Balo病变发展的方式符合复发缓解型多发性硬化诊断标准中所述的时间和空间分布的特征，考虑采用多发性硬化疾病修饰疗法来治疗Balo病变可能是合理的。然而，尚没有随机对照试验的报告，也没有多少证据可用来指导决策。Balo病变在临床和放射学方面是否孤立性发生可预测临床孤立综合征的发生，因此其本身是否应该及时的治疗目前还不清楚。根据我们的经验，许多孤立性Balo病变的患者与更传统的多发性硬化患者相比，其预后更好，因此观察和期待疗法可能是合理的。此外，在典型的临床孤立综合征的患者中，如果不伴有额外的临床静止性病变，其复发风险相对较低。或者，如果出现了Balo病变且伴有额外的传统多发性硬化脱髓鞘病变和寡克隆带阳性或者两者兼有，那么转化为多发性硬化的风险似乎可能性更高。在患者及其神经内科医生进行疾病决策时，应该考虑这些因素以及不良事件发生的相对风险的治疗。如果把Balo病变作为多发性硬化疾病过程的一部分进行治疗，我们支持将传统的一线疾病修饰疗法作为适当的第一步治疗选择。一些报道提到采用干扰素β进行治疗；然而，有关这种治疗方法的资料很少，在更多明确的推荐之前需要进行更进一步研究。在一项采用那他珠单抗治疗Balo同心圆硬化患者的病例报道中，该例患者有多发性硬化的病史，在使用干扰素β1a治疗后出现了Balo病变，在静脉注射糖皮质激素治疗后症状恶化，而在五个周期的血浆置换和五个周期的免疫吸附治疗后病情好转。患者在发病3个月后接受那他珠单抗治疗，在超过3年的随访期内无复发。预后出现Balo病变症状的患者预后不尽相同(表)。可见临床和影像学均完全恢复的患者；然而，进展性Balo同心圆硬化的患者可能会死亡或遗留显著的残疾。在这两种极端情况之间的预后更常见。表. Balo同心圆硬化患者的预后 患者例数孤立性Balo或Balo样病灶额外的MS样病灶发展为复发性疾病或MS的例数发展为NMO的例数随访时间预后Chen et al, 19995NRNR0NR1.6y100%存活Karaarslan et al,20015140030m100存活伴轻微缺损Chaodong et al,20087253/708.2y100存活伴轻微缺损Wallner-Blazek et al,201310461/601.8y100%存活，88%伴轻微缺损Scott, 20116517NR12238%<2y;26%>2y14%死亡，严重残疾或进展Tabira, 200917NRNRNRNR2.3m100%死亡Yao et al, 19946NRNRNRNR1.8m100%死亡 以单独Balo病灶发病的患者与那些复发缓解型多发性硬化发生中出现Balo病灶的患者相比较的预后研究较少。在一项纳入10例Balo样病变患者的研究中，83%的患者出现显著的或完全性的临床改善，且没有死亡病例的报告。在同一研究中，对6例 Balo样病变患者的平均长期随访期为1.8年，其中4例患者没有更进一步的髓鞘脱失。在5例已经有典型的脱髓鞘病变的患者中，只有一例患者进展，并且有至少一次的脱髓鞘复发。Chaodong及其同事对7例患者的队列进行了平均为期8年的随访。 其中只有3例患者脱髓鞘复发，且在每例患者随访期内只发生了一次，这增加了Balo同心圆硬化患者的预后比典型多发性硬化患者预后更有利的可能性，至少在初次发病之后对治疗反应较好的患者中。Karaarslan及其同事在一项纳入5例患者平均随访30个月的研究中报道了类似的良好预后，所有的患者至今都没有复发。这个趋势表明在其他非典型脱髓鞘疾病如块状脱髓鞘中已经发现的较为良好的预后。然而，很明显，在这些研究中患者的数量很小，所以有关Balo同心圆硬化患者预后的数据需要谨慎地解释。有部分只有单一Balo病变的患者会复发出现进一步的Balo病变。一例患者从初次发病恢复后，死于4年后第二次发病。有些Balo同心圆硬化的患者预后不良——一项纳入已发表的病例报告的综述表明，14%的患者死亡，严重残疾，症状恶化。在一项纳入17例死于Balo同心圆硬化的患者报告中，死亡发生在该病诊断后的5天至8个月内，死亡通常是由于继发感染如肺炎所致，但有四例患者是死于脑疝。结论和未来的发展方向Balo同心圆硬化是一种具有独特的影像学和病理学特征的罕见脱髓鞘疾病亚型。MRI显示疾病的自然病史各不相同，症状性病灶急性期治疗的恢复是可能的。多发性硬化的疾病修饰治疗在某些符合多发性硬化诊断标准的患者中可能是一种有效的治疗方法，但数据比较缺乏。尽管对Balo同心圆硬化在MRI方面的理解已有近战，但仍有几个方面的不确定性。疾病的罕见性意味不可能存在大型的研究。国际合作性研究或疾病注册研究需要达到足够数量的患者以便能够对疾病的各个方面得出结论。这种回顾性甚至前瞻性病例分析可能有助于对发生率可能的地理变异性，与多发性硬化和其他神经系统疾病相关性的频率，预后特征以及急性和长期疾病修饰治疗等方面提供有意义的数据，进而可能有助于开发循证学治疗指南。特别是，这些数据将对理解日益增多的疾病修饰治疗如何影响Balo同心圆硬化以及指导治疗抉择方面十分有意义。神经免疫学和神经病理学的进展将有望进一步阐明导致出现特征性同心圆状病灶外观的明确事件以及化学介质。尽管Balo同心圆硬化其本身值得研究，明确Balo病变免疫发病机理可能也可以为典型的多发性硬化提供更多的认识和理解。采集对疾病的自然病史及其与其他类型多发性硬化的关系，包括复发缓解型，块状髓鞘脱等是有所帮助的。只有通过继续研究这种不太常见甚至罕见的疾病，才有可能阐明Balo同心圆硬化能否真正被视为一种单独的脱髓鞘疾病或是否为一种关系密切的变异型。
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硬化_百度百科 网页新闻贴吧知道网盘图片视频地图文库资讯采购百科百度首页登录注册进入词条全站搜索帮助首页秒懂百科特色百科知识专题加入百科百科团队权威合作下载百科APP个人中心收藏查看我的收藏0有用+10同心圆性硬化播报上传视频病症本词条缺少概述图，补充相关内容使词条更完整，还能快速升级，赶紧来编辑吧！ 同心圆性硬化（concentricsclerosis）又称Balo病，属大脑白质脱髓鞘性疾病，由Balo于1928年首次报道。因其病理特点为病灶内髓鞘脱失带与髓鞘保存带呈同心圆层状交互排列，形似树木年轮或大理石花纹状而得名。同心圆性硬化的临床表现和病理改变与多发性硬化相似，故多数学者认为它可能是多发性硬化的一种变异型。 中医病名同心圆性硬化别名Balo病就诊科室神经内科多发群体20～50岁常见病因病因未明常见症状脑干、运动、感觉或膀胱直肠功能障碍，明显精神障碍科普中国致力于权威的科学传播本词条认证专家为孙杨丨副主任医师无锡市第二人民医院 神经内科审核目录1病因2临床表现3检查4诊断5鉴别诊断6治疗7预后基本信息中医病名同心圆性硬化别 名Balo病就诊科室神经内科多发群体20～50岁常见病因病因未明常见症状脑干、运动、感觉或膀胱直肠功能障碍，明显精神障碍病因播报编辑病因未明，可能与人类疱疹病毒6（HHV-6）感染后的免疫反应有关。也有学者认为是脱髓鞘疾病；多数认为是急性弥散性硬化的一个亚型或某一阶段；也有人认为是一个独立疾病。一般认为是血管或脑室的一种溶髓鞘物质进入中枢神经系统所致。临床表现播报编辑1.青壮年期发病多见，20～50岁，无明显性别差异，世界上所报告病例70%为亚洲人，以中国、菲律宾和日本最多，可能与种族有关，临床病程无特异性，典型临床表现是亚急性进行性起病的脑病，脑干、运动、感觉或膀胱直肠功能障碍，临床病程可为单相病程，病程较短，进展迅速，但也可发展成临床典型的多发性硬化。2.多数患者以明显精神障碍为首发症状。如沉默寡言、淡漠、反应迟钝、发呆、无故发笑、言语错乱和重复语言等；以后相继出现大脑弥漫性多灶性损害症状和体征，如头痛、偏瘫、失语、眼外肌麻痹、眼球浮动和假性球麻痹等。部分患者可有意识障碍，甚至呈去皮层状态。查体可见轻偏瘫、肌张力增高、腱反射亢进和病理反射等。检查播报编辑1.脑脊液检查无特异性临床异常。压力多正常。脑脊液细胞数正常或轻度增高，蛋白含量可增高，部分病例脑脊液寡克隆区带和IgG指数增高。2.CT检查可见双侧半球多发局限性低密度病灶，多为皮层或皮层下，无明显占位效应，无增强效应。3.MRI检查早期可见“煎蛋样”表现，随着病程进展可见年轮样同心圆带，一般在发病后0.5～3.0个月，可显示同心圆性病变如洋葱头样明暗相间的条纹，与病理所见非常相似。4.脑电图可见中、高波幅慢波，以前头部多见。5.脑立体定向活组织检查可为本病提供重要的病理学诊断依据。诊断播报编辑青壮年，急性或亚急性起病；最常见的临床表现是头痛、失语、偏瘫、认知或行为异常和癫痫发作；MRI示大脑白质多个散在长T1、长T2信号，且呈同心圆改变时，可拟诊为该病。确诊依靠脑组织活检。鉴别诊断播报编辑1.病毒性脑炎二者临床表现相似，但脑炎多起病急骤，男女均可发病，脑脊液检查多有细胞或蛋白轻度增高。2.同心圆性陷窝性白质脑病青壮年发病，女性多见，病程长，表现为视力减退，精神障碍和癫痫，易于鉴别。3.脑脓肿脑脓肿患者无发热，神经影像上病灶缺乏明显的占位效应，而且同心圆性病灶和脑室周围病灶缺乏显著的周边环状强化可排除脑脓肿。治疗播报编辑尚无特异治疗。治疗以肾上腺皮质激素为主，常用大剂量甲基强的松龙冲击治疗，辅以抗癫痫药、抗精神病药物等对症治疗。通常数月后病情可改善。重症患者如使用激素效果较好，可再冲击1个疗程以加强疗效。如激素治疗无效者，可试用血浆置换，但只有在病理上有抗体和补体沉积的2型患者才可能进行血浆置换治疗。预后播报编辑早期研究认为，该病是一种进行性加重的单相疾病，预后极差，多在数周到数月内，因呼吸道感染、肺炎、感染性休克而死亡。随着MRI技术的发展，越来越多的患者在生前得到确诊，并且研究显示该病对类固醇药物敏感，治疗后病灶均趋于缩小或消退，病情好转。总的来说，其预后与治疗早晚、正规与否有很大关系，一般在半年内无缓解者，疗效差。新手上路成长任务编辑入门编辑规则本人编辑我有疑问内容质疑在线客服官方贴吧意见反馈投诉建议举报不良信息未通过词条申诉投诉侵权信息封禁查询与解封©2024 Baidu 使用百度前必读 | 百科协议 | 隐私政策 | 百度百科合作平台 | 京ICP证030173号 京公网安备110000020000BALO | pages — Journal Officiel
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脑白质病变之Balo同心圆硬化，一文读懂
脑白质病变之Balo同心圆硬化，一文读懂
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神经影像教学：Balo病所致的神经功能缺失 - 丁香园
神经影像教学：Balo病所致的神经功能缺失 - 丁香园
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神经影像教学：Balo病所致的神经功能缺失
2015-05-05 21:55
来源：丁香园
作者：心不动则不痛
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近期，Bakhsheshian教授在Neurology杂志上报道了一例经典病例。患者男性，40岁，突发左侧肢体麻木无力，2周后就诊。体格检查发现左侧肢体针刺觉减弱，左侧下肢无力。完善MRI检查结果提示Balo病，即同心圆硬化（BCS, Balo concentric sclerosis），其为一种罕见的脱髓鞘疾病（如图1）。图1. MRI示T2加权像上多发性高信号同心圆病灶，最大的病灶位于右侧顶叶皮质下白质：（A）病灶在横断面T2加权像上呈同心圆样变化；（B）弥散加权像；（C）T1加权延迟扫描成像显示病灶处微弱的斑片状加强。MRI上病灶同心带信号强度存在差异，这是正常组织和脱髓鞘组织交替的原因。BCS通常由单一病灶引起，表现出急性神经症状。临床病程可为爆发性、进展性或良性。在该病例中给予患者高剂量皮质激素治疗1周，病情明显改善。
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同心圆性硬化
同心圆性硬化
同心圆性硬化
