MRI analysis of sulcation morphology in polymicrogyria

MRI analysis of sulcation morphology in polymicrogyria
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DOI:
10.1111/j.1528-1167.2009.02436.x
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发表时间:
2010-02-01
期刊:
影响因子:
5.6
通讯作者:
Barkovich, Anthony James
Barkovich, Anthony James
中科院分区:
医学1区
文献类型:
--
作者:
Barkovich, Anthony James

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方法临床信息并不常见;即使有,通常也很简短,报告精神发育迟缓和癫痫发作,有时还报告家族史;因此,本报告几乎只关注成像特征。所有MRI的质量均为良好至极佳。研究包含的图像在分析后发现,至少有一个小区域的小脑回被薄或浅的脑沟分开,或者有一个明显较厚的皮质区域(5-7 mm),皮质表面或皮质-白质交界处不规则;这些图像是本研究的基础。共分析了159例MRI扫描(7例因图像不满意而排除)。所有患者均在3个平面(矢状面、轴向面和冠状面)采集图像,并具有T1和T2加权图像。作者审查了所有图像的以下特征:异常脑沟的范围和位置;脑回和脑沟的特征;相关异常。根据位置、程度和相关异常以及临床病史(如可用),将研究分为已知的多小脑回类别,这些类别主要基于形态异常的位置和程度。将几例已知临床诊断的患者(9例患有Abladdi综合征,9例患有Zellweger综合征,3例患有巨脑畸形、多小脑回和脑积水(MPPH)综合征,7例患有先天性巨细胞病毒感染)分组在一起;除CMV外,一组中的所有患者均具有相似的PMG定位。不对应于已知遗传或家族性疾病的那些通过形态学分为以下九类:双侧侧裂周PMG(Kuzniecky等人,1993);单侧半球PMG(Chang等人,2006);双侧额叶PMG(Guerrini等人,2000);双侧额顶PMG(Chang等人,2004);双侧旁颅顶枕PMG(Guerrini等,1997);双侧外侧顶骨PMG(Barkovich等,(1999年);双侧不对称PMG;局灶性PMG(局限于单个半球的少于一个叶,尽管它们在多个不同位置,但被组合在一起);和弥漫性PMG(涉及每个半球中4个叶中的至少3个(Chang等人,2004).到目前为止,该组中最常见的PMG分布是双侧外侧裂周(表);这50例患者占分析研究的31%。其次最常见的分布是单侧半球,有31例患者(19%);值得注意的是,所有这些患者的PMG都集中在侧裂区域。其余的分布相当不常见:16例患者(10%)局灶性PMG,12例(8%)双额叶PMG;弥漫性,Aparthodi综合征(所有病例均伴有单侧额叶PMG)和Zellweger综合征(伴双侧枕周PMG)各9例(6%);先天性感染弥漫性7例(4%),伴巨细胞病毒感染6例,淋巴细胞性脉络丛脑膜炎感染1例,双侧额顶6例(4%);双侧不对称性多小脑回和MPPH各3例;双侧顶枕旁和双侧顶外侧PMG各2例(表)。
METHODSClinical information was not often available; when available, it was usually brief, reporting mental retardation and seizures and, sometimes, family history; therefore, this report focuses almost exclusively on the imaging features. All MRIs were of good to excellent quality. Studies contained images that were found, after analysis, to have at least one small region of small gyri separated by thin or shallow sulci or an area of apparently thick cortex (5–7 mm) with irregularity of the cortical surface or the cortical-white matter junction; these images are the basis of this study. A total of 159 MRI scans were analyzed (7 excluded because of unsatisfactory images). All had images acquired in 3 planes (sagittal, axial, and coronal) and had both T1 and T2 weighted images. All images were reviewed by the author for the following characteristics: extent and location of the abnormal sulcation; characteristics of the gyri and sulci; associated anomalies. Based on the location, extent, and associated anomalies, as well as the clinical history (when available), the studies were classified into known categories of polymicrogyria that are largely based upon the location and extent of the morphologic abnormality. Several patients with known clinical diagnoses (9 patients with Aicardi syndrome, 9 with Zellweger syndrome, 3 with megalencephaly, polymicrogyria, and hydrocephalus (MPPH) syndrome, and 7 with congenital cytomegalovirus infection) were grouped together; other than CMV, all patients in a group had similar localization of PMG. Those that did not correspond to known genetic or familial disorders were grouped by morphology into the following nine categories: bilateral perisylvian PMG (Kuzniecky et al., 1993); unilateral hemispheric PMG (Chang et al., 2006); bilateral frontal PMG (Guerrini et al., 2000); bilateral frontoparietal PMG (Chang et al., 2004); bilateral parasagittal parieto-occipital PMG (Guerrini et al., 1997); bilateral lateral parietal PMG (Barkovich et al., 1999); bilateral asymmetric PMG; focal PMG (restricted to less than one lobe of a single hemisphere, grouped together despite they were in multiple different locations); and diffuse PMG (involving at least 3 of the 4 lobes in each hemisphere (Chang et al., 2004).RESULTSBy far, the most common distribution of PMG in this group was bilateral perisylvian (Table); these 50 patients accounted for 31% of the studies analyzed. The next most common distribution was unilateral hemispheric, with 31 patients (19%); of note, all of these patients had PMG centered in the sylvian regions. The remainder of the distributions were considerably less common: focal PMG in 16 patients (10%), bifrontal PMG in 12 (8%); diffuse, Aicardi syndrome (with unilateral frontal PMG in all cases), and Zellweger syndrome (with bilateral perirolandic PMG) in 9 (6%) each; congenital infections (diffuse in 7 (4%), with cytomegalovirus infection in 6 and lymphocytic choriomeningitis infection in 1); bilateral frontoparietal in 6 (4%); bilateral asymmetric polymicrogyria and MPPH in 3 each; and bilateral parasagittal parieto-occipital and bilateral lateral parietal PMG in 2 each (Table).