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[JAMA---NUEROLOGY编译]线粒体细胞疾病患者的脑磁共
Héctor Manuel Barragán-Campos, MD, MSc; Jean-Noël Vallée, MD, PhD; Daouda Lô, MD; Carlos Felipe Barrera-Ramírez, MD; Marcelo Argote-Greene, MD; Jorge Sánchez-Guerrero, MD, MPH; Bruno Estañol, MD; Rémy Guillevin, MD; Jacques Chiras, MD
Arch Neurol. 2005;62:737-742.
Background Mitochondrial cytopathies (MCs) are a heterogeneous group of clinical entities, some of which have classic phenotypes. Magnetic resonance imaging (MRI) has been reported to be helpful in the diagnosis of MC.
Objective To correlate the most common brain MRI findings reported in patients with MC with the clinical findings in patients in different MC subgroups.
Design Case series.
Setting Patients with MCs seen at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Patients Twenty-one patients with MC with the following phenotypes: chronic progressive external ophthalmoplegia (n = 7), Kearns-Sayre syndrome (n = 7), mitochondrial neurogastrointestinal encephalopathy (n = 6), and myoclonic epilepsy with ragged red fiber myopathy (n = 1).
Results Brain MRI abnormalities were found in 20 (95%) of 21 patients. The most frequent abnormalities were widespread white matter hyperintensity in 19 patients (90%), supratentorial cortical atrophy in 18 patients (86%), and cerebellar atrophy in 13 patients (62%). Widespread white matter hyperintensity (P<.001) and supratentorial cortical atrophy (P = .001) were each correlated significantly with MC. Subsequent subgroup analyses showed that the absence of basal ganglia hyperintensity was correlated with Kearns-Sayre syndrome (P < .001) and the presence of supratentorial cortical atrophy was correlated with mitochondrial neurogastrointestinal encephalopathy (P = .005).
Conclusions The presence of widespread white matter hyperintensity and/or supratentorial cortical atrophy in brain MRI may help to establish the diagnosis of MC. The radiologist has a role to play in the workup of MC by confirming the diagnosis and possibly distinguishing different subgroups of MC.
文题:线粒体细胞疾病患者的脑磁共振影像学研究
背景:线粒体细胞疾病MC是一类具有不同典型临床表现的疾病.据报道,磁共振影像学检查对于MC是一种有效的临床诊断手段.
目的:对于不同亚型的MC患者进行MRI影像学结果与临床症状的表现,进行相关性研究.
患者:选择了具有以下临床症状的不同亚型MC患者21名:慢性进行性眼外肌麻痹(7例)，Kearns-Sayre综合征（7名），线粒体离病（6名），伴有碎红细胞肌病的肌阵挛型癫痫（1名）
结果：对于21名患者的MRI影像特征中，19名患者具有广范的脑白质高信号影，18名患者具有幕上皮质萎缩，小脑萎缩13名。相关分析显示：白质高信号和皮质萎缩都和MC症状充分相关，Kearns-Sayre综合征也和幕上皮质萎缩相关。
结论：磁共振上表现为广范的脑白质高信号和幕上皮质萎缩对于诊断MC都具有重要的价值。放射学工作者能够通过确认MRI影像学上可能的表现特征来辅助诊断MC。[标签:content1][标签:content2]
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作者:admin@医学,生命科学 2011-04-18 10:21
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