同心圆性硬化(concentric1sclerosis)又称Balo病，l属大脑白质脱髓鞘性疾病，l由Balo于1928年首次报道。因其病理特点为病灶内髓鞘脱失带与髓鞘保存带晕同心圆层状交互排列，l形似树木年轮或大理石花纹状而得名。同心圆性硬化的临床表现和病理改变与多发性硬化相似，l故多数学者认为它可能是多发性硬化的一种变异型。【病理】病灶主要位于额、i颞及顶叶白质，l偶见于小脑、i脑干和脊髓。病灶为圆形或不规则形，l直径2～5cm，l呈灰白相间的多层同心圆排列。镜下可见胶质细胞增生、i肥大，l小静脉周围有较多淋巴细胞及少量浆细胞浸润，l并可形成血管套。【临床表现】多见于青壮年女性，l急性或亚急性发病，l半数有低热、i乏力、i头痛等前驱症状，l1～3周症状达顶峰。多以精神障碍起病，l如淡漠、i发呆、i无故哭笑、i重复语言及幻听等，l并可出现失语、i癫癎、i轻偏瘫或四肢轻瘫、i尿便失禁，l部分可有意识障碍，l甚至呈去皮层状态，l查体可见锥体束征和假性球麻痹体征等。【辅助检查】脑脊液常规和生化检查多正常，l个别颅内压稍高。脑电图可见中高波幅慢波。头颅CT可见大脑白质多个散在低密度病灶。MRI可见病灶呈同心圆样改变(图10-3)，l很有临床诊断价值。【诊断与鉴别诊断】根据临床表现和MRI示大脑白质多个散在长T1、i长T2信号，l且呈同心圆改变时，l可拟诊为该病。确诊依靠脑活检。临床主要与病毒性脑炎和急性播散性脑脊髓炎相鉴别。【治疗及预后】治疗以肾上腺皮质激素为主，l辅以抗癫癎药、i抗精神病药物等对症治疗。经过合理治疗，l相当一部分患者可显著恢复，l部分死于并发症，l少数死于脑疝。
Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis - PMC
Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis - PMC
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Neurology. 2021 Jul 27; 97(4): e414–e422. doi: 10.1212/WNL.0000000000012230PMCID: PMC8362356PMID: 34011576Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric SclerosisEvan A. Jolliffe, MBChB, Yong Guo, MD, PhD, Todd A. Hardy, MBBS, P. Pearse Morris, MBBCh, Eoin P. Flanagan, MBBCh, Claudia F. Lucchinetti, MD, and W. Oliver Tobin, MBBCh, BAO, PhDAuthor information Article notes Copyright and License information PMC DisclaimerFrom the Departments of Neurology (E.A.J., Y.G., E.P.F., C.F.L., W.O.T.) and Radiology (P.P.M.), Mayo Clinic, Rochester, MN; Department of Neurology (E.A.J.), Capital and Coast District Health Board, Wellington, New Zealand; Brain & Mind Centre (T.A.H.), University of Sydney; and Department of Neurology (T.A.H.), Concord Repatriation General Hospital, Sydney, NSW, Australia.Corresponding author.Correspondence Dr. Tobin ude.oyam@revilo.nibotGo to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.Received 2021 Jan 7; Accepted 2021 Apr 20.Copyright © 2021 American Academy of NeurologyAssociated DataData Availability StatementAnonymized data within this article will be shared by request from any qualified investigator.AbstractObjective To describe clinical, radiologic, and pathologic features of Baló concentric sclerosis (BCS) and assess overlap between BCS and other CNS inflammatory demyelinating diseases.Methods Retrospective review of BCS cases from US and Australian tertiary care centers.Results We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis–suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within 1 month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially nonenhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on apparent diffusion coefficient, diffusion-weighted imaging, and gadolinium-enhanced imaging was observed in 67% (22/33). Aquaporin-4 immunoglobulin G (n = 26) and myelin oligodendrocyte glycoprotein immunoglobulin G (n = 21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3–100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19–49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III).Conclusions BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.Baló concentric sclerosis (BCS) is a CNS inflammatory demyelinating disease characterized by alternating rings of demyelination and relatively preserved myelin.1 Historically, BCS was thought to have a uniformly poor prognosis. However, earlier cases were based on autopsy studies and milder or self-limiting cases may have been missed or misdiagnosed.2 The availability of MRI has led to a better appreciation of the variable natural history of patients presenting with radiologically evident Baló lesions.3 BCS has been described in association with multiple sclerosis (MS) and seropositive neuromyelitis optica spectrum disorder (NMOSD), indicating a common mechanism of injury or response to injury in these disorders.4 It has also been associated with viral infections, including human herpesvirus 65 and hepatitis C.6 The frequency of oligoclonal bands in patients with Baló lesions is lower than that of typical MS, suggesting that Baló lesions represent a distinct etiology to MS.7Case reports of patients with BCS have described acute plaque pathology consistent with MS type III immunopathologic pattern, characterized by cerebral white matter distal oligodendrocyte loss within demyelinated areas.8 The pathophysiology underlying the concentricity of Baló lesions is debated.1 One hypothesis is that of a hypoxic response to demyelination causing ischemic preconditioning at the leading edge of demyelination, which protects a ring of tissue, leading to alternating myelinated and demyelinated areas.9 Whether BCS is an MS variant, a disease in its own right, or simply a pattern of injury common to a number of demyelinating diseases remains uncertain.Our objective was to describe the clinical, radiologic, and pathologic features of patients with BCS and to assess the overlap between BCS and other CNS demyelinating syndromes.MethodsWe used our institution's medical record diagnostic-linkage system to identify all patients with BCS treated at Mayo Clinic from January 1996 through December 2017 (n = 24). Additional cases were identified from an original cohort of 760 patients belonging to the Multiple Sclerosis Lesion Project (MSLP)–US cohort (n = 12)10 and a cohort based in Sydney, Australia (n = 4). The MSLP study is an international collaborative effort to study the clinical, radiologic, and pathologic correlates of the MS lesion. Patients were included from the MSLP who had (1) tissue diagnosis of inflammatory demyelination confirmed by a specialist in neuropathology of demyelinating disease (Y.G., C.F.L.) to be consistent with MS, with the presence of confluent plaques in active stage of myelin destruction, relative