本文選題：神經(jīng)軸突營(yíng)養(yǎng)不良 + 磁共振成像��； 參考：《中國(guó)臨床醫(yī)學(xué)影像雜志》2015年04期

【摘要】：目的:探討幼兒神經(jīng)軸索營(yíng)養(yǎng)不良(Infantile neuroaxonal dystrophy,INAD)的MRI表現(xiàn)特征,進(jìn)而提高對(duì)該病的影像學(xué)認(rèn)識(shí),為臨床早期診斷提供幫助。方法:對(duì)本院診斷的13例INAD患者的MRI表現(xiàn)進(jìn)行回顧分析,男9例,女4例,記錄小腦萎縮的部位、程度,小腦皮層、白質(zhì)信號(hào)改變,腦干的異常,基底節(jié)及幕上白質(zhì)信號(hào)改變�？偨Y(jié)INAD的頭顱MRI表現(xiàn)特點(diǎn)。結(jié)果:1所有病例均有小腦萎縮,8例為輕度萎縮,5例為重度萎縮;22例伴有大腦萎縮,2例伴有腦干萎縮,1例伴有胼胝體萎縮;32例小腦皮層T2-FLAIR呈高信號(hào);45例幕上腦白質(zhì)T2WI呈高信號(hào);51例雙側(cè)蒼白球T2WI、T2-FLAIR、DWI呈低信號(hào)。結(jié)論:INAD的MRI表現(xiàn)有一定的特點(diǎn),小腦萎縮是最主要的征象,并且呈進(jìn)展性;同時(shí)可以伴有大腦及腦干的萎縮;小腦皮層T2WI信號(hào)增高;基底節(jié)鐵沉積增多所致對(duì)稱(chēng)性T2WI信號(hào)減低;幕上白質(zhì)脫髓鞘,主要表現(xiàn)為深部腦白質(zhì)T2WI信號(hào)增高;灰質(zhì)核團(tuán)較少受累。
[Abstract]:Objective: to investigate the MRI features of infantile neuroaxonal dystrophy (INAD) in order to improve the imaging understanding of the disease and to provide help for early clinical diagnosis. Methods: MRI findings of 13 patients with INAD diagnosed in our hospital were retrospectively analyzed, including 9 males and 4 females. The location and degree of cerebellar atrophy, the changes of cerebellar cortex and white matter signal, the abnormality of brain stem, the signal changes of basal ganglia and supratentorial white matter were recorded. To summarize the cranial MRI features of INAD. Results all cases had cerebellar atrophy in 8 cases, mild atrophy in 5 cases, severe atrophy in 22 cases, brain stem atrophy in 2 cases, cortical atrophy of corpus callosum in 1 case and corpus callosum atrophy in 32 cases. T2-flair of cerebellar cortex showed high signal intensity in 45 cases of supratentorial white matter. In 51 cases of bilateral globus pallidus, T _ 2 WI T _ 2-FLAIRI DWI showed hypointensity on T _ 2WI. Conclusion there are some characteristics in the MRI features of the brain, cerebellar atrophy is the most important sign and is progressive, it can be accompanied by brain and brain stem atrophy, cerebellar cortex T2WI signal is increased, the basal ganglia iron deposition is increased, the symmetrical T 2WI signal is decreased. Supratentorial white matter demyelination was mainly characterized by increased T _ 2WI signal in the deep white matter and less involvement of gray matter nuclei.
【作者單位】： 航天中心醫(yī)院影像科;北京大學(xué)第一醫(yī)院;
【分類(lèi)號(hào)】：R748;R445.2

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