sparing of axons, and glial scarring; (2) no clinical, radiologic, serologic, or pathologic evidence of neoplasm, infection, vascular, or nondemyelinating inflammatory etiology; and (3) no structural or immunocytochemical evidence for an inflammatory demyelinating disease induced by known virus infections, such as subacute sclerosing panencephalitis or progressive multifocal leukoencephalopathy. We conducted a retrospective review of the clinical, radiologic, and pathology records of patients in whom a diagnosis of BCS was considered, MRI demonstrated demyelinating lesions with concentricity, or lesional brain pathology demonstrated alternating bands of demyelination and relatively preserved myelin.Clinical and radiologic data were reviewed by 2 neurologists (E.A.J., W.O.T.) to confirm the diagnosis of BCS by consensus agreement based on interpretation of clinical and radiologic features. The index attack was defined as the constellation of neurologic symptoms leading to diagnosis. Encephalopathy was defined as behavioral change or alteration in consciousness. Improvement in attack-related, targeted neurologic deficits was graded as follows: no improvement if there was no gain in neurologic function; mild improvement if there was improvement in neurologic status without affecting function; moderate improvement if there was definite improvement in function; and marked improvement if there was major functional improvement.11 Disability was measured with the Expanded Disability Status Scale (EDSS).12“Baló concentric sclerosis” lesions were defined radiologically as 2 or more concentric rings of alternating hyperintensity and hypointensity on T2-weighted MRI sequences.13 “Baló-like” lesions were defined as alternating bands of signal intensity (≥2 alternations) on non-T2 weighted MRI sequences or lesions with atypical radiologic features (layered alternating bands, or mosaic patterns) with biopsy evidence of alternating bands of demyelination and relatively preserved myelin. Barkhof criteria for MS were applied to each available MRI.14Neuroimaging was independently evaluated by an experienced neuroradiologist (P.P.M.) blinded to the clinical diagnosis. Radiographic features of interest were index lesion location, size, mass effect, edema, T2-weighted lesion load, diffusion-weighted imaging (DWI) characteristics, and gadolinium enhancement pattern. When multiple active lesions were present, the largest lesion was characterized for analysis. Pathologic tissue from brain biopsy or autopsy was examined by neuropathologists with expertise in CNS demyelinating disorders (C.F.L. and Y.G.). Pathologic material was collected in the Department of Neuropathology at the Mayo Clinic, Rochester, MN; the Neuropathologic Institute at the University of Göttingen, Germany; and the Institute of Brain Research at the University of Vienna, Austria. Active MS lesions were grouped into 1 of 4 distinct immunopathologic patterns, as described previously.15Neuropathologic Techniques and Immunocytochemistry All tissue blocks were classified with regard to lesional activity. Paraffin-embedded 5-μm sections were stained with hematoxylin & eosin, Luxol fast blue myelin stain, periodic acid–Schiff reaction, and Bielschowsky silver impregnation axonal stain.15 Immunohistochemistry was performed without modification on paraffin sections using an avidin-biotin or an alkaline phosphatase/anti–alkaline phosphatase technique as described in detail previously.16 The primary antibodies were omitted in negative controls. In situ hybridization was performed using digoxigenin-labeled riboprobes specific for proteolipoprotein. The source and specificity of the probes, the labeling techniques, and the methods of in situ hybridization have been described in detail previously.17 To visualize degenerating cells in tissue sections, DNA fragmentation within cell nuclei was determined with the method of in situ tailing.18 The sections were then processed for immunohistochemistry with antibodies against myelin oligodendrocyte glycoprotein (MOG), glial fibrillary acidic protein, T cells, and macrophages as described above. Apoptotic oligodendrocytes were defined by nuclear condensation and fragmentation in cells stained by either MOG or cyclic nucleotide phosphodiesterase antibodies. Stored serum was examined for aquaporin-4 (AQP4) immunoglobulin G (IgG) and MOG-IgG with fluorescence-activated cell-sorting assays.19 Other methods used at the time of presentation for AQP4-IgG included cell-based assay (1 patient), ELISA (2 patients), and tissue immunofluorescence (2 patients).20Standard Protocol Approvals, Registrations, and Patient Consents The institutional review boards of the Mayo Clinic, Rochester, MN (IRB 16-010508) and Royal Prince Alfred Hospital, Sydney, Australia (HREC/17/RPAH/145) approved this study. All patients consented to the use of their medical records for research purposes at the time of their clinical evaluation.Statistical Methods All statistical analyses were performed with the patient as the unit of analysis. Even though some patients had biopsy followed by autopsy, they contributed only 1 data point to any analysis, thus maintaining statistical independence among observations. Chi-square tests and Fisher exact tests were used to assess associations. p Values were interpreted as goodness of fit measures indicating how consistent observed data were with the null hypothesis, recognizing that hypothesis testing depends on an idealized set of assumptions such as random sampling from a population.Data Availability Anonymized data within this article will be shared by request from any qualified investigator.ResultsClinical Characteristics Of the 40 patients identified, 29 patients had T2-weighted imaging findings consistent with BCS lesion, 7 patients had Baló-like lesions, 2 patients had atypical lesions with biopsy evidence of alternating bands of demyelination, and 2 patients did not have MRI available but had BCS confirmed on autopsy.The clinical characteristics of the cohort are shown in table 1. At presentation, 7 patients had either an established diagnosis of demyelinating disease (MS, n = 5) or a history suggestive of prior demyelinating events (2 patients). One patient was taking dimethyl fumarate. No other patients were on disease-modifying therapy at presentation. Approximately one-third of patients (13/40) reported prodromal symptoms of fever, malaise, or headache. The most common neurologic features were motor (27/40 [68%]) or sensory deficits (15/40 [38%]) and dysphasia (10/40 [25%]). Ten patients (25%) presented with behavioral change or impaired consciousness consistent with encephalopathy. The median EDSS at presentation was 3.75 (range 1.0–9.5). Initial misdiagnosis was not uncommon: 3 patients were initially thought to have a brain tumor, 1 patient a cerebral abscess, and 1 patient received IV thrombolysis for treatment of suspected stroke.Table 1Patient CharacteristicsOpen in a separate windowNeuroimaging Results Brain MRI was available to review for 38 patients (table 2). Two patients identified by autopsy did not have MRI for review. Ten patients (26%) presented with solitary brain lesions. Additional T2-weighted high-signal lesions were encountered in 74% (28/38), and almost half of the patients (17/38) had more than 1 concurrent gadolinium-enhancing Baló lesion (figure 1). A third of patients (13/38) had lesions suggestive of MS on the index MRI, of whom 10 met Barkhof criteria. Of the patients who underwent biopsy, 5 had an MRI prior to biopsy that fulfilled Barkhof criteria.Table 2Radiographic Characteristics of Lesions Demonstrating Bands of Concentric SclerosisOpen in a separate windowOpen in a separate windowFigure 1Concurrent Baló and Baló-like LesionsFluid-attenuated inversion recovery (FLAIR) and T1-weighted MRI demonstrating concurrent Baló (small arrow) and Baló-like (large arrow) lesions in a single patient.Index lesions were exclusively located in the cerebral hemispheres (frontal lobe, n = 26; parietal lobe, n = 10; temporal lobe, n = 2). The median lesion size in maximum axial dimension on the index T2-weighted images was 31 mm (range 13–79). Edema was present in 76% (29/38), and 24% (9/38) had mass effect, which was predominantly mild. Index lesions had a median of 3 concentric rings (range 2–10). Concentric rings were better visualized on T2-weighted imaging compared to T2/fluid-attenuated inversion recovery (FLAIR) and were better visualized on apparent diffusion coefficient (ADC) imaging compared to DWI. When evaluating the index MRI, concentric rings were better appreciated on ADC when compared to DWI in 25/32 patients with ADC imaging available and were better appreciated on DWI in 9/32 patients when compared to ADC. In 1 patient, the concentric rings were equally apparent on ADC and DWI. Similarly, concentric rings were seen in 31 patients on T2 imaging on the index MRI, compared to 16 patients on FLAIR imaging. In patients in whom both T2 and FLAIR were available, concentric rings were better visualized on T2 in 19/29 patients, they were equally apparent in T2 and FLAIR in 9/29 patients, and were more evident on FLAIR when compared to T2 imaging in 1 patient. Single or multiple gadolinium-enhancing rings were observed in 42% (single, n = 6; multiple, n = 10), and open enhancing rings were observed in 21% (n = 8) of index lesions. Homogenous enhancement within a lesion was not observed.Early MRI performed within 1 month of the index scan was available for 27 patients. The index lesions expanded radially with a sequential increase in the number of T2 rings in 52% (14/27). Discordance between the visualized rings as they appear on diffusion-weighted and gadolinium-enhanced imaging was observed in 70% (19/27) of index lesions with DWI. The lesions often demonstrated an initial phase of DWI restriction followed by a T2 hypointense ring and then later evolution of gadolinium enhancement (figure 2). Multiple enhancing rings were present either on the index MRI (n = 10) or developed on subsequent scans (n = 9). Enhancement was most prominent in the outer ring in 74% (14/19). MRI performed at least 3 months after the index MRI was available for 22 patients. T2 rings remained visible in 64% (14/22). In no patient did the T2/FLAIR abnormality disappear completely.Open in a separate windowFigure 2Temporal Evolution of a Baló Concentric SclerosisDiffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC), T2-weighted (T2), fluid-attenuated inversion recovery (FLAIR), and postgadolinium T1-weighted (T1 Gad) MRI demonstrating evolution of a single lesion with initial peripheral diffusion restriction associated with central enhancement progressing to develop multiple concentric rings, associated with peripheral enhancement, followed by resolution of enhancement and ADC changes with T2-weighted abnormalities being persistently visible at 122 days from the initial MRI.Cerebrospinal Fluid CSF analysis was completed in 33 patients (median 20 days after symptom onset; range 1–544 days). Where recorded, the white cell count was elevated in 25% (7/28) (range 6–27 cells/μL; normal 0–5); protein was elevated in 67% (16/24) (range 40–134 mg/dL; normal 0–35); 2 or more unique CSF oligoclonal bands were detected in 67% (20/30).Antibody Evaluation Patient serum was evaluated for AQP4-IgG or MOG-IgG either at the time of presentation or subsequently on stored serum in 26 and 21 patients respectively and were all negative.Pathology Pathologic specimens from 17 patients (biopsy, n = 15; autopsy, n = 4) were evaluated at Mayo Clinic. Two patients underwent serial biopsy and autopsy. All pathologic specimens demonstrated alternating bands of demyelination and relatively preserved myelin. Immunopathologic phenotyping was performed in 13 patients, and all demonstrated an oligodendrocytopathy pattern (pattern III).15Treatment Outcome The treating physician's diagnosis at the time of index attack was BCS (n = 21), tumefactive demyelination (TD) (n = 10), MS (n = 8), or acute disseminated encephalomyelitis (ADEM) (n = 1). The initial treatment strategy was corticosteroids in 70% (28/40) and concurrent plasmapheresis and corticosteroids in 5% (2/40). A moderate or marked response to corticosteroids alone was seen in 46% (13/28); however, 11% (3/28) had a mild response and 43% (12/28) had no response. One patient who received plasmapheresis in addition to corticosteroids had a moderate response, and the second had no response.In patients who had an incomplete response to the initial treatment for the index attack, a range of therapy options was utilized. A moderate or marked response to treatment was seen in 79% (15/19) of patients. These were treated with additional corticosteroids (6/7); plasmapheresis (5/5); corticosteroids and plasmapheresis (2/2); corticosteroids, IV immunoglobulin (IVIg), and plasmapheresis (1/1); or IV cyclophosphamide (1/1). Mild or no response was seen in 21% (4/19) of patients. These were treated with IVIg (2/2), corticosteroids (1/7), or corticosteroids and cyclophosphamide (1/1).Six patients received prolonged therapy for the index attack, and 4 patients had a moderate or marked response to corticosteroids (2/5), plasmapheresis (1/1), and plasmapheresis and cyclophosphamide (1/1) at 6 months. Seven patients did not receive any treatment. One patient had transient symptoms improving within days, and 5 others either markedly improved or completely recovered in the absence of treatment over 1–6 months. One patient who presented in 1964 did not receive immunotherapy treatment and died 22 days after symptom onset. The initial treatment for 3 patients was unknown.Four patients (10%) (BCS, n = 2; TD, n = 2) died during the index attack. The median time from first manifesting symptoms to death was 23 days (range 19–49). One patient did not receive treatment and died of brainstem compression leading to respiratory arrest. One patient was treated with corticosteroids and plasmapheresis, followed by additional corticosteroids and cyclophosphamide, and ultimately died of pulmonary embolus. One patient had a rapid progression to coma over days and died despite receiving corticosteroids. The cause of death in the fourth patient was unknown.Long-term Outcome Four patients died during the index attack, and 4 patients did not have follow-up data available. Clinical follow-up data of more than 3 months were available for 32 patients (median follow-up 26.5 months; range 3–177). Seventeen patients (53%) were treated with MS disease-modifying medications (interferon, n = 10; natalizumab, n = 5; glatiramer, n = 2) after the index attack, and 2 patients began treatment after further relapses (dimethyl fumarate, n = 1; glatiramer, n = 1). There was no significant difference in the rates of treatment with disease-modifying medication between patients who presented with multiple additional T2 hyperintense lesions on the index MRI (15/24 [63%]) and patients who presented with isolated BCS lesions (2/8 [25%]) (p = 0.11).At a medium follow-up of 27.5 months (range 3–100), 56% (18/32) of patients had a monophasic disease with no further relapse. Of the patients who relapsed, 4 had presented with isolated BCS lesions and 10 had multiple T2 hyperintense lesions on the index MRI (p = 0.70). The median time to relapse was 5 months (range 1–18). Two patients presented with another BCS lesion when they relapsed (2 and 9 months after the index attack). Of the patients with a clinical diagnosis of BCS, 50% (9/18) relapsed during follow-up, compared to 43% (3/7), 33% (2/6), and 0% (0/1) of patients initially diagnosed with RRMS, TD, and ADEM, respectively (p = 0.94). Physician diagnosis at last follow-up was MS (n = 20), BCS (n = 13), TD (n = 6), and ADEM (n = 1). Ten patients (48%) initially diagnosed with BCS had been reclassified by the treating physician as MS, and 2 patients initially diagnosed with TD were reclassified as BCS. The median EDSS at last follow-up was 2.5 (range 0–10). Of 9 patients who were followed for more than 5 years after symptom onset, 4/9 (44%) had subsequent clinical attacks. One patient developed secondary progressive MS and died 14.5 years after the initial event at age 58 years. The cause of death was not recorded.DiscussionAlthough BCS has previously been described as a monophasic fulminant disease, this study describes 40 patients, 10% of whom died from their initial attack. The majority of the cohort did not have a diagnosis of CNS demyelinating disease at presentation. Whereas the majority of patients were ultimately diagnosed with MS, there are clinical, radiologic, and pathologic features in this cohort that point to this, representing a specific subset of MS. The median age of this cohort was 38, which is slightly older than the typical age at onset of MS. Only 67% of patients had positive oligoclonal bands, in contrast with 85%–95% of patients with typical MS. This is consistent with findings of Jarius and colleagues.7 Half of the patients had monophasic disease. Only one-third had imaging features consistent with MS, and most compellingly, all patients with active demyelinating disease had pattern III immunopathology, indicating a common underlying mechanism of injury.We observed BCS lesions occurring de novo, in patients with preexisting MRI changes suggestive of demyelinating disease, and in the context of an established diagnosis of MS. Almost half of the patients diagnosed with BCS experienced subsequent clinical or radiologic relapses consistent with MS. While considered a variant of MS, BCS lesions may not be exclusive to MS and could represent a pattern of injury common among demyelinating lesions. Concentric lesions have also been observed in cases of NMOSD21 and loss of AQP4 expression as can be seen in neuromyelitis optica has also been described in BCS lesions.22 Nonetheless, we did not detect AQP4-IgG or MOG-IgG in any patient with available serum.Serial MRI in the acute phase of the disease reveals how BCS lesions evolve over time. We observed lesions expanding radially with a sequential increase in the number of T2 hyperintense rings. The visualized rings did not precisely match, and discordance between the rings as they appear on ADC, DWI, and gadolinium-enhanced imaging was frequently observed (67%). The lesions often demonstrated an initial phase of DWI restriction followed by a T2 hypointense ring and then later evolution of gadolinium enhancement. Early and dynamic ischemia in the layers of BCS lesions has been reported previously23,24 and observed in MRI studies of biopsy-proven, Baló-like demyelinating brain lesions.25 Diffusion restriction prior to gadolinium enhancement suggests that the initial insult in patients with BCS is a primary brain insult, with secondary breakdown of the blood–brain barrier and ingress of autoinflammatory cells. In a quantitative study, Koelblinger et al26 demonstrated that diffusion restriction in Baló lesions was more prominent in the outer layers of active lesions, consistent with an initial central lesion, growing outward. A hypoxia-like event may precede the demyelinating process in BCS, and tissue preconditioning may give rise to the laminated appearance of BCS lesions.9 Expression of hypoxia-induced tissue preconditioning–related proteins has been observed at the rim of periplaque regions and may provide relative resistance to tissue injury in expanding lesions.9,24Four fundamentally different patterns of demyelination have been described in MS lesions suggesting different targets of injury and mechanisms of demyelination between different subgroups of MS and possibly at different stages of disease development.15 Pattern III lesions are characterized by a distal oligodendrocyte dystrophy. They are composed mainly of T lymphocytes with macrophages and activated microglia and are distinguished from pattern I and II lesions by the absence of immunoglobulin and complement. In a pathologic cohort of MS lesions, a Baló-like concentric pattern was seen at the edge of immunopathologic pattern III lesions only, in the absence of typical Baló-like MRI features.15 In a separate autopsy series of acute MS or fulminant exacerbations of chronic MS with Baló-like lesions seen only on pathology, all cases with concentric demyelination demonstrated immunopathologic pattern III.9 The current series is the first one to our knowledge to demonstrate that radiologic Baló and Baló-like features correlate strongly with an underlying pattern III immunopathology.BCS was historically considered to have a uniformly poor prognosis. MRI has shown that the natural history of the disease varies and favorable outcomes with treatment are not uncommon.27 Recently, in a cohort of patients with Baló-like lesions, marked clinical improvement or full recovery from the initial attack was reported in 83%.27 In our cohort of patients with BCS lesions, we observed a spectrum of possible outcomes, from relatively benign disease, in which symptoms resolved without any immunotherapy intervention, to fulminant disease, resulting in patient death. Of the 4 cases that resulted in death, 1 occurred in an era before immunotherapy was standard clinical care for MS or demyelinating disease.The majority of patients received corticosteroids initially, but almost half required additional therapy. Plasmapheresis is effective in patients with severe demyelinating disease who fail to respond to corticosteroids.11 However, plasmapheresis is thought to be effective in patients with antibody- and complement-mediated tissue destruction and less effective in other patterns of injury. Patients with MS with pattern II pathology are more likely to respond favorably to plasmapheresis than patients with patterns I or III.28 Patients with ring-enhancing lesions, which tend to be more common in pattern II, have been observed to have a more favorable response to plasmapheresis.29 In this cohort, a range of therapy options were utilized and often in combination, making it difficult to comment on specific outcomes. However, 14 patients received plasmapheresis and 11 reported a moderate or marked improvement upon treatment. Two of the 3 patients with biopsy pattern III who received plasmapheresis reported a moderate response.The number of patients in previous studies examining outcomes in patients with BCS lesions has been small. In 1 study, 4 of 7 patients followed for a mean of 8 years remained relapse-free.30 In other studies, all 5 patients2 and 4 of 6 patients27 with a mean follow-up of 2.5 and 1.8 years, respectively, remained relapse-free. In our cohort, 18 of 32 patients had a monophasic disease with no further relapse at last follow-up (median 27.5 months; range 3–100 months). Three patients had greater than 5 years of follow-up without further relapses. Patients who presented with multiple T2 lesions on the index MRI were no more likely to start disease-modifying medication than patients presenting with an isolated BCS lesion, and the rates of relapse on treatment were the same.Limitations of this study include the lack of agreed diagnostic criteria for BCS. Baló-like lesions are often defined radiologically as lesions with multiple concentric rings or a pattern of alternating bands of signal intensity (≥2 alternations) on any sequence, and pathologically as alternating rings of demyelination and relatively preserved myelin. We included patients who met the radiologic definition or had biopsy evidence to supporting a diagnosis of BCS. As a retrospective study, data were not always available, and follow-up was limited in some cases. The quality of MRI varied considerably among cases as MRI technology improved over the years, and earlier scans did not include diffusion-weighted imaging as part of routine MRI evaluations. Clinical and pathologic cases were identified from a tertiary referral-based population. Smaller lesions or lesions that respond to first-line treatments may not initiate a referral. Therefore, the nature of the cases identified here may not be representative of the spectrum of disease.BCS is a rare disorder previously thought to be a fulminant demyelinating disorder with poor outcomes or patient death. However, BCS lesions have a spectrum of possible presentations, which include benign disease. BCS lesions may occur in patients with coexisting MS or may herald the onset of a demyelinating disease that is subsequently clinically and radiologically similar to typical MS. When immunopathologic phenotyping was performed, all lesions demonstrated an oligodendrocytopathy pattern (pattern III). Mechanisms leading to the characteristic concentric layering are not completely understood, but tissue ischemia and tissue preconditioning may give rise to the laminated appearance of BCS lesions.GlossaryADCapparent diffusion coefficientADEMacute disseminated encephalomyelitisAQP4aquaporin-4BCSBaló concentric sclerosisDWIdiffusion-weighted imagingEDSSExpanded Disability Status ScaleFLAIRfluid-attenuated inversion recoveryIgGimmunoglobulin GIVIgIV immunoglobulinMOGmyelin oligodendrocyte glycoproteinMSmultiple sclerosisMSLPMultiple Sclerosis Lesion ProjectNMOSDneuromyelitis optica spectrum disorderTDtumefactive demyelinationAppendix. Authors
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FootnotesCME Course: NPub.org/cmelistStudy FundingThe authors report no targeted funding.DisclosureE.A. Joliffe and Y. Guo have no disclosures relevant to this manuscript. T.A. Hardy attended the Mayo Clinic as a recipient of the Ian Ballard Travel Award funded by MS Research Australia. P.P. Morris and E.P. Flanagan have no disclosures relevant to this manuscript. C.F. Lucchinetti receives funding from Novartis (CFTY720DUS37T) and the NIH (R01NS49577-7). W.O. Tobin has received research funding from the NIH (1R01NS113803-01A1), Mallinckrodt Inc. and the Mayo Clinic Center for MS and Autoimmune Neurology. Go to Neurology.org/N for full disclosures.References1. Hardy TA, Reddel SW, Barnett MH, Palace J, Lucchinetti CF, Weinshenker BG. Atypical inflammatory demyelinating syndromes of the CNS. Lancet Neurol.
2016;15(9):967-981. [PubMed] [Google Scholar]2. Karaarslan E, Altintas A, Senol U, et al.. Baló's concentric sclerosis: clinical and radiologic features of five cases. Am J Neuroradiol.
2001;22(7):1362-1367. [PMC free article] [PubMed] [Google Scholar]3. Hardy TA, Miller DH. Baló's concentric sclerosis. Lancet Neurol.
2014;13(7):740-746. [PubMed] [Google Scholar]4. Graber JJ, Kister I, Geyer H, Khaund M, Herbert J. Neuromyelitis optica and concentric rings of Baló in the brainstem. Arch Neurol.
2009;66(2):274-275. [PubMed] [Google Scholar]5. Pohl D, Rostasy K, Krone B, Hanefeld F. Balo's concentric sclerosis associated with primary human herpesvirus 6 infection. J Neurol Neurosurg Psychiatry.
2005;76(12):1723-1725. [PMC free article] [PubMed] [Google Scholar]6. Hoftberger R, Garzuly F, Dienes HP, et al.. Fulminant central nervous system demyelination associated with interferon-alpha therapy and hepatitis C virus infection. Mult Scler.
2007;13(9):1100-1106. [PubMed] [Google Scholar]7. Jarius S, Wurthwein C, Behrens JR, et al.. Balo's concentric sclerosis is immunologically distinct from multiple sclerosis: results from retrospective analysis of almost 150 lumbar punctures. J Neuroinflamm.
2018;15(1):22. [PMC free article] [PubMed] [Google Scholar]8. Hardy TA, Tobin WO, Lucchinetti CF. Exploring the overlap between multiple sclerosis, tumefactive demyelination and Baló's concentric sclerosis. Mult Scler.
2016;22(8):986-992. [PubMed] [Google Scholar]9. Stadelmann C, Ludwin S, Tabira T, et al.. Tissue preconditioning may explain concentric lesions in Balo's type of multiple sclerosis. Brain.
2005;128(pt 5):979-987. [PubMed] [Google Scholar]10. Pittock SJ, McClelland RL, Achenbach SJ, et al.. Clinical course, pathological correlations, and outcome of biopsy proved inflammatory demyelinating disease. J Neurol Neurosurg Psychiatry.
2005;76(12):1693-1697. [PMC free article] [PubMed] [Google Scholar]11. Weinshenker Brian G, O'Brien Peter C, Petterson Tanya M, et al.. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol.
1999;46(6):878-886. [PubMed] [Google Scholar]12. Kurtzke JF. Rating neurologic impairment in multiple sclerosis. Neurology.
1983;33:1444. [PubMed] [Google Scholar]13. Seewann A, Enzinger C, Filippi M, et al.. MRI characteristics of atypical idiopathic inflammatory demyelinating lesions of the brain: a review of reported findings. J Neurol.
2008;255(1):1-10. [PubMed] [Google Scholar]14. Barkhof F, Filippi M, Miller DH, et al.. Comparison of MRI criteria at first presentation to predict conversion to clinically definite multiple sclerosis. Brain.
1997;120(Pt 11):2059-2069. [PubMed] [Google Scholar]15. Lucchinetti C, Brück W, Parisi J, Scheithauer B, Rodriguez M, Lassmann H. Heterogeneity of multiple sclerosis lesions: implications for the pathogenesis of demyelination. Ann Neurol.
2000;47(6):707-717. [PubMed] [Google Scholar]16. Vass K, Lassmann H, Wekerle H, Wisniewski HM. The distribution of Ia antigen in the lesions of rat acute experimental allergic encephalomyelitis. Acta Neuropathol.
1986;70(2):149-160. [PubMed] [Google Scholar]17. Breitschopf H, Suchanek G, Gould RM, Colman DR, Lassmann H. In situ hybridization with digoxigenin-labeled probes: sensitive and reliable detection method applied to myelinating rat brain. Acta Neuropathol.
1992;84(6):581-587. [PubMed] [Google Scholar]18. Gold R, Schmied M, Giegerich G, et al.. Differentiation between cellular apoptosis and necrosis by the combined use of in situ tailing and nick translation techniques. Lab Invest.
1994;71(2):219-225. [PubMed] [Google Scholar]19. Jitprapaikulsan J, Chen JJ, Flanagan EP, et al.. Aquaporin-4 and myelin oligodendrocyte glycoprotein autoantibody status predict outcome of recurrent optic neuritis. Ophthalmology.
2018;125(10):1628-1637. [PubMed] [Google Scholar]20. Waters PJ, McKeon A, Leite MI, et al.. Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology.
2012;78(9):665-671. discussion 669. [PMC free article] [PubMed] [Google Scholar]21. Masuda H, Mori M, Katayama K, Kikkawa Y, Kuwabara S. Anti-Aquaporin-4 antibody-seronegative NMO spectrum disorder with Balo's concentric lesions. Intern Med.
2013;52:1517-1521. [PubMed] [Google Scholar]22. Matsuoka T, Suzuki SO, Iwaki T, Tabira T, Ordinario AT, Kira J. Aquaporin-4 astrocytopathy in Balo's disease. Acta Neuropathol.
2010;120(7):651-660. [PubMed] [Google Scholar]23. Ripellino P, Khonsari R, Stecco A, Filippi M, Perchinunno M, Cantello R. Clues on Balo's concentric sclerosis evolution from serial analysis of ADC values. Int J Neurosci.
2016;126(1):88-95. [PubMed] [Google Scholar]24. Takai Y, Misu T, Nishiyama S, et al.. Hypoxia-like tissue injury and glial response contribute to Balo concentric lesion development. Neurology.
2016;87(19):2000. [PMC free article] [PubMed] [Google Scholar]25. Abou Zeid N, Pirko I, Erickson B, et al.. Diffusion-weighted imaging characteristics of biopsy-proven demyelinating brain lesions. Neurology.
2012;78(21):1655-1662. [PMC free article] [PubMed] [Google Scholar]26. Koelblinger C, Fruehwald-Pallamar J, Kubin K, et al.. Atypical idiopathic inflammatory demyelinating lesions (IIDL): conventional and diffusion-weighted MR imaging (DWI) findings in 42 cases. Eur J Radiol.
2013;82(11):1996-2004. [PubMed] [Google Scholar]27. Wallner-Blazek M, Rovira A, Fillipp M, et al.. Atypical idiopathic inflammatory demyelinating lesions: prognostic implications and relation to multiple sclerosis. J Neurol.
2013;260(8):2016-2022. [PubMed] [Google Scholar]28. Keegan M, König F, McClelland R, et al.. Relation between humoral pathological changes in multiple sclerosis and response to therapeutic plasma exchange. Lancet.
2005;366(9485):579-582. [PubMed] [Google Scholar]29. Magaña SM, Keegan B, Weinshenker BG, et al.. Beneficial plasma exchange response in central nervous system inflammatory demyelination. Arch Neurol.
2011;68(7):870-878. [PMC free article] [PubMed] [Google Scholar]30. Wang C, Zhang KN, Wu XM, et al.. Baló's disease showing benign clinical course and co-existence with multiple sclerosis-like lesions in Chinese. Mult Scler J.
2008;14(3):418-424. [PubMed] [Google Scholar]Articles from Neurology are provided here courtesy of American Academy of Neurology
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